Editor
Homonymous
Visual Field Defects
123
Homonymous Visual Field Defects
Karolína Skorkovská
Editor
Homonymous Visual
Field Defects
Editor
Karolína Skorkovská
Department of Ophthalmology and Optometry
St. Anne’s University Hospital
Brno
Czech Republic
v
vi Foreword
vii
Acknowledgement
The authors would like to thank Prof. MUDr. Zdeněk Kadaňka, CSc.,
Department of Neurology, University Hospital Brno and Faculty of Medicine,
Brno, Czech Republic, for reviewing this book.
ix
Contents
Index�������������������������������������������������������������������������������������������������������� 175
xi
Contributors
xiii
xiv Contributors
Abstract
Vision is the primary sense in humans. There are approximately one
million axons in the optic nerve, constituting almost 40% of the total
number of axons in all cranial nerves. The primary sensors for sight are
the 130 million rods and seven million cones found in the retina. With
the release of glutamate, they transform electromagnetic waves of light
with a wavelength between 400 and 700 nm to graded changes of the
membrane potential. The signal from photoreceptors continues to the
bipolar cells and then to the retinal ganglion cells. Their axons pass
through the optic nerve, the optic chiasm, form the optic tract, and reach
the lateral geniculate body of the thalamus. The axons coming from the
nasal hemiretina are crossed in the optic chiasm, while axons from the
temporal hemiretina stay uncrossed. Neurons of the lateral geniculate
body send their axons to the optic radiation and terminate in the primary
visual cortex – the striate area in the ipsilateral occipital lobe where the
first analysis of visual information is performed. Further processing
takes place in extrastriate visual areas in the occipital, parietal, and tem-
poral lobes. The visual pathway shows a precise retinotopical organiza-
tion at all levels that gives the anatomical background for symptoms
when some part of optic pathway is damaged.
Keywords
Visual pathway • Vascularization • Pathophysiology • Retina • Optic nerve
• Optic chiasm • Optic tract • Lateral geniculate • Optic radiation • Striate
cortex • Extrastriate cortex
Retina
I. - III.
Optic nerve
Optic chiasma
Optic tract
Lateral
geniculate
IV. body
Optic radiation
Striate area
Fig. 1.1 Schematic drawing of the visual pathway and its neuronal composition
1 Anatomy of the Human Visual Pathway 3
1.2 The Retina light hitting the centre and inhibited by light hit-
ting the peripheral area are called ON-neurons.
The retina is the innermost thin layer of tissue Neurons that have the opposite reaction to the
covering the back of the eye. It develops from the light are known as OFF-neurons.
optic vesicles of the hindbrain. Each optic vesicle
“caves in” to form the optic cup, which consists
of two layers and is connected to the developing 1.2.1 T
he First Neuron: Rods
brain by the optic stalk. The outer layer of the and Cones
optic cup becomes the pigment epithelium of
the retina, and the inner layer differentiates into the The outer part of the retina adjacent to the
complex neural layer of the retina. The optic stalk choroid is pigment epithelium composed of
becomes the optic nerve. cuboidal cells with pigmented granules in
The retina is functionally divided into small their cytoplasm. Internal to this layer is a layer
spots called receptive fields, composed of the cir- of photoreceptors. There are two types of pho-
cular receptive field centre and the peripheral toreceptors in the retina, the rods and cones,
area (Fig. 1.2). The neurons that are excited by which represent the first neuron of the optic
Photoreceptors
Fig. 1.3 A highly simplified picture of cellular connec- 1.2.2 Second Neuron: Bipolar Cells
tions in the retina (Adapted from Brodal [1] by permission
of Oxford University Press, USA)
The bodies of bipolar cells form the inner nuclear
layer of the retina. Their dendrites are in contact
with the base of the rods and cones. In cones
pathway (Fig. 1.3). The retina contains 130 there are two kinds of bipolar cell: ON bipolar
million rods, which are much more sensitive cells are excited when light hits the photorecep-
than the cones and react to extremely small tor and are inhibited in the dark. The second type
amounts of light. They are responsible for of bipolar cell is excited in the dark and inhibited
vision when the light is dim – scotopic vision. in light; therefore, they are called OFF bipolar
Rods contain the photopigment rhodopsin – cells. In rods all bipolar cells are hyperpolarized
composed of a protein part, opsin – and retinal, when the light hits the rods (Fig. 1.4).
1 Anatomy of the Human Visual Pathway 5
Amacrine cell
OFF bipolar
ON bipolar
Horizontal cell
Cone
Rod
Fig. 1.4 Schematic drawing of two types of bipolar cells and their connection with the ganglion cells of the retina
(Adapted from Brodal [1] by permission of Oxford University Press, USA)
1.2.3 T
he Third Neuron: Retinal the receptive field. This inhibition is processed
Ganglion Cells via horizontal cells [1–4]. Apart from the divi-
sion of ganglion cells to ON and OFF, anatomic
The dendrites of ganglion cells are in contact studies of the monkey found that these cells dif-
with ON or OFF synaptic centers via axons of fer greatly in size. Therefore, we can distin-
bipolar cells. The ON ganglion cells are excited guish the M-cells (magnocellular) and P-cells
when the light hits the centre of the receptive (parvocellular). The P-cells have smaller cell
field and inhibited by light on the periphery of bodies, a less extensive dendritic tree, and
6 M. Joukal
Lateral
geniculate M P
body
III. M III. P
II. II.
Retina
I. I.
Fig. 1.5 Schematic diagram of the magnocellular (M) and parvocellular (P) retinal ganglion cells projection through
the lateral geniculate body to the visual cortex
a thinner axon than M-cells. The P-cells are h orizontal cells. Amacrine cells are intercalated
most numerous; they constitute about 80% of between bipolar cells and ganglion cells within
all ganglion cells in the retina. A major differ- the inner nuclear layer. They are in contact with
ence in comparison to M-cells is that the P-cells the axons of the bipolar cells and dendrites of the
respond preferentially to light with a particular ganglion cells. Many bipolar cells of rods exert
wavelength. This means that P-cells are color- their effect on ganglion cells only or mainly via
specific, whereas M-cells do not have such amacrine cells. Amacrine cells are responsible
specificity. Axons of M- and P-cells terminate for interaction between ON and OFF synaptic
on M- and P-neurons of the lateral geniculate centers, which is important for the increase of
body, respectively (Fig. 1.5) [7]. contrast and the detection of motion. The hori-
zontal cell processes establish contact with the
inner segments of the photoreceptors and with
1.2.4 Interneurons of the Retina the dendrites of bipolar cells. Therefore, they
serve for regulation of transmission from the
There are two kinds of interneurons in the photoreceptors to the bipolar cells. Horizontal
retina that are responsible for visual information cells are responsible for the typical receptive
processing based on modulation of bipolar
fields of the bipolar cells and ganglion cells with
and ganglion cells activity – amacrine cells and central excitation and lateral inhibition.
1 Anatomy of the Human Visual Pathway 7
There are two parallel signal pathways from and runs within the nerve to the eyeball. It
the cones. The ON ganglion cells increase the emerges at the optic disc where it divides into the
impulse frequency when the light hits the cones terminal branches for each quadrant of the retina.
to which they are connected. The OFF ganglion The retina can also be supplied by variant cilio-
cells are stimulated in darkness. This organiza- retinal arteries that are branches of ciliary arter-
tion increases the range of light intensities more ies. This variant is found in approximately 20%
than if there were only one channel. The func- of the population (Fig. 1.6) [3, 8, 9].
tional connection of neurons in the retina comes Each segment of the capillary network is
from photoreceptors to bipolar cells and then to drained by retinal venules that continue into pro-
ganglion cells. The axons of ganglion cells form gressively larger vessels. These venules constitute
the optic nerve. Comparison of the number of the central retinal vein that exits the eyeball. Latent
photoreceptors (more than 100 million) and collaterals between the central retinal vein and the
ganglion cells (one million) shows that there is a choroidal venous drainage can be located at the
large convergence of signals in the retina. In border between the optic nerve and the retina [10].
addition to the direct connection of neurons, the
signal is also conducted via interneurons [1–4].
1.2.6 Lesions of the Retina
7
6
1.4.2 Lesions of the Optic Chiasm Such defects may be mistaken for cecocentral
scotomas and attributed to a toxic, metabolic, or
In a contrast to lesions of the optic nerve, where even hereditary process rather than to a tumor;
monocular blindness occurs, lesions in the optic however, in true bitemporal hemianopic scotomas
chiasm produce various visual symptoms accord- color perception and visual acuity are spared, in
ing to their localization. These lesions can be contrast to that in central scotomas [19].
divided into those that affect the anterior angle,
the body, and the posterior angle of the optic chi-
asm [19]. The anterior angle lesions, where the 1.4.3 B
lood Supply of the Optic
fibers from the nasal hemiretina are localized, Tract
induce varying degrees of temporal defect in the
ipsilateral eye. The specific visual field defect is The optic tract is mainly supplied by the anterior
called “junctional scotoma” and affects the con- choroidal artery (branch of the internal carotid
tralateral superior temporal field. In case of exten- artery) and by the posterior communicating artery
sive lesion in the anterior angle of the optic chiasm (see Fig. 1.6). The anterior half is supplied by both
monocular blindness can also occur [11, 20]. arteries, while the posterior half is supplied only
Bitemporal hemianopia is a specific symptom of by the anterior choroidal artery. The collaterals of
lesions located in the body of the optic chiasm the anterior choroidal artery are found on the tem-
where the crossed fibers from the nasal hemireti- poral side of the optic tract, while on the nasal side
nae of both eyes are affected (Fig. 1.8) [1, 19]. they come from the posterior communicating
Posterior angle lesions in the optic chiasm are artery. Within the optic tract a very rich microvas-
expressed by bitemporal hemianopic scotomas. cularization provides possible collateral blood cir-
culation [21]. The superior part of the optic chiasm
and the optic nerve is drained to the venous plexus
Bitemporal hemianopsia
that is opened to the anterior cerebral veins. The
inferior part is drained by the venous plexus that
empties into the basal vein [10].
Homonymous hemianopsia
of the internal carotid artery) and two or three
posterior choroidal arteries that are branches of
the P2 segment of the posterior cerebral artery
(see Fig. 1.6) [24]. These arteries form a network
on the surface of the lateral geniculate body and
can provide c ollateral blood circulation in case of
occlusion of one artery. The superficial capillary
network gives off small arterioles that directly
supply the lateral geniculate body [21]. Each six
layers of the lateral geniculate body contain an
individual capillary network connected by
anastomoses.
The lateral and medial horn of the lateral
Optic tract geniculate body is supplied by the anterior cho-
roidal artery, whereas the hilum by the posterior
choroidal artery (branch of the posterior cerebral
artery). In more than 48% of individuals the lat-
eral geniculate body receives blood also from
other branches of the posterior cerebral artery
such as the hippocampal, anterior temporal, pos-
terior temporal, and parietooccipital artery, and
the middle posterior choroidal artery [25].
Fig. 1.9 Lesion of the visual pathway behind the chiasm 1.5.2 L
esions of the Lateral
produces homonymous hemianopia
Geniculate Body
ventrodorsal direction) separated by axons and den- Lesions of the lateral geniculate nucleus are
drites. The two anterior layers are formed by bodies found less frequently than those of the optic tract,
of large neurons and are therefore called magnocel- and most frequently are caused by infarction of
lular layers. The posterior four layers are composed the anterior or lateral choroidal arteries [24]. The
of small cells and are called parvocellular layers. lateral and medial portions of the lateral genicu-
Large retinal ganglion cells (M-cells) send their late nucleus represent the superior and inferior
axons to the magnocellular layers of the lateral hemifields, respectively. These portions are sup-
geniculate body, whereas the small retinal ganglion plied mainly by the anterior choroidal artery;
cells (P-cells) send their axons to the parvocellular therefore, its occlusion causes a quadruple sec-
layers. Three layers receive the crossed axons while toranopia that is an incomplete wedge-shaped
the other three layers receive the uncrossed axons. homonymous hemianopia [26, 27]. The hilum of
The bodies of neurons in layer 2, 3, and 5 receive the the LATERAL GENICULATE nucleus is sup-
information from the ipsilateral temporal hemiret- plied by the lateral choroidal artery; its occlusion
ina, while layers 1, 4, and 6 receive information induces homonymous horizontal sectoranopia
from the contralateral nasal hemiretina (Fig. 1.10). [11, 28, 29].
1.5.1 B
lood Supply of the Lateral 1.6 Optic Radiation
Geniculate Body
Neurons of the lateral geniculate body send their
The lateral geniculate body has dual bloody axons to the cortex. These axons form the optic
supply: from the anterior choroidal artery (branch radiation as a part of the posterior limb of the
1 Anatomy of the Human Visual Pathway 11
Fig. 1.10 Schematic
drawing of the lateral
geniculate body
organization. Six cellular
layers (1–6) formed by
magnocellular (M) and
parvocellular (P) cells
connect with the ipsilateral
(I) temporal hemiretina
and contralateral (C) nasal
hemiretina
n.opticus
chiasma
opticum
tr.opticus
P
6
5 P
C 4 P
I 3 M
C 2 M
I 1
I
C
artery, and anterior and middle temporal arter- visual field. Lesions in the inferior temporal com-
ies. Lastly, the posterior segment of the optic ponents of the optic radiation result in a contralat-
radiation receives branches from the middle eral superior quadrantanopia or wedge- shaped
cerebral artery and the posterior cerebral artery. defect (“pie-in-the-sky”). Damage to the superior
All these branches penetrate directly through parietal fibers of the optic radiation induces con-
the optic fibers [31]. tralateral inferior quadrantanopia or lower
homonymous field defect [19]. Homonymous
hemianopia with macular splitting can occur in
1.6.2 Lesions of the Optic Radiation the case of large lesions of the optic radiation,
often caused by infarction [22].
The fibers of the optic radiation have retinotopic
organization, thus even small structural lesions
produce circumscribed, sharply marginated, abso- 1.7 The Visual Cortex
lute, congruent homonymous contralateral visual
field defects (Fig. 1.11) [32]. The superior fibers 1.7.1 Primary Visual Cortex
carry information from the inferior visual field,
and the inferior fibers inform about the superior The primary visual cortex or striate area (also
known as V1 or visual area 1) is localized along-
side the calcarine sulcus on the medial side of the
Scotoma occipital lobe. The striate area has been named
according to the white stripe of myelinated axons
that runs parallel to the cortical surface. The stri-
ate area contains the retinotopic map of the visual
field and approximately 50% of that area repre-
sents only the central 5° of the visual field
(Fig. 1.12) [32]. The primary visual cortex is
formed by six layers of neurons (laminae I-VI) as
part of the neocortex and forms the area 17 of
Brodmann. The axons of the lateral geniculate
body terminate mainly on the lamina IV where
the information is transmitted to the other corti-
cal centers. The cells of the striate area with simi-
lar orientation selectivity are organized to
columns perpendicular to the surface of the cor-
tex. The striate area can be divided into three
basic systems responsible for processing particu-
lar modalities of vision. The first system is
formed by three cortical columns that are specific
for perception from the left and right eyes. This
organization is important for binocular vision
Striate area and basic for depth perception. The second sys-
tem is composed of cells that receive information
from identical retinal positions and have the same
Fig. 1.11 Lesion in the optic radiation or striate area
causes circumscribed, congruent, contralateral, homony- axes of orientation; this provides the perception
mous visual field defects (scotomas) of movement. The third system is organized into
1 Anatomy of the Human Visual Pathway 13
Calcarine sulcus
Visual field
Retina
Ipsilateral Contralateral
Left
Righ
t 1.7.2 Extrastriate Visual Cortex
Left
Rig
ht
Information from the primary visual area is sent
to the associated cortical centers called extrastri-
Fig. 1.13 Schematic picture of the columnar organiza-
tion in the striate cortex (Adapted from Dubový and ate visual areas where the final processing of
Klusáková [2], with permission) vision takes place. As mentioned previously, the
14 M. Joukal
Parietal cortex
(area 7)
Prefrontal
cortex
MT
V4 V2
V1
Extrastriate cortex
(inferotemporal)
Fig. 1.14 Ventral and dorsal pathways from the striate to of movement and space (Adapted from Brodal [1] by per-
the extrastriate cortex. The ventral stream is important for mission of Oxford University Press, USA)
object identification, and the dorsal stream for perception
primary visual cortex is also known as V1; the from the parietooccipital artery in 16%, and
parts of the extrastriate visual cortex are called from the posterior temporal artery in 6%. The
V2–V5. They are composed of Brodmann areas calcarine artery in 75% of cases does not follow
18 and 19 with several subdivisions [10]. the calcarine sulcus. It can be localized at the
Information from the primary visual cortex floor of the calcarine fissure, on the medial sur-
reaches the extrastriate areas via the ventral and face of the occipital lobe paralleling the fissure,
dorsal stream (Fig. 1.14). The ventral stream or upward and posterior to the calcarine sulcus.
passes downward from the occipital lobe to the Sometimes the artery splits into inferior and
temporal lobe and carries information about object superior branches that accompany the superior
identification including shape, contrast, and color, and inferior margin of the calcarine sulcus,
also called the “what” pathway. Information about respectively [35].
spatial features and movement, called the “where” The calcarine artery is supplemented by the
pathway, runs in the dorsal stream upward from parietooccipital artery and/or the posterior tem-
the occipital lobe to the parietal lobe [1–3, 33]. poral artery that are direct branches of the poste-
rior cerebral artery. Furthermore, there are many
possible anastomoses between the cortical
1.7.3 B
lood Supply of the Visual branches (intratree anastomoses) of the posterior
Cortex cerebral artery and between the posterior cerebral
artery and middle cerebral artery [10] supplying,
The occipital lobe of the forebrain is supplied in particular, the macular visual cortex.
by cortical branches of the posterior cerebral The inferior surface of the occipital lobe is
artery (see Fig. 1.6). The calcarine artery and drained via inferior occipital veins to the occipi-
the parietooccipital artery arise from the distal tobasal vein, which opens to the lateral tentorial
part (P3) of the posterior cerebral artery. The sinus. The blood drainage of the lateral surface of
visual cortex that is localized alongside the cal- the occipital lobe is provided by superficial corti-
carine sulcus is mostly supplied by the calcarine cal veins that open to the occipital vein. The
artery. This artery originates directly from the occipital vein faces anteriorly and often opens to
posterior cerebral artery in 78% of individuals, the superior sagittal sinus.
1 Anatomy of the Human Visual Pathway 15
1.7.4 L
esions of the Primary Visual localization of the lesion in the ventral or dorsal
Cortex stream. Lesions in the ventral stream are expressed
by cerebral achromatopsia when the patient
Lesions of the occipital lobe produce almost 40% reports seeing in shades of grey. This could be
of homonymous hemianopias, and 70% of them combined with prosopagnosia, superior quadran-
are arterial infarctions [23]. Lesions of the occip- tanopia, or topographagnosia, when the patient is
ital pole are responsible for contralateral homon- lost in familiar surroundings. The destruction of
ymous scotomas that are extremely congruous. bilateral lingual and fusiform gyri by infarction of
Lesions that are localized more anteriorly involve the posterior cerebral artery results in prosopag-
the peripheral vision; in particular, lesions of the nosia, when the patient is unable to identify and
most anterior edge of the striate cortex can pro- recognize familiar faces. Infarction in the left pos-
duce monocular peripheral temporal defects, the terior cerebral artery territory destroys the inferior
so-called temporal crescent. The lesions that occipitotemporal region, resulting in acquired
affect the area above and below the calcarine sul- alexia, which is expressed by loss of the ability to
cus cause the inferior and superior quadran- read in previously literate subjects with normal
tanopias, respectively [11, 22]. Bilateral lesions visual acuity [1, 11, 37].
of the occipital lobe simultaneously, or more usu- Lesions of the dorsal stream produce Bálint
ally sequentially, can induce any combination of syndrome, which was originally described in
bilateral homonymous hemianopia with or with- patients with bilateral parietal lobe lesions but
out macular sparing and various degrees of con- also in patients with bifrontal lesions. This syn-
gruency [11, 19]. When the striate cortex is drome is a combination of optic apraxia, the
totally damaged, mostly from the cerebrovascu- inability to shift the gaze voluntarily; simultanag-
lar lesions, cortical blindness occurs [36]. nosia, the inability to comprehend the totality of
The macular area of the visual cortex is local- the picture or scene; and optic ataxia that is
ized in a “watershed area” on the boundary expressed by the impairment of visually guided
between the areas perfused by the posterior and grasping or reaching, despite adequate strength
the middle cerebral arteries (see Fig. 1.6). The and coordination [11, 38, 39].
visual cortex subserving midperipheral and
peripheral fields is supplied only by the posterior Conclusion
cerebral artery. Therefore, during times of block- Organization of the visual pathway shows a
age of one of the arteries that supply the water- precise retinotopical organization at all levels.
shed area, such as in atherosclerosis, the Homonymous visual field defects arise due to
ipsilateral macular cortex may be spared from the damage of the optic pathway behind the
ischemia by virtue of its dual supply. This may be optic chiasm by various pathological pro-
an explanation for the phenomenon of macular cesses. Knowledge of the visual pathway anat-
sparing. On the other hand, when there is gener- omy and its peculiarities enables good
alized hypoperfusion state (e.g. in heart failure or correlation with clinical symptoms of visual
intraoperative hypotension), the first area of the pathway damage and provides the background
visual cortex to be affected is that supplied by ter- for appropriate diagnostic and therapeutic
minal branches, the macular visual cortex, result- procedures.
ing in a central homonymous hemianopia.
1.7.5 L
esions of the Extrastriate References
Cortex
1. Brodal P. The central nervous system: structure and
function. 4th ed. New York: Oxford University Press;
Lesions of the temporal extrastriate visual cortex 2010.
cause various changes in the ability to recognize 2. Dubový P, Klusáková I. Základy neuroanatomie a ner-
visual objects. Symptoms differ according to vových drah II. Brno: Masarykova University; 2013.
16 M. Joukal
3. Moore KL, Dalley AF, Agur AM. Clinically oriented 23. Pambakian AL, Kennard C. Can visual function be
anatomy. 7th ed. Baltimore/Philadelphia: Lippincott restored in patients with homonymous hemianopia?
Williams & Wilkins; 2013. Br J Ophthalmol. 1997;81(4):324–8.
4. Gray H, Williams PL, Bannister LH. Gray’s anatomy: 24. Luco C, Hoppe A, Schweitzer M, Vicuña X, Fantin
the anatomical basis of medicine and surgery. 38th ed. A. Visual field defects in vascular lesions of the lat-
New York: Churchill Livingstone; 1995. eral geniculate body. J Neurol Neurosurg Psychiatry.
5. Dacey DM, Liao HW, Peterson BB, Robinson FR, 1992;55(1):12–5.
Smith VC, et al. Melanopsin-expressing ganglion 25. Zeal AA, Rhoton AL. Microsurgical anatomy of the
cells in primate retina signal colour and irradiance posterior cerebral artery. J Neurosurg. 1978;48(4):
and project to the LGN. Nature. 2005;433(7027): 534–59.
749–54. 26. Osborne BJ, Liu GT, Galetta SL. Geniculate quadru-
6. Hattar S, Liao HW, Takao M, Berson DM, Yau ple sectoranopia. Neurology. 2006;66(11):E41–2.
KW. Melanopsin-containing retinal ganglion cells: 27. Frisén L. Quadruple sectoranopia and sectorial optic
architecture, projections, and intrinsic photosensitiv- atrophy: a syndrome of the distal anterior choroi-
ity. Science. 2002;295(5557):1065–70. dal artery. J Neurol Neurosurg Psychiatry. 1979;
7. Shapley R, Hugh PV. Cat and monkey retinal gan- 42(7):590–4.
glion cells and their visual functional roles. Trends 28. Frisén L, Holmegaard L, Rosencrantz M. Sectorial
Neurosci. 1986;9:229–35. optic atrophy and homonymous, horizontal sec-
8. Hayreh SS. The central artery of the retina. Its role in toranopia: a lateral choroidal artery syndrome?
the blood supply of the optic nerve. Br J Ophthalmol. J Neurol Neurosurg Psychiatry. 1978;41(4):374–80.
1963;47:651–63. 29. Saeki N, Fujimoto N, Kubota M, Yamaura A. MR
9. Hayreh SS. The Cilio-retinal arteries. Br J Ophthalmol. demonstration of partial lesions of the lateral genicu-
1963;47:71–89. late body and its functional intra-nuclear topography.
10. Kupersmith MJ. Neuro-vascular neuro-ophthalmology. Clin Neurol Neurosurg. 2003;106(1):28–32.
Berlin/Heidelberg: Springer; 1993. 30. Fujita N, Tanaka H, Takanashi M, Hirabuki N, Abe
11. Swienton DJ, Thomas AG. The visual pathway—
K, Yoshimura H, Nakamura H. Lateral geniculate
functional anatomy and pathology. Semin Ultrasound nucleus: anatomic and functional identification
CT MRI. 2014;35(5):487–503. by use of mr imaging. AJNR Am J Neuroradiol.
12. van Overbeeke Jk Sekhar LN. Microanatomy of the 2001;22(9):1719–26.
blood supply to the optic nerve. Orbit. 2003;22(2):81–8. 31. Sido G. Particular aspects of the arterial blood sup-
13. Kupfer C, Chumbley L, Downer JC. Quantitative his- ply of the optic radiations. Oftalmologia. 2002;52(1):
tology of optic nerve, optic tract and lateral geniculate 66–70. (Article in Romanian).
nucleus of man. J Anat. 1967;101(Pt 3):393–401. 32. Schiefer U, Hart W. Functional anatomy of the human
14. Kennard C, Leigh RJ. Neuro-ophthalmology. Series: visual pathway. In: Schiefer U, Wilhelm H, Hart
Handbook of clinical neurology. 3rd ser., vol. 102. W, editors. Clinical neuro-ophthalmology. Berlin/
Edinburgh/New York: Elsevier; 2011. Heidelberg: Springer; 2007. p. 19–28.
15. Kelts EA. The basic anatomy of the optic nerve
33. Grill-Spector K, Malach R. The human visual cortex.
and visual system (or, why Thoreau was wrong). Annu Rev Neurosci. 2004;27:649–77. Review
NeuroRehabilitation. 2010;27(3):217–22. 34. Hubel DH, Wiesel TN. Ferrier lecture. Functional
16. Wichmann W, Müller-Forell W. Anatomy of the
architecture of macaque monkey visual cortex. Proc
visual system. Eur J Radiol. 2004;49(1):8–30. R Soc Lond B Biol Sci. 1977;198(1130):1–59.
17. Collette JM, Francois J, Neetens A. Vascularization 35. Smith CG, Richardson WF. The course and distribu-
of the optic pathway. V Chiasma Br J Ophthalmol. tion of the arteries supplying the visual (striate) cor-
1957;40(12):730–41. tex. Am J Ophthalmol. 1966;61(6):1391–6.
18. Hughes B. Blood supply of the optic nerves and chi- 36. Aldrich MS, Alessi AG, Beck RW, Gilman S. Cortical
asma and its clinical significance. Br J Ophthalmol. blindness: etiology, diagnosis, and prognosis. Ann
1958;42(2):106–25. Neurol. 1987;21(2):149–58.
19. Levin AL. Topical diagnosis of chiasmal and ret-
37. Rizzo M, Barton JJ. Central disorders of visual
rochiasmal disorders. In: Miller NR, Newman NJ, function. In: Miller NR, Newman NJ, Biousse V,
Biousse V, Kerrison JB, editors. Walsh and Hoyt’s Kerrison JB, editors. Walsh and Hoyt’s clinical neuro-
clinical neuro-ophthalmology. 6th ed. Philadelphia: ophthalmology. 6th ed. Philadelphia: Lippincott
Lippincott Williams & Wilkins; 2005. p. 503–74. Williams & Wilkins; 2005. p. 575–646.
20. Bird AC. Field loss due to lesions at the anterior 38. Rizzo M, Vecera SP. Psychoanatomical substrates of
angle of the chiasm. Proc R Soc Med. 1972;65(6): Bálint’s syndrome. J Neurol Neurosurg Psychiatry.
519–20. 2002;72(2):162–78.
21. François J, Neetens A, Collette M. Vascularization of 39. Chechlacz M, Humphreys GW. The enigma of Bálint’s
the optic pathway. IV. Optic tract and external genicu- syndrome: neural substrates and cognitive deficits.
late body. Br J Ophthalmol. 1956;40:341–54. Front Hum Neurosci. 2014;8:123. doi:10.3389/
22. Zhang X, Kedar S, Lynn MJ, Newman NJ, Biousse fnhum.2014.00123.
V. Homonymous hemianopias: clinical-anatomic cor-
relations in 904 cases. Neurology. 2006;66(6):906–10.
Pathological Physiology
of the Visual Pathway 2
Petr Marsalek, Marek Hajný, and Martin Vokurka
Abstract
Homonymous hemianopia is a visual defect caused by various pathological
processes of the central nervous system, particularly if located beyond the
optic chiasm. In the first part of this chapter, we describe the physiological
principles of cortical visual processing. In the second part, we discuss the
pathological physiology and etiopathogenesis of the disorders.
Causes of homonymous defects include cerebral stroke (primarily), as
well as neurodegeneration, demyelinization, hypoxia, trauma, tumors, and
carbon monoxide intoxication.
Keywords
Carbon monoxide poisoning • Cerebral achromatopsia • Cerebral
stroke • Hemorrhagic stroke • Homonymous hemianopia • Ischemic
stroke • Pathological physiology • Pathophysiology • Visual field defect
2.1.3 Cortical Imagery Is Crossed water, spatial and temporal frequencies can simi-
larly be composed in one percept. Spatial fre-
In normal and pathological physiology, there quency and temporal frequency are two
have been tendencies to search for both utility elementary visual characteristics used by move-
and economy in a particular functional system. ment detectors in all visual systems, including
Such a search for efficiency and teleology raises vision in vertebrates and invertebrate animals [1,
the following questions, “Why are all the path- 2] and artificial vision in robots. These detectors
ways in the central nervous system in man are realized in man by the uncrossed fibers.
crossed? Does it bring a functional advantage?”
It might be that an injury or any disorder affect-
ing the receptor organ does not affect its neural 2.1.5 S
tereo Vision: Only One
center, given that it is on the other side of the of Many Consequences
body. This is on one hand an advantage, on the of Viewing with Two Eyes
other hand, crossed pathways have longer dis-
tances for signal to travel and the crossings are For the proper fusion of two images of both eyes,
particularly vulnerable. coordinated eye movements and proper focusing
Retinal output, which comprises one million are required. Such motor actions of eye conver-
neural fibers, is located within the cerebral cortex gence and divergence, accommodation, and adap-
on the side of the head opposite to the location of tation involve subcortical centers. However, even
the eye. The crossing is a point reflection, in a when the two conditions of correct vergence and
sense. The left visual hemifield projects into the focus are met, the central image processing might
right cortical hemisphere, lower visual hemifield misinterpret information due to occlusions, con-
above the calcarine sulcus and vice versa. flicts between the left and right retina, and other
discrepancies in the visual scene. All these con-
flicts are resolved by ocular dominance, but fore-
2.1.4 T
he Uncrossed Part most by alternating the inputs from the left and
of the Visual Pathway Serves right eye at the rate of 1 per second [3]. Either the
Spatial and Stereoscopic domination or the alternation results in a unified
Vision top level percept generated by higher cortical
areas. The remaining unconflicting content con-
Approximately less than half (47%, see Chap. 1, tributes to stereo vision. This true stereo vision
Anatomy of the Human Visual Pathway) of the starts at near point (punctum proximum) and does
central visual pathway is uncrossed. This has an not reach farther than 3 m [4]. The rest of our spa-
obvious functional importance. Uncrossed fibers tial vision is typically due to monocular modali-
carry the information about spatial relations from ties such as perspective, parallax, image fading,
the corresponding parts of the two retinas. This and color changes with distance. In all modalities
information is utilized in the dorsal group of sec- (e.g., space, motion, color, shading, and more),
ondary visual analyzers (associative visual areas). visual space is multidimensional, recreated by our
This dorsal pathway is called the “where” stream vision as a mosaic of facets in several dimensions.
of the visual pathway, as it serves to locate, where In contrast, these more general properties of
the visual objects are and where they are moving vision in its multidimensionality—stereo vision—
to. The ventral, “what” stream serves object rec- give us geometrical dimensionality in the narrow
ognition (see also 2.1.7). Any visual system capa- sense of the term, the three-dimensional space
ble of sensing motion and space (e.g., vision of perception, as described by Julesz [4], Grossberg
insects and flies in particular), must use and pro- et al. [5], and others.
cess spatial frequency information. Spatial fre- Therefore, the inner image of the outer world,
quency changes occur on any static surface, though endowing a firm illusion of constancy and
where high and low contrast stripes are alternat- stability, is continuously and quickly recreated
ing. Like slowly moving ripples on the surface of by our visual processes. In accord with the
2 Pathological Physiology of the Visual Pathway 21
p henomenological approach we adopt in this text, small group of patients [8]. Subjects with blind-
we can divide the visual processes into early vision sight are capable of a restricted set of visual dis-
(the early 150 ms of cortical processing) and criminations of orientation and motion in their
advanced (high level, late) vision, which includes blind (hemi)-field. Their cortical blindness does
all cortical processing beyond this rather arbitrary not bring them a conscious percept, as they deny
time limit. Clearly, the above-mentioned alternating seeing anything. Yet they are able to track fast-
of inner image due to the conflicts of left and right moving stimuli with their hand and possess
eye images falls into the advanced vision category. visual-motor coordination in similar conditions
These observations have proffered different [9]. They are unable to report these skills or use
opinions of contemporary visual physiology. As them outside of experimental conditions, given
the primary visual cortices (Brodmann area 17) that they are not aware of them. Apparently, in
contain a raw material from the point of view of blindsight, the visual information bypasses some
proper binocular fusion, some authors argue that of the pathways necessary for conscious percep-
these cortices are not accessible to our conscious tion and according to some authors may be driven
perception. Some of these concepts regarding the by subcortical centers [10].
attended and unattended parts of the cortical Also, there is debate as to whether or not face
visual processing are still subject to discussion recognition and object recognition are the same
within the visual science community [6]. modality, with one cortical processing site
Another controversial issue related to homon- (fusiform gyrus, face fusiform area), or include
ymous visual field defects is the existence of the more processing units and, therefore, more
“blindsight” phenomenon [7]. With the advent of modalities. Wilson et al. [11] systematically map
functional imaging methods, the blindsight has the dimensionality of the human face attributes,
been demonstrated reproducibly in a defined like concave or convex noses (see Fig. 2.4 top
right) [12], male or female face, and many other is characteristically fast, motion perceiving,
attributes. Other visual modalities from both and color-blind, while the parvocellular is
ventral “what” and dorsal “where” streams are colorful, slower, and static (Figs 2.5 and 2.6).
multidimensional. Other similar dichotomies can be exempli-
fied like black versus white contrast and other
Hight level
visual tasks
Intermediate
cortical analyses
Early
cortical analyses
Fig. 2.6 Parallel and sequential processing in the visual lists the categories of the individual visual processes
cortex. This figure corresponds to Table 2.2. Figure shows (From Van Essen and DeYoe [12], with permission)
relations of early vision processes (bottom) to late vision (Gazzaniga MS, editor. The cognitive neurosciences.
(and high level visual tasks, top). This schema is Figure 24.7, page 394,© 1994 Massachusetts Institute of
simplification of the information flow, while Table 2.2 Technology, by permission of The MIT Press)
2 Pathological Physiology of the Visual Pathway 23
70
processed throughout the visual association cortices.
50
They are often in specific neocortical layers, parts of
30 MW cones cortical columnar organization, blobs and interblobs,
20 LW cones and regions distinguished by histological dyes [15,
16] (Table 2.2 and Fig. 2.6).
Rods
10 Since not all the modalities are localized, in
Table 2.2 and Fig. 2.6 we review only the high
5 level, phenomenological aspect of the visual corti-
cal hierarchy. Figure 2.6 illustrates that in percep-
tual qualities of object, one modality is, or can be,
Fig. 2.7 Physiological trichromacy arises from three
types of cones working in scotopic light conditions.
recreated from the other, such as the structure from
However, color vision defects in HH are due to central motion, shape from texture, form within contours,
defects. While color deficits like protanopia and deutero- David Marr’s two and half dimensional (2.5D)
anopia arise from retinal defects, color blindness in rare sketch from the primal sketch [17]. There are
types of homonymous disorders arise from the defects in
central color opponent-processing. Cortical color oppo-
many other correspondences between the source
nent systems are illustrated in the bottom middle cell of and the target computational processes. The neces-
Fig. 2.4 sity of hierarchy in the vision was documented by
24
Table 2.2 Perceived visual scene and object consist of individual modalities
Anatomic
Processing levels Modality structures
Visual streams Where (Ventral) Infero-temporal What Postero-parietal (Dorsal) Dorsal and
ventral streams
High level tasks Visual navigation 3D spatial Car driving Face recall Object recall Reading Associative
relationship areas
Intermediate Place Trajectory Visual – Motor 2.5 D Surface properties Form Associative
visual tasks areas
Intermediate Motion from Structure from Shape from texture Contour based Shape from Shape from Associative
visual tasks shading motion form shading disparity areas
Intermediate Optic flow Pattern motion, MT Orientation Illusory contours, Complex shapes Correction for Brodmann areas
analysis MSTD contrast, V1 V1, V2, [16] and patterns V2, illumination V4 18, 19
V4
Early cortical Binocular disparity Direction, speed, Spatial frequency, Orientation, ID, Color Unified photopic Brodmann area
analysis MD, ID MD MD, ID, BD MD and scotopic 17
processes
Pathways Magnocellular Magnocellular Magnocellular Parvocellular Parvocellular Parvocellular Retina, chiasm,
LGN
Adapted from Van Essen and DeYoe [12] and Van Essen and Gallant [15]. From the bottom to the top are individual stages of visual processing from the retina to the highest
visual cortices. The direction of information flow is also from the bottom to the top, however, multiple parallel and collateral processing is involved. The condition described as
illusory contours is that described in Von der Heydt et al. [16]
Abbreviations: 3D three-dimensional, 2.5 D 3D environment of the observer projected onto the 2D planes of the retinas, allowing for some depth perception, MD magnocellular
dominated, ID interblob dominated, BD blob dominated, MSTD dominated by processing in the area MST, V1, V2, V3, V4, V5 (MT) primary (and secondary) visual areas, V5 is
also denoted as MT, MT medio-temporal, LGN lateral geniculate nucleus
P. Marsalek et al.
2 Pathological Physiology of the Visual Pathway 25
the pioneering work of Marr. This work does not connections run from the V1 to V2 and then to V4,
contain many descriptions of processes realized by MT (medio temporal cortex, denoted also as V5)
neurons serving vision, because they were not so and medial superior temporal area (MST, BA 5a).
described at that time. However, the theory pre- Particularly high level faculties are required for
sented there can be successfully applied to both the reading of either phonetic alphabets (such as
biological and robotic vision. It contains a clear Arabic, Bengali, Cyrillic, Greek, Hebrew, Latin,
physical, geometrical, and algebraic account on Japanese Kana, Korean, and so on) or iconic (such
computation involved in vision. This account is as Chinese, denoted Kanji by Japanese, ancient
definitive regarding the abstract processes of hieroglyphic, and others) characters. We mention
vision and successfully stands the test of time. here only the simplest division of alphabets into
Despite the anatomical discontinuities in the these two categories. This is because these two fac-
cerebral cortex, normal visual function gives us ulties quite probably involve two different cortical
seamless perception and subjective representa- analyzers, as it is described by functional magnetic
tion of the outer world. The creation and recre- resonance imaging. Reading disabilities— alexia
ation of the outer world by powerful cortical and dyslexia in one of these two alphabets, or in
processes gives us an illusion of constant and both—may accompany homonymous disorders.
consistent perception with smooth transitions. Perception of stereo disparities (between left
This includes true optical illusions. Illusory con- and right eye) contributes to stereo vision, among
tours demonstrated to arise in the processing of other modalities making up spatial vision. Some
the V2 area are one of the classical examples of of perceptual phenomena related to these modali-
these cortical phenomena [16]. Discontinuities ties are out of the scope of this review and in
related to homonymous defects follow the divi- homonymous defects remain largely intact. An
sion of the visual field into four quadrants. important exception is the defect in the cortical
We must be aware of many additional dichoto- processing of color, which has been described as
mies of the secondary visual cortices, which central color blindness, also termed cerebral
originate in the division between the parvocellu- achromatopsia, in one hemisphere or quadrant.
lar versus the magnocellular pathways. They Primary visual area V1 does not contribute to
divide the mosaic of cortical modalities into two a conscious perception of the outer scene. This
groups, called the “what” and “where” visual has a functional cause, as the primary visual cor-
streams. The first is involved in object and face tex includes perceptual conflicts due to the con-
recognition, while the second serves object loca- flicting information entering both eyes. Higher
tion and movement detection. visual areas are needed to resolve these conflicts.
The “what” (ventral) stream function is the Therefore, the conscious and attentional process-
visual object recognition. It originates from the ing is located in higher areas and not in the pri-
parvocellular pathway, which has slower conduc- mary visual cortex [6].
tion velocity than the magnocellular and trans- Most dichotomies in visual perception arise
mits also color information. Relaying connections from the division of labor between the magnocel-
of the “what” stream can be summarized as: from lular and parvocellular pathways. Magnocellular
the V1 to V2, to V3, to V4, then to IT (inferior neurons are fast and sensitive to high temporal
temporal cortex) and to BA 7a (Brodmann area frequency. Consequently, they are sensitive to
7a, frontal eye field; some of the feed forward motion and are color blind. Parvocellular path-
connections run in parallel). ways are slower, sensitive to higher spatial fre-
The “where” (dorsal) stream function is loca- quency, and bring more details requisite for fine
tion and determining the movement of the salient recognition and color vision. A third category of
points within the visual field. It detects and com- koniocellular neurons have distinct anatomic
pares angular velocity within the visual field. It locations between the two and are not numerous.
originates from the magnocellular, faster pathway, The dichotomies in cortical processing can be
transmitting information in the shades of gray. Its described in both detailed anatomical structure
26 P. Marsalek et al.
and also in higher level cortical processing. The its way into the population between temporary
propagation of visual action potentials to higher retreats. Rarer causes are atrophy or surgical
cortical areas has unique time synchronization removal of some visual areas due to virtually all
properties. Synchrony of action potentials and other causes cited here. Multiple sclerosis also
local field potentials in human and other primates causes homonymous hemianopia (HH).
is related to movement, continuity, and coherence Parkinson disease and Alzheimer disease are
of visual objects [18]. This is documented also by sometimes included. However, these two dis-
both native electroencephalogram recording and eases are not addressed in detail, given the com-
event-related potentials [19]. The timing of action plex effect of these nosological units on the
potentials is important. Their detailed studies cerebral cortex.
bring a new understanding of the binding prob- Congenital occipital hemianopia is due to pre-
lem, bistable percepts, and effects of signal con- natal or perinatal posterior brain damage, includ-
vergence among other phenomena [20]. ing porencephaly, cerebral ischemia, occipital
To understand how visual objects and percepts lobe dysplasia, congenital ganglioglioma, vascu-
are encoded into action potentials remains one of lar malformations associated with Sturge-Weber
the major challenges of visual electrophysiology. syndrome (occurring with or without facial port-
Encoding can be classified as both explicit and wine stains) or occipital arteriovenous malforma-
implicit [6]. Explicit code has a straightforward tions, prenatal injury to the periventricular white
relation to some stimulus qualities (modalities, matter, etc. Most cases of congenital HH are due
see, for example, the best stimuli for simple and to unilateral or asymmetric cerebral lesions, but
complex cells). In implicit code this relation is congenital optic tract syndromes or damage to
not known. the LGN can rarely occur. Congenital hemiano-
Higher cortical processing detects the location pia is usually discovered as an incidental finding
of a target within the visual field; serves object in early adulthood, with the patient having no
recognition, spatial navigation, and reading; and prior knowledge of a visual field defect. Pediatric
enables movement detection and tracking. specialists recognize it often only when it is
Oculomotor programs and eye movement coordi- coupled with other neurological abnormalities
nation are executed in subcortical centers, yet the (e.g., hemiplegia, epileptic seizures, Sturge-
signal that determines eye position is also present Weber syndrome, or complications of arteriove-
in the cortex. nous malformations, etc.). Visual field defects in
congenital hemianopia are invariably absolute in
density, complete, and splitting the macula. With
2.2 athological Physiology
P the possible exception of incongruity in partial
and Etiology of Homonymous hemianopias, there are no known perimetric
Visual Field Defects features that distinguish congenital occipital
hemianopia from acquired homonymous hemi-
2.2.1 H
omonymous Defects Arise anopia. However, what distinguishes an occipital
in the Optic Tract and Beyond hemianopia as congenital is the classic ophthal-
moscopic finding called homonymous hemiano-
The second part of this chapter deals with the pic atrophy, and the presence of a relative afferent
etiopathogenesis of homonymous disorders. pupillary defect, both of which are assigned
Congenital homonymous hemianopia is an to the transsynaptic retrograde degeneration of
uncommon entity and is associated with specific the retinogeniculate striate pathways (see also
features. Acquired homonymous visual field Chap. 4).
defects may arise from hypoxia, vascular prob- Cerebral stroke is the leading cause of hom-
lems such as hemorrhage or ischemia, trauma, onymous visual field defects. It can be ischemic
and tumors. This list continues with infectious and/or hemorrhagic and can be due to different
causes, including tuberculosis, which still makes pathologies of the circulatory system. Hypoxia of
2 Pathological Physiology of the Visual Pathway 27
the mammalian central nervous system is not tol- areas and the higher visual processing is not
erated for long. Irreversible damage starts affected. In laboratory conditions the defects in
between 7 and 10 min from the onset of hypoxia. particular modalities associated with migraine
Hypoxia is better tolerated in newborns and in were classified [22]. From that we can conclude
cooled tissue when metabolic demands are not that driving with a visual migraine is possible, yet
high. The lowered temperature also occurs in not advisable.
cold water drowning or during surgery with We shall treat carbon CO poisoning as a sepa-
extracorporal circulation. Given that gray matter rate cause although very rare. The reason is that
possesses nucleated neurons and a corresponding CO poisoning is a hypoxic insult that selectively
transcription system, gray matter is more sensi- affects different groups of neurons. Transport
tive to hypoxia than white matter, which consists hypoxia in CO poisoning frequently damages the
of axonal fibers, myelin, and glia. visual cortex, because visual areas are more sen-
Multiple sclerosis is caused by antibodies that sitive to this type of hypoxia than other brain
attack the myelin sheath. This condition may regions. Quite variable deficits are due to differ-
cause homonymous disorders and is frequently ent exposition times and different CO concentra-
recurrent. The myelin sheath is either destroyed tions, resulting in oxygen content differences in
by the direct action of the immune system or is the blood. This hypoxia results in various central,
insufficiently produced by the myelin-producing therefore mostly homonymous, visual defects.
cells. Multiple sclerosis is partly reversible, as Patients with CO poisoning may have diverse
the neuronal bodies with nuclei are not affected symptoms, whose explanation requires under-
and the myelin sheath is repaired by glial activity. standing the mechanisms of central visual pro-
Proposed causes of multiple sclerosis include cessing. Some cases manifest a homonymous
both genetic and environmental factors such as color blindness (also called cerebral achromatop-
infections. It is more frequent in women and is sia), motion blindness (also called stroboscopic
the most common autoimmune disorder of the vision), or blindsight (discussed above); in short,
human central nervous system. virtually all homonymous defects can result from
Migraine and epilepsy are seizures that can be CO poisoning. In most cases with good residual
restricted to visual cortices and give rise to the phe- vision, affected patients are astonished when they
nomenology of homonymous defects. They mani- are shown the results of their examination, as
fest as both a scotoma and HH and are restricted to they were not aware that they were afflicted with
a given region of the visual cortex. Epileptic sei- color blindness or other defects in the homony-
zures can occur locally in the visual cortices and mous hemifield.
then spread to a confined region of cerebral cortex. Most head injuries causing homonymous dis-
This is also known as Jacksonian epilepsy. orders are located in multiple sites of the cerebral
A migraine starts as a relatively small discrete cortex, and therefore do not occur as the clear cut
scotoma. Then it travels at slow constant speed cases of HH of other origin. The defects cross
with phosphenes, flickers, checkers, scintillations, anatomic and perfusion boundaries. The cause of
and other similar phenomena in front of the sco- head injury is typically due to some form of vio-
toma and moves across one of the quadrants [21]. lent trauma, where patients are usually males
As the subjects are fully conscious of these phe- under the age of 30. To attempt to restore the
nomena, they can check the central origin of the visual function in patients with traumatic HH, no
scotoma by closing one eye followed by the other, special rehabilitation or approach is used.
which will not alter their perceptions. Some sub- However, more detailed diagnostics can reveal
jects can even drive a car with a migraine-related some additional defects, as both the subjective
scotoma. Experiments in motor vehicle driving and instrumental measurements of visual defects
simulators show what visual skills are required are more difficult in comparison to other sensory
for driving. Classical description of visual and motor subsystems due to the anosognosia
migraine hints that it is located in primary visual characteristic of visual disorders.
28 P. Marsalek et al.
Tumors in general can be classified according are affected. Contrast sensitivity declines with
to their invasiveness as benign or malignant, and the order of 10% when compared with the age
according to their original tissue as primary and matched group [23].
secondary, also called metastases. If affecting the
postchiasmal visual pathway, they can cause Conclusion
HVFD. Expanding brain tumors cause intracra- Clearly, cortical defects as a result of HH,
nial hypertension, which leads to the correspond- hemifield scotomas, hemifield color blindness,
ing symptoms: headache, nausea, vomiting, and other homonymous defects, and blindsight can
other symptoms from vegetative, circulatory, and manifest in many different visual disabilities.
respiratory centers; squinting, blurred vision, and Typically, patients are not aware of the defects,
papilledema. Progression of intracranial hyper- nor the extent and exact quality of the defects,
tension leads to the loss of consciousness and even though they realize that something is
coma and can cause death by suppression of wrong. Less noticed defects are in the non-
these vital centers. Based on diagnosis, treatment dominant (typically, the right) hemisphere.
options include conservative methods like radia- Specifically, in the parietal lobe, where the
tion and chemotherapy, stereotactic surgery, or outer body scheme is represented, defects are
gamma radiation surgery. These choices influ- manifested as hemineglect, as a specific
ence the prognosis and resulting functional instance of an anosognosia. Consequently,
defects of the affected part of the cortex. Intra- patients are not aware of the damage. Most HH
operative stimulation is used to functionally map patients are indifferent to their symptoms.
the borders between the Brodmann areas and Even though the defect persists after a
subregions. While areas associated with speech stroke (which is the most frequent cause of
and language must be spared and locations in HH), patients may report subjective improve-
hemispheres dominant for language are treated ment. After rehabilitation and spontaneous
differently than the nondominant, visual areas are restitution, they feel better and some are able
not considered exclusive in this regard. Therefore, to resume their daily tasks and routine [24].
brain tumors causing homonymous disorders can Rehabilitation can be demonstrated in some
be indicated for surgical extirpation based on the visual tasks, but not as restoring vision in the
pathological diagnosis. While these tumors deficient region of the visual field. Despite
exhibit variable symptoms, their surgical removal having lost the ability to read, patients can
leads to a complete loss of function. regain their reading abilities by training. They
Homonymous disorders are not associated accomplish this by targeting the text out of
with the Alzheimer disease in general. Current their scotoma, while bringing more input to
theories on the pathogenesis of Alzheimer dis- the intact parts of the brain. They learn to use
ease include actions of more than ten genes, prismatic glasses and other compensatory aids
accumulation of tau protein, amyloid protein, and for the same purpose of subjectively widening
decrease of nonspecific cholinergic activation of the visual field. The patients subjectively note
cerebral cortex. Alzheimer disease is a type of improvements, even though visual field wid-
dementia and manifests as cognitive and memory ening cannot be demonstrated objectively [24,
deficit. Dementias in general include more 25]. Therefore, the prognosis of HH is modest,
etiopathogenetical nosological categories. In
yet positive.
dementias, higher cognitive functions, as lan- Further, when axons from the retina cannot
guage and orientation are affected, but visual pro- supply their signal to the major target, the lat-
cessing is not specifically affected. In general, eral geniculate nucleus or LGN, the ganglion
patients with dementia recognize visual objects cells, the LGN cells, and the pyramidal cells
to the extent permitted by their declining cogni- of the fourth layer of the visual cortex undergo
tion. Therefore higher visual functions, but not an involution and reorganization within the
these related to perimeter or contrast sensitivity, possible plasticity of the adult cortex.
2 Pathological Physiology of the Visual Pathway 29
Acknowledgments We thank both Craig Smith for copy 12. Van Essen DC, DeYoe EA. Concurrent processing in
editing and Veronika Sýkorová for drawing figures. This the primate visual cortex. In: Gazzaniga MS, editor.
work is supported by the Graduate Students’ Research The cognitive neurosciences. Cambridge: MIT Press;
Program SVV 2016 no. 260 265 and by the Institutional 1995. p. 383–400.
Support for Long-term Development of Research 13. Jacobs G. Primate photopigments and primate color
Organizations (PRVOUK), no. P24/ LF1/ 3, at Charles vision. Proc Natl Acad Sci U S A. 1996;93:577–81.
University in Prague, the Czech Republic. 14. Hegde J, Van Essen DC. Selectivity for complex
shapes in primate visual area V2. J Neurosci. 2000;
20(5):RC61.
15. Van Essen D, Gallant JL. Neural mechanisms of form
and motion processing in the primate visual system.
References Neuron. 1994;13:1–10.
16. von der Heydt R, Peterhans E, Baumgartner G. Illusory
1. Poggio T, Reichardt W. A theory of the pattern contours and cortical neuron responses. Science. 1984;
induced flight orientation of the fly Musca domestica. 224(4654):1260–2.
Kybernetik. 1973;12(4):185–203. 17. Marr D. Vision: a computational investigation into the
2. Srinivasan MV, Laughlin SB, Dubs A. Predictive cod- human representation and processing of visual infor-
ing: a fresh view of inhibition in the retina. Proc R Soc mation. New York: Henry Holt and Company; 1982.
Lond B Biol Sci. 1982;216(1205):427–59. 18. Eckhorn R, Gail A, Bruns A, Gabriel A, Al-Shaikhli B,
3. Logothetis NK, Schall JD. Binocular motion rivalry in Saam M. Neural mechanisms of visual associative pro-
macaque monkeys: eye dominance and tracking eye cessing. Acta Neurobiol Exp (Wars). 2004;64(2):239–52.
movements. Vis Res. 1990;30(10):1409–19. 19. Hruby T, Marsalek P. Event-related potentials – the P3
4. Julesz B. Foundations of cyclopean perception. wave. Acta Neurobiol Exp (Wars). 2003;63(1):55–63.
Chicago: University of Chicago Press; 1971. 20. Marsalek P, Koch C, Maunsell J. On the relation-
5. Grossberg S, Yazdanbakhsh A, Cao Y, Swaminathan G. ship between synaptic input and spike output jitter in
How does binocular rivalry emerge from cortical mech- individual neurons. Proc Natl Acad Sci U S A. 1997;
anisms of 3-D vision? Vis Res. 2008;48(21):2232–50. 94(2):735–40.
6. Koch C. The quest for consciousness: a neurobiological 21. Goodwin D. Transient complete homonymous hemi-
approach. Englewood: Roberts and Company; 2004. anopia associated with migraine. Optometry. 2011;82:
7. Humphrey NK, Weiskrantz L. Vision in monkeys 298–305.
after removal of the striate cortex. Nature. 1967; 22. Shepherd AJ, Beaumont HM, Hine TJ. Motion pro-
215(5101):595–7. cessing deficits in migraine are related to contrast sen-
8. Schmid MC, Mrowka SW, Turchi J, Saunders RC, sitivity. Cephalalgia. 2012;32(7):554–70.
Wilke M, Peters AJ, et al. Blindsight depends on the 23. Uc EY, Rizzo M, Anderson SW, Shi Q, Dawson JD.
lateral geniculate nucleus. Nature. 2010;466(7304): Driver landmark and traffic sign identification in early
373–7. Alzheimer’s disease. J Neurol Neurosurg Psychiatry.
9. Zeki S. The motion vision of the blind. NeuroImage. 2005;76:764–8.
1995;2(3):231–5. 24. Grunda T, Marsalek P, Sykorova P. Homonymous
10. Weiskrantz L, Barbur JL, Sahraie A. Parameters
hemianopia and related visual defects: restoration
affecting conscious versus unconscious visual dis- of vision after a stroke. Acta Neurobiol Exp (Wars).
crimination with damage to the visual cortex (V1). 2013;73(2):237–49.
Proc Natl Acad Sci U S A. 1995;92(13):6122–6. 25. Peli E. Field expansion for homonymous hemianopia
11. Wilson HR, Wilkinson F, Lin LM, Castillo M. Perception by optically induced peripheral exotropia. Optom Vis
of head orientation. Vis Res. 2000;40(5):459–72. Sci. 2000;77(9):453–64.
Ischemic Stroke and Homonymous
Visual Field Defects 3
Ondřej Volný, Michal Haršány, and Robert Mikulík
Abstract
Cerebrovascular diseases are major causes of morbidity and mortality in
developed and developing countries. The major burden of cerebrovascular
diseases is due to long-term disability and economy losses. In this chapter,
we describe basic epidemiology, etiology, management, treatment
(intravenous thrombolysis and mechanical thrombectomy), and secondary
prevention of ischemic stroke. Special emphasis is put on ischemic strokes
in the territory of posterior cerebral artery, which represent 5–10% of all
ischemic strokes. Over 90% of the patients have visual field defects. The
most frequent type of visual field defect is represented by homonymous
hemianopsia occurring in about three-quarters of the patients with occlu-
sions of posterior cerebral artery.
Keywords
Ischemic stroke • Posterior cerebral artery • Intravenous thrombolysis •
Mechanical thrombectomy
O. Volný, MD (*)
Department of Neurology, St. Anne’s University
Hospital, Brno, Czech Republic
Stroke Research Program, St. Anne’s University
Hospital, International Clinical Research Centre,
Pekarska 53, Brno, 656 91, Czech Republic
e-mail: Ondrej.Volny@seznam.cz;
Ondrej.Volny@fnusa.cz
M. Haršány
International Clinical Research Center, R. Mikulík
St. Anne’s University Hospital and Faculty of Department of Neurology, St. Anne’s University
Medicine, Masaryk University, Brno, Czech Republic Hospital and Faculty of Medicine, Masaryk
University, Brno, Czech Republic
Department of Neurology, Comprehensive Stroke
Center, University Hospital Hradec Kralove, International Clinical Research Center, St. Anne’s
Brno, Czech Republic University Hospital, Brno, Czech Republic
a b c
d e f
g h i
Fig. 3.1 Mechanical thrombectomy of acute occlusion and proximal middle cerebral artery (c). The patient
of right middle cerebral artery using stent-retrievers. A received intravenous thrombolysis and underwent
62-year-old woman presented with left-sided hemipare- mechanical thrombectomy (d–h). A 24-h follow-up non-
sis, central facial palsy, and aphasia (National Institutes contrast CT (i) showed only a hypodensity within the
of Health Stroke Scale 14). Admission noncontrast com- right lentiform nucleus (arrow head). (d, e) Antero-
puted tomography (CT) scan (a) showed a dense middle posterior and lateral angiogram show no flow in the mid-
cerebral artery sign (arrow head); there was no evidence dle cerebral artery territory (arrow heads show clot
of early ischemic changes evaluated by the Alberta localization); (f) immediate flow restoration after the
Stroke Program Early CT Score (b). CT angiography stent-retriever placement; (g, h) complete recanalization
confirmed acute occlusion of the right terminal internal after the stent retrieval
3 Ischemic Stroke and Homonymous Visual Field Defects 35
a b
c d
Fig. 3.2 Ischemic stroke of the right posterior cerebral intracranial arteries showing an occlusion of the right prox-
artery. (a) Admission noncontrast computed tomography imal segment of the posterior cerebral artery (PCA) (green
(CT), very discrete early ischemic changes in the right thal- arrow), clinically causing the left homonymous hemianop-
amus (slightly hypodense area in the deep structures) (white sia. (d) Control CT (after 24 h) demonstrating a demarca-
arrow). (b, c) Axial and coronal CT angiography scans of tion of infarcted area in the right PCA territory
3 Ischemic Stroke and Homonymous Visual Field Defects 37
a b c
Fig. 3.3 Magnetic resonance imaging (MRI) of acute emic stroke. (c) Hyperintensity on T2 fluid-attenuated
ischemic stroke in right posterior cerebral artery. (a) MRI inversion recovery (FLAIR) in the same area as changes
diffusion weighted imaging (DWI) demonstrating a on DWI and ADC maps correlates with acute ischemic
restriction of diffusion in the right occipital lobe (hyper- stroke in the right posterior cerebral artery territory (non-
signal area) (white arrow), corresponding with (b) abnor- correlating hyperintense area on T2 FLAIR represents old
mally low value on apparent diffusion coefficient (ADC) infarction, black arrow)
(hyposignal area) (white arrow) representing acute isch-
Thalamus hemorrhage
Male, 59 y
Fig. 3.5 Left thalamus hemorrhage in a 59-year-old male Wilhelm, Center for Ophthalmology, University Hospital,
patient causing a right wedge-shaped homonymous visual Tübingen, Germany)
field defect (sectoranopia) (Courtesy of Prof. Helmut
3 Ischemic Stroke and Homonymous Visual Field Defects 39
brain” concept). Pre-hospital and in-hospital Thrombectomy within 8 hours after symptom onset in
management, increased public awareness by ischemic stroke. N Engl J Med. 2015;372(24):2296–306.
11. Saver JL, Goyal M, Bonafe A, Diener HC, Levy EI,
campaigns, proper and periodic education of Pereira VM, et al.; SWIFT PRIME Investigators.
ambulance teams, and the desire for improv- Stent-retriever thrombectomy after intravenous
ing the in-hospital management should lead to t-PA vs. t-PA alone in stroke. N Engl J Med.
more patients being treated early and success- 2015;372(24):2285–95.
12. Goyal M, Demchuk AM, Menon BK, Eesa M,
fully, with higher rates of functional recovery Rempel JL, Thornton J, et al.; ESCAPE Trial
and independence after stroke. Investigators. Randomized assessment of rapid endo-
vascular treatment of ischemic stroke. N Engl J Med.
Funding Ondřej Volný, Michal Haršány and Robert 2015;372(11):1019–30.
Mikulík are supported by project no. LQ1605, National 13. Berkhemer OA, Fransen PS, Beumer D, van den
Program of Sustainability II. Berg LA, Lingsma HF, Yoo AJ, et al.; MR CLEAN
Investigators. A randomized trial of intraarterial
treatment for acute ischemic stroke. N Engl J Med.
2015;372(1):11–20.
14. Campbell BC, Mitchell PJ, Kleinig TJ, Dewey
References HM, Churilov L, Yassi N, et al.; EXTEND-IA
Investigators. Endovascular therapy for ischemic
1. Lopez AD, Mathers CD, Ezzati M, Jamison DT, stroke with perfusion- imaging selection. N Engl
Murray CJ. Global and regional burden of disease and J Med. 2015;372(11):1009–18.
risk factors, 2001: systematic analysis of population 15. Wahlgren N, Moreira T, Michel P, Steiner T, Jansen
health data. Lancet. 2006;367(9524):1747–57. O, Cognard C, et al. Mechanical thrombectomy in
2. Feigin VL, Forouzanfar MH, Krishnamurthi R, acute ischemic stroke: consensus statement by ESO-
Mensah GA, Connor M, Bennett DA, et al. Global Karolinska stroke update 2014/2015, supported
and regional burden of stroke during 1990-2010: find- by ESO, ESMINT, ESNR and EAN. Int J Stroke.
ings from the global burden of disease study 2010. 2016;11(1):134–47.
Lancet. 2014;383(9913):245–54. 16. Powers WJ, Derdeyn CP, Biller J, Coffey CS, Hoh BL,
3. Menken M, Munsat TL, Toole JF. The global burden Jauch EC, et al. 2015 American Heart Association/
of disease study: implications for neurology. Arch American Stroke Association focused update of the
Neurol. 2000;57(3):418–20. 2013 guidelines for the early management of patients
4. Astrup J, Siesjo BK, Symon L. Thresholds in cere- with acute ischemic stroke regarding endovascular
bral ischemia – the ischemic penumbra. Stroke. treatment: a guideline for healthcare profession-
1981;12(6):723–5. als from the American Heart Association/American
5. Menon BK, Smith EE, Modi J, Patel SK, Bhatia R, Stroke Association. Stroke. 2015;46(10):3020–35.
Watson TW, et al. Regional leptomeningeal score on 17. Pexman JH, Barber PA, Hill MD, Sevick RJ, Demchuk
ct angiography predicts clinical and imaging outcomes AM, Hudon ME, et al. Use of the alberta stroke
in patients with acute anterior circulation occlusions. program early CT score (ASPECTS) for assessing
AJNR Am J Neuroradiol. 2011;32(9):1640–5. CT scans in patients with acute stroke. AJNR Am
6. Lees KR, Bluhmki E, von Kummer R, Brott TG, Toni J Neuroradiol. 2001;22(8):1534–42.
D, Grotta JC, et al. Time to treatment with intrave- 18. Connolly SJ, Ezekowitz MD, Yusuf S, Eikelboom
nous alteplase and outcome in stroke: an updated J, Oldgren J, Parekh A, et al.; RE-LY Steering
pooled analysis of ECASS, ATLANTIS, NINDS, and Committee and Investigators. Dabigatran versus war-
EPITHET trials. Lancet. 2010;375(9727):1695–703. farin in patients with atrial fibrillation. N Engl J Med.
7. Mazighi M, Serfaty JM, Labreuche J, Laissy JP, 2009;361(12):1139–51.
Meseguer E, Lavallée PC, et al.; RECANALISE 19. Patel MR, Mahaffey KW, Garg J, Pan G, Singer
investigators. Comparison of intravenous alteplase DE, Hacke W, et al.; ROCKET AF Investigators.
with a combined intravenous-endovascular approach Rivaroxaban versus warfarin in nonvalvular atrial
in patients with stroke and confirmed arterial occlu- fibrillation. N Engl J Med. 2011;365(10):883–91.
sion (RECANALISE study): a prospective cohort 20. Granger CB, Alexander JH, McMurray JJ, Lopes
study. Lancet Neurol. 2009;8(9):802–9. RD, Hylek EM, Hanna M, et al.; ARISTOTLE
8. Saver JL, Goyal M, Diener HC, SWIFT PRIME Committees and Investigators. Apixaban versus war-
Investigators. Stent-retriever thrombectomy for farin in patients with atrial fibrillation. N Engl J Med.
stroke. N Engl J Med. 2015;373(11):1077. 2011;365(11):981–92.
9. Goyal M, Demchuk AM, Hill MD. Endovascular 21. Pessin MS, Lathi ES, Cohen MB, Kwan ES, Hedges
therapy for ischemic stroke. N Engl J Med. TR, Caplan LR. Clinical features and mechanism of
2015;372(24):2366. occipital infarction. Ann Neurol. 1987;21(3):290–9.
10. Jovin TG, Chamorro A, Cobo E, de Miquel MA, Molina 22. Cals N, Devuyst G, Afsar N, Karapanayiotides T,
CA, Rovira A, et a1; REVASCAT Trial Investigators. Bogousslavsky J. Pure superficial posterior cerebral
3 Ischemic Stroke and Homonymous Visual Field Defects 41
artery territory infarction in the lausanne stroke regis- 30. Neau JP, Bogousslavsky J. The syndrome of poste-
try. J Neurol. 2002;249(7):855–61. rior choroidal artery territory infarction. Ann Neurol.
23. Kumral E, Bayulkem G, Ataç C, Alper Y. Spectrum of 1996;39(6):779–88.
superficial posterior cerebral artery territory infarcts. 31. Wada K, Kimura K, Minematsu K, Yamaguchi
Eur J Neurol. 2004;11(4):237–46. T. Incongruous homonymous hemianopic scotoma.
24. Lister WT, Holmes G. Disturbances of vision from J Neurol Sci. 1999;163(2):179–82.
cerebral lesions, with special reference to the corti- 32. Han YS, Lee E, Kim JS. Horizontal nystagmus and
cal representation of the macula. Proc R Soc Med. homonymous hemianopia due to lateral genicu-
1916;9(Sect Ophthalmol):57–96. late body hemorrhage. Eur Neurol. 2009;61(6):
25. Ogawa K, Ishikawa H, Suzuki Y, Oishi M, Kamei S. 371–3.
Clinical study of the visual field defects caused by occip- 33. Heldner MR, Zubler C, Mattle HP, Schroth G, Weck
ital lobe lesions. Cerebrovasc Dis. 2014;37(2):102–8. A, Mono ML, et al. National institutes of health stroke
26. Brindley GS, Janota I. Observations on cortical
scale score and vessel occlusion in 2152 patients with
blindness and on vascular lesions that cause loss acute ischemic stroke. Stroke. 2013;44(4):1153–7.
of recent memory. J Neurol Neurosurg Psychiatry. 34. Smith WS, Lev MH, English JD, Camargo EC, Chou
1975;38(5):459–64. M, Johnston SC, et al. Significance of large vessel
27. Isa K, Miyashita K, Yanagimoto S, Nagatsuka K,
intracranial occlusion causing acute ischemic stroke
Naritomi H. Homonymous defect of macular vision and tia. Stroke. 2009;40(12):3834–40.
in ischemic stroke. Eur Neurol. 2001;46(3):126–30. 35. Gilhotra JS, Mitchell P, Healey PR, Cumming RG,
28. Fisher CM. The posterior cerebral artery syndrome. Currie J. Homonymous visual field defects and
Can J Neurol Sci. 1986;13(3):232–9. stroke in an older population. Stroke. 2002;33(10):
29. Kim JS. Posterior cerebral artery disease. In: Grotta 2417–20.
JC, Albers GW, Broderick JP, Kasner SE, Lo EG, 36. Leys D, Mounier-Vehier F, Lavenu I, Rondepierre
Mendelow AD, et al., editors. Stroke: pathophysiol- P, Pruvo JP. Anterior choroidal artery territory
ogy, diagnosis, and management. 6th ed. Philadelphia: infarcts. Study of presumed mechanisms. Stroke.
Elsevier; 2016. p. 393–412 .e5. 1994;25(4):837–42.
Neuro-Ophthalmological
Examination in Homonymous 4
Visual Field Defects
Eleni Papageorgiou and Evangeli Tsironi-Malizou
Abstract
Homonymous hemianopia is the hallmark of postchiasmal brain damage
and a sign of serious underlying neurological disease. Patients are often
unaware of their visual deficit and may present with vague symptoms.
However, there are various neuro-ophthalmological signs, which will alert
the clinician to the possibility of a homonymous visual field defect. First,
precise history taking and questions about quality of life are of outmost
importance in order to proceed to the appropriate diagnostic investiga-
tions. Although perimetry is the mainstay for diagnosis in patients with
suspected homonymous visual field defects, there are numerous addi-
tional tests, which provide useful information regarding the etiology,
extent, localization, and functional significance of the underlying brain
lesion. Even in the era of modern neuroimaging, the clinician should
assess visual acuity, color vision, ocular motility, reading ability, pupil
responses, neuropsychological status, and also perform funduscopy and
optical coherence tomography, in order to make an individualized assess-
ment and choose the most appropriate therapeutic and rehabilitation
interventions.
Keywords
Homonymous hemianopia • Stroke • Postchiasmal • Color desaturation
• Hemifield slide • Hemianopic dyslexia • Confrontation perimetry • Bow-tie
atrophy • Congenital hemianopia
4.1 Introduction
are present, further investigation by means of underlying transient ischemic attack (TIA) or
modern neuroimaging usually determines the stroke. Patients may also complain of distur-
cause of vision loss. However, homonymous bances of equilibrium, in particular a feeling
hemianopia may be also encountered as an iso- that the projection of the body’s center of grav-
lated finding in a patient who visits the ophthal- ity shifts towards the hemianopia, a condition
mologist due to visual disturbances, which do not sometimes termed “homonymous hemianopic
always clearly point towards the underlying cere- visual ataxia.” It was suggested that hemiano-
bral lesion. Hence, a thorough clinical examina- pia increases lateral oscillations in patients in
tion will help the clinician to promptly identify a the standing position and the postural distur-
homonymous visual field defect and proceed to bance is due to tonic visual input from the intact
the necessary diagnostic investigations. hemifield [4].
Useful information can be gained if the clini-
cian escorts the patient to the office. The patient’s
4.2 Taking a History behavior in the waiting room, the patient’s gait
and navigational ability, response to handshak-
Taking a precise history is an essential part of ing, and movements of the face and eyes may
the neuro-ophthalmological examination and provide useful clues about his visual field or level
will help the clinician differentiate the cause of of vision. Patients with homonymous hemianopia
vision loss. Some patients notice the visual may ignore or bump onto objects on their blind
field loss immediately, but others are not aware side, such as door-frames or people, and may find
of an incipient hemianopia and often complain it difficult to navigate in unfamiliar and crowded
about monocular visual loss or bilateral places. An anomalous compensatory head pos-
“blurry” vision [2]. Hence the clinician should ture, such as a head turn to the blind hemifield,
try to determine if the vision loss was monocu- should also be noted [3, 5].
lar or binocular. It is not uncommon for a Specific questions about quality of life and
patient to misinterpret a right homonymous the activities that may be compromised in per-
hemianopia as loss of vision in the right eye. sons with hemianopia can provide information
Additionally, isolated hemianopia does not about the degree of visual field loss and associ-
always lead the patient to see a doctor because ated neurological comorbidities. Patients with
they are unaware of the seriousness of their homonymous scotomas often complain about
condition [3]. Especially if the visual field mobility problems, experience driving difficul-
defect is peripheral and does not affect the mac- ties, and frequently lose their place or become
ular area, it may remain undetected for a long frustrated during reading. There is a misconcep-
time. Some patients with hemianopia seek med- tion that patients perceive a homonymous hemi-
ical advice weeks or even months after lesion anopia as a dark curtain or an opaque black area.
onset, often because they or their family notice The loss from hemianopia reflects a void in the
a compromise in their everyday visual func- vision, and it seems that the brain fills it in per-
tioning, and not because they are aware of the ceptually to blend with whatever the patient is
visual field loss. The clinician should try to viewing [6].
determine the exact time of onset, severity, and Further questioning should be targeted to
duration of symptoms. An acute loss of vision potential causes of a homonymous visual field
(over minutes or hours) points towards trau- defect (tumor, trauma, arteriovenous malforma-
matic or vascular causes, while a subacute or tion, neurosurgery, demyelination, infection, peri-
chronic presentation (over days or weeks) sug- natal injury, dementia, migraine). The majority of
gests an inflammatory, demyelinating, com- homonymous hemianopias results from stroke,
pressive, or degenerative etiology. Sometimes due to embolus, thrombosis, hemorrhage, or dis-
the patient may recall a recent episode of dizzi- section; hence, one should obtain the patient’s
ness, numbness, or diplopia, suggesting an cardiovascular status, such as blood pressure,
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 45
grid have missing or blurred lines. Corresponding or syllables of the line, because the left margin
areas of blurry vision in either eye may suggest a disappears into the scotoma as they scan right-
small central or paracentral scotoma due to ward [7]. Right HVFDs cause more severe read-
occipital stroke [3]. ing difficulties, with loss of the anticipatory
parafoveal scanning process, and significant
reduction of reading speed, resulting in a charac-
4.6 Reading Ability teristic reading disorder termed “hemianopic
dyslexia,” which in some patients is nearly equiv-
Testing for reading ability with the near correc- alent to spelling [7]. Reduced amplitude of read-
tion in place may provide useful information ing saccades to the right and prolonged fixation
about the central visual field. If funduscopy lead to prolonged reading times. Reading speed
excludes macular disease in a patient with read- improves with increasing degree of macular spar-
ing complaints, then a homonymous visual field ing (Fig. 4.2).
defect is possible. Reading ability is commonly Bitemporal hemianopia, usually due to chias-
affected in homonymous scotomas, and patients mal processes such as pituitary adenomas, is
have reading difficulties that reflect the laterality another type of visual field defect associated
of the visual field defect and depend on the degree with reading difficulties that are actually caused
of macular sparing. Standardized assessment of by unstable binocular alignment. In bitemporal
reading speed is ideally performed by validated hemianopia, the peripheral visual field loss is
tests, such as the IReST (International Reading mild, because the function of each blind tempo-
Speed Texts), which are provided in 17 languages ral hemifield is taken over by the nasal hemifield
and allow comparability of results before and of the contralateral eye. The patients often com-
after interventions. plain about transient horizontal diplopia,
Fluent reading demands at least 2° of visual disappearance or vertical splitting of the image
angle to the left and right and 1° above and below during reading, in the absence of extraocular
the central fixation point. Reading disorders of muscle palsies [9]. The symptoms arise due to
patients with homonymous visual field defects the loss of binocular fusion, as there is absence
(HVFDs) result from the loss of parafoveal field of overlapping seeing retinal regions throughout
regions that form a “perceptual window” for the binocular visual field, and each hemifield is
reading [3]. In western societies this reading win- seen by one eye only [10].
dow extends 3–4 characters to the left of fixation This lack of retinal correspondence between
and 7–11 characters to the right; due to the asym- the remaining nasal fields of both eyes decom-
metry of this perceptual window, right-sided pensates any preexisting phoria into a tropia,
HVFDs cut a larger part of the reading window with a resulting visual field defect often referred
and therefore impair reading more than left-sided to as the “hemifield slide” phenomenon [9].
HVFDs. Left HVFDs cause difficulties with eye Depending on the phoria, the two nasal hemi-
movements required to find the beginning of a fields slide relative to each other horizontally or
new line, resulting in omissions of the first word vertically, producing horizontal diplopia (with
Fig. 4.2 (a) Fluent reading requires a central intact visual mous paracentral scotoma can have deleterious effects on
field of at least 4° horizontally and 2° vertically. (b) reading ability if it involves the central “reading window.”
Outside this central area visual acuity is degraded. (c) (f) A left homonymous hemianopia with macular splitting
Right homonymous hemianopia with macular splitting will cause difficulties in finding the beginning of lines and
leads to severe impairment of reading ability, called words. (g) If there is macular sparing, reading ability will
“hemianopic dyslexia.” (d) In case of macular sparing, be intact. (h) A small left homonymous paracentral sco-
reading ability will be intact even in complete right hom- toma will result in reading difficulties, as the central
onymous hemianopia. (e) However, a small right homony- visual field area will be affected
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 47
a c
d e
f g
Fig. 4.2 (continued)
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 49
OS OD
b
1 2 3 4 5 6
A2 Binocular 90° static visual field
90 60 30 90 60 30
Fig. 4.3 (a) Postfixational blindness and the hemifield slide restriction of the peripheral visual field (the temporal cres-
phenomenon in bitemporal hemianopia. A1: monocular 30° cents). (c) In left esotropia there is a vertical scotoma between
static visual fields show bitemporal hemianopia. A2: the cor- the two nasal hemifields, resulting in disappearance of central
responding binocular 90° visual field shows mild peripheral parts of an image. (d) In left exotropia the purple area is dip-
visual field loss. A3: at near fixation there is a blind area lopic due to overlapping of the two nasal hemifields. (e) In left
beyond the fixation point. (b) In orthotropia there is only mild hypertropia there is vertical splitting of the image
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 51
c 1 2 5 6 d 1 2 3 4 4 5 6
90 60 30
90 60 30
Fig. 4.3 (continued)
52 E. Papageorgiou and E. Tsironi-Malizou
Fig. 4.4 (a) Perimetric results in a 32-year-old male visual field using the Octopus 101 instrument’s semiauto-
patient after surgery for a parieto-occipital arteriovenous mated kinetic perimetry (SKP). Graphical representation
malformation. Monocular static automated perimetry of the area of the visual field loss in the binocular visual
within the central 30° visual field in the Octopus 101, field (gray transparent region obtained with stimulus
demonstrating congruous complete left homonymous III4e, angular velocity 3°/s). The patient has a left com-
hemianopia with macular splitting. (b) Binocular 90° plete homonymous hemianopia
54 E. Papageorgiou and E. Tsironi-Malizou
widely used confrontation tests [17]. Initially, to distinguish. The patient is asked to count the
the examiner holds his hands within both hemi- number of fingers [18]. Finally, simultaneous
fields on either side of the vertical midline, and comparison of red desaturation between hemi-
starts waving in only one hemifield at a time. fields is possible by presenting two small red
The patient reports if he sees something mov- objects on either side of the vertical meridian,
ing. This test can be followed by the “finger and can further assist in the detection of hom-
wiggle testing,” where the examiner holds his onymous scotomas (see Sect. 4.4) [3]. Detailed
hands within both hemifields, but wiggles only testing of each of the four quadrants is possible
the index finger rather than the entire hand. As a in all of the above variations of confrontation
final step in the examination, the finger counting perimetry (Fig. 4.5).
confrontation is performed [16]. The examiner For infants and young children the examiner
holds his closed fists simultaneously in both attracts his attention centrally, while an assis-
hemifields to either side of the vertical merid- tant introduces a toy or a light into various
ian, and then raises one, two, or five fingers quadrants of the peripheral visual field. For
briefly, because other combinations are difficult example, the examiner can place a sticker on
a b
c d
Fig. 4.5 (a) Confrontation perimetry is performed mon- moving. (c) During “finger wiggle testing” the examiner
ocularly with the patient fixating on the examiner’s nose. holds his hands within both hemifields, but wiggles only
The examiner closes her eye opposite to the eye the patient the index finger. (d) During finger counting confrontation
has closed and uses her monocular visual field for com- the examiner holds his closed fists simultaneously in both
parison to the patient’s visual field. (b) The examiner hemifields to either side of the vertical meridian, and then
holds his hands within both hemifields on either side of raises one, two, or five fingers briefly, because other com-
the vertical midline, and starts waving in only one hemi- binations are difficult to distinguish. The patient is asked
field at a time. The patient reports if he sees something to count the number of fingers
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 55
his nose and ask the child to observe it, while a child to mimic the finger pattern. In children, an
brightly colored object is moved into the child’s eye patch can be used for monocular viewing
peripheral field. A shift in fixation, head or (Fig. 4.7).
hand movement toward the target, or change in Confrontation visual field is a quick and easy
facial expression of the infant can indicate that to perform test, which can be performed in any
the stimulus was perceived in the periphery. setting, but has a limited ability to detect more
This test is referred to as “evoked saccadic subtle defects; it is not quantitative and cannot be
technique” and is based on the principle that used for follow-up evaluations. For the clinical
stimuli presented in the peripheral field gener- setting, Schiefer et al. [3] have described a practi-
ate reflex eye movements that bring the object cal modification of confrontation perimetry, a
of interest onto the central area (fovea) of the testing that is especially useful in cases of hom-
retina, movements that develop at a very young onymous visual field loss. The patient fixes on
age (Fig. 4.6). the center of the examiner’s face at a distance of
Older children can be shown how to mimic about 30 inches, and he or she reports whether or
finger patterns, which is a simple alternative to not the entire face is simultaneously visible.
the finger-counting confrontation. With the child Depending on the missing portions of the face,
fixating on the examiner’s face, the examiner the examiner can estimate the extent of a hom-
raises one, two, or five fingers briefly and asks the onymous defect (Fig. 4.8).
a b
c d
Fig. 4.6 (a) Confrontation perimetry in infants. The peripheral visual field. (b–d) A rapid eye, head, or hand
examiner attracts the infant’s attention centrally, while an movement toward the target indicates that the stimulus
assistant silently introduces an interesting toy into the was perceived in the periphery
56 E. Papageorgiou and E. Tsironi-Malizou
a b
c d
Fig. 4.7 (a) Confrontation perimetry in children. The finger in the peripheral hemifield. (c) The child mimics
child is asked to show the same number of fingers as the the examiner. (d) In order to avoid eye movements, the
examiner. (b) The examiner tries to maintain the child’s child’s face can be turned away from the hemifield being
fixation centrally, while he quickly displays a number of tested
a b c
7.5°
4° 4°
1° 1° 1°
75 cm 75 cm 75 cm
Fig. 4.8 (a) Estimation of macular sparing in a patient ular sparing is less that 1°. (b) If the examiner’s right eye
with a right homonymous hemianopia by means of a mod- is just visible, then macular sparing is approximately 4°.
ified confrontation visual field test. The patient is asked to (c) If the examiner’s face is visible except for his right ear,
look at the examiner’s nose from a distance of about then macular sparing is approximately 7.5°
75 cm. If the examiner’s right eye is not visible, then mac-
findings on funduscopy. However, recent optical tal or early acquired occipital lesions that are
coherence tomography (OCT) findings have accompanied by clinically detectable optic disc
demonstrated retinal ganglion cell (RGC) layer pallor and band atrophy. Finally, retinal vascular
and peripapillary retinal nerve fiber layer (RNFL) changes, such as arteriolar narrowing, arteriove-
thinning corresponding to the hemianopic visual nous nicking, changes in the arteriolar wall (arte-
field loss in patients with acquired occipital riosclerosis), or diabetic retinopathy signs, should
lesions (see Sect. 4.11) [20, 21]. This finding pro- be carefully documented, as they may suggest a
vides evidence for transsynaptic retrograde vascular etiology of the homonymous visual field
degeneration, similar to that observed in congeni- defect.
58 E. Papageorgiou and E. Tsironi-Malizou
c
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 59
Fig. 4.9 (a) Βow-tie pattern of optic atrophy in a Monocular static automated perimetry within the central
44-year-old female patient with left optic tract lesion due 30° visual field in the Octopus 101, demonstrating incon-
to motor vehicle induced traumatic brain injury. The right gruous right homonymous hemianopia with macular
optic disc (contralateral to the lesion) demonstrates a splitting due to a left optic tract lesion. (c) Monocular 90°
horizontal band-shaped pattern of optic atrophy (dashed visual field using the Octopus 101 instrument’s semiauto-
lines), while the left optic disc (ipsilateral to the lesion) mated kinetic perimetry (SKP). Graphical representation
shows temporal optic atrophy (white arrow). There is of the right homonymous hemianopia in the above patient
also a right RAPD (Image courtesy of Prof. Ulrich (gray transparent region obtained with stimulus III4e,
Schiefer, Hochschule Aalen, Aalen, Germany). (b) angular velocity 3°/s)
60 E. Papageorgiou and E. Tsironi-Malizou
of retinal ganglion cells in both congenital and type of nonorganic visual field defect in gen-
acquired hemianopias. Patients with congenital eral. In case of an alleged hemianopic field
hemianopia also show a subtle RAPD in the eye defect, the most important examination is bin-
with the band-shaped atrophy, namely, contralat- ocular perimetry. Goldmann perimetry is a
eral to the brain lesion. This relative afferent pap- valuable tool that may confuse a simulator due
illary defect is also attributed to transsynaptic to its relatively complex procedure. Automated
degeneration of the pupillomotor fibers that syn- static perimetry is generally not helpful in the
apse in the pretectal area of the midbrain [27]. evaluation of suspected functional vision loss
Acquired homonymous hemianopia that due to inconsistent responses and poor testing
occurs later in childhood is most commonly due parameters [32]. For example, crossed or spi-
to trauma and tumors [24]. In the pediatric group raling isopters in Goldmann may look like gen-
most lesions involve the optic radiations, fol- eralized constriction in automated perimetry.
lowed by the occipital lobes, whereas in adults Correlation of the Amsler grid findings with the
the lesions usually damage the occipital lobes, perimetric results will also help to differentiate
followed by the optic radiations. In approxi- organic from nonorganic vision loss. Hence,
mately one third to one half of pediatric patients repeated perimetric testing with different
some spontaneous improvement can be expected methods and instruments may show discrepan-
within the first few months after lesion onset cies and diagnose a functional vision loss [33].
[26]. However, the recognition of homonymous The presence of the physiologic blind spot in
hemianopia frequently is delayed in this the temporal hemifield is also useful. In true
population. homonymous hemianopia, the physiologic blind
spot will be detectable in the eye with the tempo-
ral seeing hemifield during monocular perimetry
4.14 Functional Visual Loss [34]. In bitemporal hemianopia only the nasal
hemifields are preserved; hence the physiologic
Nonorganic hemianopic visual field defects are blind spots will not be detectable during mon-
occasionally encountered and may be psycho- ocular or binocular perimetry. In binasal hemi-
genic or caused by malingering. The predomi- anopia, which is very rare, only the temporal
nant pattern is the “missing half” hemianopia, hemifields are preserved; hence both blind spots
which is an ipsilateral hemianopia on the will be detectable in binocular perimetry [3].
“affected” eye, normal visual field in the fellow The blink response to a threatening gesture
eye, and a complete hemianopia toward the should be also absent in patients with true
affected side on binocular visual field. The sec- homonymous or heteronymous hemianopia,
ond most common pattern is ipsilateral blindness when the gesture is presented into the area of the
combined with hemianopia in the binocular visual field defect [7].
visual field. This type of defect can be diagnosed Additionally, in patients claiming homony-
by first performing visual field testing monocu- mous visual field loss, testing of reading ability
larly and then binocularly. The discrepancy will produce reading disabilities, particularly
between the monocular and binocular fields pro- when there is no macular sparing. Bitemporal
vides an immediate diagnosis of the functional and binasal hemianopias may present with
nature of this alleged visual loss [30] (Fig. 4.10). similar reading disturbances in monocular view-
Additionally, an organic monocular temporal ing. When tested binocularly, patients with
hemianopia has never been described without an bitemporal hemianopia may manifest the hemi-
RAPD [31]. field slide phenomenon with impaired reading
Other types of nonorganic visual field ability, which has been described in Sect. 4.6. In
defects include bitemporal hemianopia, binasal this case there are no corresponding parts of the
hemianopia, and a nonspecific concentric visual remaining seeing nasal visual fields and each
field constriction, which is the most common point in space is seen monocularly [34]. This
62 E. Papageorgiou and E. Tsironi-Malizou
LE RE LE RE
a b
Binocular Binocular
LE RE
c
Binocular
Fig. 4.10 (a) The most common pattern of hysterical visual field. (b) Another type of functional visual field loss
hemianopia is the “missing half” hemianopia. This is an is right eye blindness combined with right homonymous
ipsilateral hemianopia on the “affected” right eye, normal hemianopia in the binocular visual field. (c) Less often
visual field in the fellow eye, and a complete homonymous patients report bitemporal hemianopia combined with right
hemianopia toward the affected right side on binocular homonymous hemianopia in the binocular visual field
lack of motor control of binocular alignment the fixation point becomes invisible because it
leads to decompensation of underlying hetero- falls within a triangular postfixational scotoma
phorias and to horizontal separation (in preex- [10]. Finally, placement of a 20 prism diopters
isting esophoria), overlap (in preexisting base-out prism in front of one eye in true binasal
exophoria), and/or vertical displacement (in or bitemporal hemianopias will not induce any
preexisting hyperphoria) of the remaining nasal corrective movements, due to the lack of
hemifields. Postfixational blindness can be also fusional vergence eye movements. However, in
used to unmask a bitemporal hemianopia. When functional disease the prism will produce cor-
the patient with true bitemporal hemianopia fix- rective eye movements in order to eliminate the
ates a close target, a small object 30 cm behind diplopia [34].
4 Neuro-Ophthalmological Examination in Homonymous Visual Field Defects 63
Abstract
Retrochiasmal brain damage can lead to various types of homonymous visual
field defects (HVFDs), with the occipital lobe being the most common lesion
location, followed by the optic radiation, the optic tract, and lateral geniculate
nucleus (LGN). The etiology depends on the age of the patient. Older patients
tend to have vascular lesions, whereas younger patients may have tumors,
traumatic injuries, and arteriovenous malformations. HVFDs can be classified
according to their size, shape, macular sparing, and congruency, giving rise to
a variety of perimetric findings. Complete homonymous hemianopias are non-
localizing and may be caused by lesions in any part of the retrochiasmal visual
pathway, including the optic tract, LGN, optic radiation, and occipital lobe.
However, a highly congruous complete homonymous hemianopia is usually
due to an occipital lobe lesion. Homonymous quadrantanopias usually result
from lesions of the occipital lobe and the optic radiation. Less frequent types
of HVFDs are paracentral circumscribed homonymous scotomas due to small
lesions at the tip of the occipital lobe, homonymous sectoranopias in lesions of
the LGN, and peripheral HVFDs due to lesions in the intermediate striate
cortex. A unique HVFD is the “temporal crescent,” after damage to the most
anterior portion of the occipital lobe. Bilateral homonymous visual field
defects due to bilateral postchiasmatic lesions may present as checkerboard
visual fields or bilateral altitudinal defects. Such deficits may pose a diagnostic
dilemma, as they have to be differentiated from anterior ischemic optic neu-
ropathy, ischemic retinal lesions, choroiditis, choroidal colobomas, glaucoma,
optic nerve hypoplasia, tilted discs, or drusen.
Keywords
Homonymous hemianopia • Homonymous quadrantanopia • Horizontal
sectoranopia • Quadruple sectoranopia • Peripheral hemianopia • Paracentral
a Monocular Binocular
temporal visual
crescent field
Optic tract
Lateral
geniculate
nucleus (LGN)
Anterior
choroidal
artery
Calcarine
fissure (edges
retracted)
Fig. 5.1 (a) Projection of the visual fields onto the retina, the lateral posterior choroidal artery (hilum and midzone
lateral geniculate nucleus (LGN), and visual cortex. The of the LGN), and the anterior choroidal artery (lateral and
four quadrants of the visual fields send signals to the pri- medial aspects of the LGN). Depending on the lesion site,
mary visual cortices via the LGN. The superior visual partial infarctions of the right LGN produce a left horizon-
fields are represented below the calcarine fissure and the tal sectoranopia or a left quadruple sectoranopia. (b)
inferior visual fields are represented above the calcarine Anatomy of blood supply and representation of left visual
fissure. The right lateral geniculate nucleus is supplied by field in the right LGN seen in coronal section from behind
68 E. Papageorgiou and E. Tsironi-Malizou
in the retrochiasmal visual pathway, as there are shape, depth, and size in both eyes, while in
typical HVFDs associated with damage of cer- incongruous HVFDs the visual field is affected
tain brain areas (Fig. 5.2) However, this is not to a different extent in each eye. Due to the reti-
always possible, as studies have shown that every notopic organization of fibers within the visual
type of HH, except for unilateral loss of temporal pathways, the more posterior the location of a
crescent and homonymous sectoranopia, can be lesion to the visual pathway, the more congru-
found in all lesion locations along the retrochias- ous the visual field defect is likely to be [5].
mal visual pathway. Additionally, a complete Immediately posterior to the LGN, crossed and
homonymous hemianopia has no localizing uncrossed fibers corresponding to the same area
value, as it can occur with a lesion anywhere pos- of contralateral hemifield are spatially sepa-
terior to the optic chiasm [3]. In those cases the rated. As these fibers course posteriorly through
clinician should carefully seek for additional the optic radiation, they organize and they lie
localizing neurological signs and symptoms prior closer together as the optic radiations approach
to neuroimaging. The laterality of HVFD also the occipital lobe. Hence lesions involving the
provides information for neurological manifesta- occipital lobe characteristically produce con-
tions that may not have been obvious to the gruous HVFDs, whereas the most incongruous
patient. Right-sided HVFDs are associated with hemianopias occur with optic tract and LGN
reading or speech disorders, and left-sided lesions. However, this “rule of congruency”
HVFDs lead to visual hemineglect and difficul- should be used with caution, as at least 50% of
ties with spatial orientation and face and color lesions in locations other than the occipital lobe
recognition. also produce congruous homonymous visual
In complete homonymous hemianopia the field loss, especially if these lesions are stroke-
entire hemifield of both eyes is affected, while related. Interestingly, it has been shown that
in incomplete homonymous hemianopia there is 50% of optic tract lesions and 59% of optic radi-
sparing of a portion of the visual field in at least ation lesions produce congruous HVFDs, with
one eye (Fig. 5.3). Incomplete HVFDs can be the optic radiation being the most common loca-
further classified as either congruous or incon- tion for conditions resulting in incongruous
gruous. Congruous HVFDs have identical visual field defects [6].
Fig. 5.2 Sites of damage to the afferent visual pathway (5) Bitemporal hemianopia from central chiasmal
and corresponding visual field defects. Visual field loss in damage.
the monocular temporal crescent is associated with dam- (6) Right superior homonymous quadrantanopia from
age in the contralateral anterior occipital lobe, contralat- damage to the left temporal lobe (inferior portions of
eral anterior portion of the temporal lobe (Meyer loop), or the optic radiation).
the ipsilateral nasal retina. (7) Right inferior homonymous quadrantanopia from
(1) Left monocular temporal hemianopia due to a left damage to the left parietal lobe (superior portions of
preschiasmal lesion affecting the fibers from the the optic radiation).
nasal hemiretina. (8) Left complete homonymous hemianopia due to
(2) Left optic neuropathy. widespread damage of the right optic radiation in the
(3) Anterior junction syndrome due to damage of the vicinity of the occipital lobe.
anterolateral margin of the chiasm to the right side. (9) Right homonymous hemianopia with macular spar-
The lesion involves the anterior Wilbrand knee, ing from damage to the left occipital lobe.
which carries fibers from the contralateral inferior (10) Left (monocular) crescentic scotoma due to a lesion
nasal retina. of the right Meyer loop in the anterior temporal lobe.
(4) Right incomplete homonymous hemianopia with (11) Left (monocular) crescentic scotma due to a lesion
poor congruence due to a left optic tract lesion. of the right anterior (rostral) visual cortex
5 Types of Homonymous Visual Field Defects 69
Left Right
visual field visual field
4 3
6 5
10
Fibers from
superior
retina
11
a b c d
e f g h
Fig. 5.3 Various types of homonymous visual field defects rior cerebral artery (PCA). (d) Right homonymous superior
in the binocular 90° visual field (Octopus, semiautomated quadrantanopia due to ischemic infarction of the left PCA.
kinetic perimetry). (a) Left complete homonymous hemi- (e) Right incomplete homonymous superior quadrantanopia
anopia (HH) without macular sparing due to brain surgery due to left occipital infarction. (f) Left homonymous supe-
for a right parieto-occipital arteriovenous malformation rior paracentral scotoma due to infarction of the right occipi-
(AVM). (b) Left incomplete HH due to ischemic infarction tal pole. (g) Right homonymous inferior paracentral scotoma
in the parieto-occipital region. There is sparing of the due to infarction of the left occipital pole (binocular 30°
peripheral temporal crescent. (c) Right incomplete HH with kinetic perimetry). (h) Left incomplete HH without macular
macular sparing due to ischemic infarction of the left poste- sparing due to traumatic injury of the right occipital lobe
a
LA RA
LA RA
Meyer’s loop
b c
d
5 Types of Homonymous Visual Field Defects 73
Fig. 5.4 (continued)
Fig. 5.4 (a) Lateral view of the right visual pathway. left superior homonymous quadrantanopia presenting as
Information from the superior visual field (blue) is repre- “pie in the sky.” The defect in the contralateral (left) eye is
sented in the inferior retina and travels ventrally through slightly smaller and also appears to spare points near the
the temporal lobe. Damage to this site leads to superior vertical midline. (e) Axial CT of a 23-year-old female
quadrantanopia. Information from the inferior visual field with hemorrhage of an arteriovenous malformation
(red) is represented in the superior retina and travels dor- (AVM) involving the right occipital lobe. (f) One week
sally through the parietal lobe. Lesion to this site produces after AVM rupture there is a complete left homonymous
an inferior quadrantanopia. (b) Preoperative postcontrast hemianopia with macular sparing (30° TAP). (g) Six
coronal T1 magnetic resonance image (MRI) of a 50-year- months after AVM rupture there is partial recovery of the
old male with an astrocytoma involving the right temporal homonymous defect to a left inferior quadrantanopia
lobe. (c) Preoperative T2 axial MRI of the same patient. (Images 5.4b–g courtesy of Prof. Ulrich Schiefer,
(d) 30° Tuebingen automated perimetry (TAP) shows a Hochschule Aalen, Germany)
74 E. Papageorgiou and E. Tsironi-Malizou
Fig. 5.4 (continued)
the fibers subserving the macula are not located bances of memory, auditory agnosia, disturbance
anteriorly [5]. Damage of the Meyer loop within of hearing and sound discrimination, and auditory
the anterior temporal lobe is associated with a con- or formed visual hallucinations, which should
tralateral homonymous superior wedge- shaped prompt for a careful evaluation of the visual field.
visual field defect (“pie in the sky”) (Fig. 5.4b–d). A large area in the tip of the temporal lobe has
In temporal lobe lesions the superior no fibers of the Meyer loop. Studies on temporal
quadrantic defect is usually incongruous, incom- lobectomy have shown that up to 4 cm of the ante-
plete, denser above than below, and the inferior rior temporal lobe can be resected in most patients
margin of the defect may cross beyond the hori- without producing a field defect [3]. Hence tumors
zontal meridian. The incongruity is not as severe in this area have to be relatively large, in order to
as in optic tract lesions. Additionally, the defect produce a homonymous quadrantanopia. The
is usually but not always larger and denser and tumor initially may cause only subtle incomplete
also appears closer to the vertical midline in the defects in the superior visual field, but as it grows,
ipsilateral than in the contralateral eye [10]. the fibers of the Meyer loop are gradually affected,
Those findings were based on studies of patients and the field defect progresses from the vertical
with temporal lobe resection and support the down to the horizontal meridian in a stepwise fash-
hypothesis that certain fibers from the ipsilateral ion. With 5–7 cm resections most patients will have
eye travel more anteriorly and laterally in the a superior homonymous quadrantanopia, whereas
Meyer loop. However, there are also reports from with temporal lobectomies extending beyond 8 cm,
patients with temporal lobectomy, who showed almost all patients end up with a complete homon-
more congruous visual fields with no consistent ymous hemianopia [3]. Due to the anatomical het-
laterality regarding the severity, a finding that erogeneity of Meyer loop, diffusion tensor
reflects the individual anatomic disposition of the tractography of the optic radiations is currently
nerve fibers within the temporal lobe [11]. being used in order to assess an individual patient’s
Τemporal lobe lesions are usually tumors or in risk of postoperative visual field defect [12, 13].
some cases infections (abscesses). Congenital mal-
formations, hemorrhage, infarction, demyelinating Inferior Homonymous Quadrantanopia Inferior
disease, and trauma (lobectomy) are encountered homonymous quadrantanopia is encountered less
more rarely. In temporal lobe lesions, the patient frequently and is caused by damage to the optic
may suffer from additional neurologic symptoms, radiation in the superior parietal lobe or the visual
such as complex partial seizures, headache, distur- cortex superior to the calcarine fissure (Fig. 5.4e–g).
5 Types of Homonymous Visual Field Defects 75
Compressive lesions that affect only the superior neglect (usually left-sided neglect with lesions of
part of the optic tract are extremely rare (meningio- the right parietal lobe), visual agnosia, and are often
mas, cerebral tuberculomas, meningeal carcinoma- unaware of their visual field loss (anosognosia).
tosis). More common causes of an inferior Lesions of the dominant hemisphere can cause
homonymous quadrantanopia are ischemic infarcts aphasia, apraxia, agnosia, acalculia, and agraphia.
of the occipital lobe, or tumors (astrocytomas, oli-
godendrogliomas) and hemorrhages of the parietal
lobe. Traumatic injuries are also associated mainly 5.2.3 Homonymous Paracentral
with inferior homonymous quadrantanopias. The Scotomas
reason is that traumas of the inferior occipital lobe,
which would normally result in superior homony- A unilateral lesion at the tip of the occipital lobe
mous quadrantanopias, are often fatal due to their causes a small, circumscribed, paracentral homon-
high complication rate and location in the vicinity ymous hemianopic visual field defect. Such defects
of the cerebral sinuses [4]. are relatively rare. Even if their extent is limited to
Inferior homonymous quadrantanopias are usu- some degree only, they lead to severe impairment
ally more congruous than those produced by lesions of reading ability, which is often the reason for the
of the temporal lobe. The congruity of the field patient seeking medical advice [14]. Visual loss is
defects increases as the causative lesion approaches sudden and is not associated with other focal neu-
the posterior optic radiation and visual cortex, rological deficits. Paracentral homonymous hemi-
because the retinotopic organization improves and anopic scotomas are circumscribed, respect both
the fascicles of visual axons become more compact. the horizontal and the vertical meridian, and are
Hence lesions of the superior visual cortex or the located adjacent to the area of central fixation, usu-
termination of the optic radiation cause congruous ally within the central 10° (Fig. 5.5) [15]. They
inferior quadrantanopias respecting both the verti- may be perceived as “floaters,” obstructing the cen-
cal and horizontal meridians. In anteriorly located tral field of vision and leading to inability to read.
lesions, HVFDs tend to be more incongruous: the Patients with right-sided defects present with hemi-
defect may have sloping borders and cross the hori- anopic dyslexia, i.e., abnormally slow reading due
zontal meridian. The reason is that within the pari- to inability to see entire words and to find the fol-
etal lobe the fibers of the optic radiation are not yet lowing word. Patients with left-sided defects have
clearly separated into those serving the inferior difficulties in finding the next line. This condition
visual quadrant and those serving the superior has been described as “macular hemianopic read-
visual quadrant. ing disorder” by Wilbrand and Saenger [16].
However, in contrast to the progressive nature Distance activities, such as driving or watching
of superior homonymous quadrantanopias in television, are less affected by the scotoma because
temporal lobe tumors, the visual field in the infe- the peripheral visual field is intact. Visual acuity is
rior quadrant is lost as a unit in cases of compres- intact and the optic discs are normal, because the
sive parietal lesions, because the fibers of the lesion is located at the tip of the occipital lobe.
optic radiation are affected as a group within the Interestingly, the majority of patients complain
parietal lobe. Large lesions of the parietal lobe about glare, which is similar to the image which
may even result in complete homonymous hemi- persists after looking into the sun. Photophobia
anopias with macular splitting. has been attributed to the cortical origin of these
If the lesion is located in the parieto-occipito- scotomas, as patients with occipital strokes com-
temporal junction, then an abnormal optokinetic plain about similar symptoms [17, 18].
response is present. The optokinetic nystagmus In general, homonymous paracentral scotomas
(OKN) response is poorer when the OKN stimulus result from lesions located at the occipital pole;
is moved toward the affected hemisphere than when hence defects that are located immediately adjacent
it is moved in the opposite direction [5]. Lesions of to the central fixation point are usually congruous.
the parietal lobe are often accompanied by addi- However, there are some reports of incongruous
tional neurological symptoms. Patients may com- homonymous paracentral scotomas, which leads
plain of numbness and tingling, suffer from visual some authors to believe that if the scotomas are
76 E. Papageorgiou and E. Tsironi-Malizou
Fig. 5.5 (a) Axial T2 magnetic resonance image (MRI) of a T1 MRI of a 78-year-old male showing a lesion of the left
23-year-old female demonstrating resection of an arteriove- occipital pole due to an ischemic infarction of the posterior
nous malformation at the right occipital lobe. (b) Automated cerebral artery. (d) Automated 30° static perimetry of the
30° static perimetry of the above patient 1 year after the above patient 6 months post-stroke. A small, right homony-
operation. A small, left homonymous superior paracentral mous superior paracentral scotoma is shown in the central 10°
scotoma is shown in the central 10° (foreground). (c) Axial (foreground) (From Ganssauge et al. [18], with permission)
5 Types of Homonymous Visual Field Defects 77
Fig. 5.5 (continued)
78 E. Papageorgiou and E. Tsironi-Malizou
located further from the central fixation point, they a denomas, meningiomas, gliomas, and craniopha-
are not necessarily congruous. This fact may actu- ryngiomas) and aneurysms of the internal carotid
ally provide evidence that retinal correspondence artery, followed by cavernous angiomata, arterio-
declines as the distance from the fovea increases. venous malformations, demyelinating disease,
Cerebrovascular accidents, in particular isch- abscess, trauma, and neurosurgery (anterior tem-
emic strokes, are the most common cause of para- poral lobectomy, placement of intraventricular
central homonymous scotomas, followed by shunt) [1]. Congenital absence of the optic tract
traumatic brain injury. Tumors and demyelinating with contralateral homonymous hemianopia is a
lesions are rare causes of homonymous paracen- rare entity and has been attributed to either a pri-
tral scotomas [14]. A careful history, the age of mary failure of development or secondary atrophy
the patient, the onset (sudden or gradual), and the due to focal injury during the perinatal period [19].
presence of accompanying symptoms may help to Lesions of the optic tract cause a triad of charac-
differentiate the etiology and to request the appro- teristic clinical findings that often permit localiza-
priate neuroimaging and laboratory testing. tion of the lesion: homonymous hemianopia, a
Due to their very limited extent and central loca- relative afferent pupillary defect (RAPD) and “bow-
tion, paracentral homonymous hemianopic scoto- tie” optic atrophy, all contralateral to the brain
mas often go undetected during confrontation lesion. In unilateral optic tract lesions visual acuity
perimetry, and may also be misinterpreted as a non- is usually spared unless there is bilateral tract dam-
specific parafoveal depression or unstable fixation age or extension of the lesion to the optic chiasm or
on standard automated perimetry [14]. Amsler grid optic nerves. Optic tract lesion HVFDs are usually
testing is quick and effective in detecting such sub- partial and produce markedly incongruous HVFDs
tle paracentral scotomas, given that visual acuity is due to the poor topographic alignment of corre-
better than 20/40. If a small homonymous paracen- sponding retinal fibers from the two eyes [5].
tral scotoma is suspected, automated perimetry However, there are exceptions to this rule, because
should specifically assess the central visual field approximately 50% of optic tract lesions produce
with a spatially denser perimetric grid. congruous HVFDs [6]. Complete transaction of the
optic tract is less frequent than partial lesions, and
produces a congruous complete hemianopia.
5.2.4 Homonymous Hemianopia Retrograde degeneration of the axons in the
optic tract causes a distinct pattern of optic atro-
Complete homonymous hemianopias may be phy over time, which is evident in both eyes. The
caused by lesions in any part of the retrochiasmal optic disc contralateral to the brain lesion demon-
visual pathway, including the optic tract, LGN, strates “bow-tie” atrophy in the nasal and tempo-
optic radiation, and occipital lobe. Hence a com- ral sectors, while the optic disc ipsilateral to the
plete homonymous hemianopia is nonlocalizing, brain lesion has temporal disc pallor [20]. This
and the examiner should seek for other signs of neu- pattern of optic atrophy can be explained by the
rological dysfunction prior to obtaining neuroimag- underlying organization of the retinal ganglion
ing studies. Most lesions of the optic radiations and cell layers: The affected optic tract contains
occipital lobe are associated with vascular disease, crossed fibers from the contralateral nasal retina
while most optic tract lesions are due to compres- (nasal disc) and nasal half of the macula (tempo-
sive masses. Additionally, HVFDs due to optic tract ral disc in the papillomacular bundle), and
lesions tend to be incongruous, whereas lesions of uncrossed fibers from the ipsilateral superior and
the optic radiations cause only mild incongruity and inferior sectors that correspond to the temporal
occipital lobe lesions are highly congruous. retina (temporal disc).
Lesions of the Optic Tract Etiologies of optic Lesions of the Optic Radiation The type of
tract lesions are similar to those of chiasmal homonymous visual field loss in lesions of the
lesions, and combined damage to the optic tracts, optic radiation reflects the site of damage. The
chiasm, and optic nerve is not uncommon. The optic radiation can be affected in various loca-
most frequent causes are tumors (pituitary tions during its course and produce characteristic
5 Types of Homonymous Visual Field Defects 79
HVFDs. Large lesions of the parietal lobe pro- congruous homonymous hemianopias. The most
duce complete homonymous hemianopias with common causes of unilateral occipital lobe damage
macular splitting, because the entire optic radia- are vascular and traumatic, followed by arteriove-
tion travels into the deep white matter of the pari- nous malformations, infections, tumors, and demy-
etal lobe. Mildly incongruous, incomplete elinating disease. The patient usually exhibits sudden
homonymous hemianopias, denser below than visual loss and sometimes headache. If there is
above, may also occur in those cases. However, involvement of the entire striate cortex, then a macu-
the HVFDs are more congruous than those lar splitting homonymous hemianopia is present.
observed in optic tract lesions. A complete hom- However, macular splitting homonymous hemiano-
onymous hemianopia can also be caused by large pias arise from lesions anywhere in the visual path-
lesions of the optic radiation close to its termina- way; hence they do not have localizing value.
tion in the striate cortex. On the other hand, par- The macular area has a disproportionally large
tial lesions of the dorsal fibers in the parietal lobe representation in the visual cortex retinotopic map,
produce a contralateral inferior homonymous which is called cortical magnification (Fig. 5.6).
quadrantanopia, and lesions of the ventral fibers Approximately 50–60% of the visual cortex repre-
in the anterior temporal lobe cause a contralateral sents the central 10°, and about 80% of the visual
superior homonymous quadrantanopia, as cortex represents the central 30° [24]. This cortical
described above (see Fig. 5.4). However, with magnification offers an extremely high central
damage extending beyond 8 cm from the anterior acuity (spatial resolution of 60–100 cycles/
temporal tip, almost all patients will have a com- degree), which declines towards the visual field
plete homonymous hemianopia, because both periphery. A macular sparing hemianopia is one
upper and lower optic radiations are affected. that spares an area of at least 3° of radius from
Until recently it was thought that lesions of the fixation [5]. Macular sparing, which is considered
retrogenicate pathway do not lead to optic atrophy characteristic for occipital lobe lesions, is most
(with the exception of some congenital lesions) or likely due to the large macular representation and
pupillary defects. However, recent optical coher- the collateral blood supply from the middle cere-
ence tomography advances have demonstrated that bral artery, which protects the macular region from
subtle retinal nerve fiber layer loss also occurs in ischemia [4]. Strokes tend to produce macular
retrogeniculate lesions, even if it is not visible clini- sparing much more commonly than other causes,
cally [21–23]. Unlike lesions of the optic tract, the such as tumors or trauma. In contrast, more ante-
most common causes of optic radiation damage are rior lesions, for example in the optic radiation, pro-
infarcts in the posterior cerebral or middle cerebral duce macular splitting, because there is no
artery territories, followed by tumors, arteriove- collateral circulation. In general, a patient with a
nous malformations, and infections. macular sparing homonymous hemianopia most
likely has a lesion in the occipital lobe, and the
Lesions of the Occipital Lobe In the striate cor- cause is almost always a posterior cerebral artery
tex there is strict retinotopic organization of the infarction due to cardiac emboli or vertebrobasilar
fibers subserving the various portions of the occlusive disease [4].
visual field. The macula is represented at the An occipital lesion can be restricted to either
posterior pole of the occipital lobe, and the far the upper or lower bank of the striate cortex,
peripheral visual field at the anterior portion of producing a highly congruous homonymous
the visual cortex, on the medial occipital surface inferior or superior quadrantanopia, respectively.
[24, 25]. The most anterior bank of striate cortex Isolated damage to the occipital lobe usually does
(8–10%) represents the monocular temporal cres- not produce other neurologic manifestations, and
cent (temporal 30° of the contralateral eye). This the patient is fully or partially aware of the hemi-
area receives input only from the contralateral anopia, unless the lesion is more extensive or
eye, since the nasal hemifield of the ipsilateral involves more anterior structures in the parietal
eye does not extend beyond 60°. lobe. In such cases there is anosognosia for the
Occipital lesions affecting both the upper and visual field loss, especially if the lesion is located
lower banks of the striate cortex cause highly in the right cerebral hemisphere.
80 E. Papageorgiou and E. Tsironi-Malizou
50
2.5
180
5
10
Fovea 20 40
10 20
2.5 5
40
315
270
90
45
50
2.5
180
5
10
20
40
315
270
Fig. 5.6 Representation of the right visual field in the left (b) Corresponding right (nasal) hemifield of left eye. (c)
visual cortex. (a) Retinotopic organization of the left Right (temporal) hemifield of right eye. The monocular
visual cortex. There is disproportionate representation of temporal crescent is the most peripheral part of the visual
the central visual field, termed “cortical magnification.” field (gray)
5 Types of Homonymous Visual Field Defects 81
5.2.5 Peripheral Homonymous temporal crescent, are extremely rare (Fig. 5.7).
Visual Field Defects Peripheral HVFDs commonly result from lesions
in the intermediate area of the striate cortex,
HVFDs typically involve the central 10° of between the anterior and posterior confines, and
vision. Peripheral homonymous scotomas, which spare the most anterior portion of the striate cor-
extend beyond 30° from fixation, but spare the tex and the occipital pole [26].
a b
c
Fig. 5.7 (a) A 39-year-old female with left inferior hom- the most anterior part of the visual cortex. (c) A 54-year-
onymous peripheral scotoma, extending between 30° and old male with right homonymous peripheral scotomas
50° along the horizontal meridian (90° binocular semiau- extending between 30° and 60° are present in both eyes
tomated kinetic perimetry). (b) Axial fluid-attenuated (90° static perimetry). (d) Axial FLAIR MRI of the above
inversion recovery (FLAIR) magnetic resonance image patient shows ischemia of the left occipital cortex with
(MRI) of the above patient shows ischemic infarction of sparing of the occipital pole and anterior occipital lobe
the right occipital cortex that spares the occipital pole and (From Papageorgiou et al. [27], with permission)
82 E. Papageorgiou and E. Tsironi-Malizou
a
b c
Fig. 5.8 (a) Octopus monocular 30° static automated sparing of the temporal crescent beyond the peripheral
perimetry of a 53-year-old male shows a left homony- 60°. (c) Axial CT demonstrates ischemic infarction of the
mous hemianopia with macular sparing. (b) Octopus bin- right occipital lobe with sparing of its most anterior
ocular 90° semiautomated kinetic perimetry (SKP) reveals portion
is posterior cerebral artery infarction that can inflammation, or infection affecting both striate
affect both striate cortices, either simultaneously or cortices concurrently.
sequentially, due to the common origin of the right If both occipital lobes are simultaneously
and left posterior cerebral arteries from the basilar affected, there is complete vision loss, i.e., cere-
artery. It has been estimated that 16% of patients bral blindness, which is usually transient, lasting
with a unilateral occipital infarction develop bilat- from minutes to days, and may recover to a variable
eral infarction over 6 months [32]. Other causes of degree of bilateral incomplete HVFDs with mac-
bilateral HVFDs are occipital tumors, trauma, ular sparing.
84 E. Papageorgiou and E. Tsironi-Malizou
a c
b d
Fig. 5.9 (a) Humphrey 24–2 static automated perimetry and occipital cortex and an old left PCA territory infarct.
of a 57-year-old male demonstrating bilateral superior (c) Humphrey 24–2 static automated perimetry of an
homonymous quadrantanopia, which resembles superior 83-year-old female showing bilateral homonymous visual
altitudinal visual field defects. (b) Axial fluid-attenuated field defects, i.e., a left incomplete homonymous hemi-
inversion recovery magnetic resonance image (MRI) anopia and a right homonymous horizontal sectoranopia
showing bilateral lesions of the inferior occipital lobe. with macular sparing. (d) Axial T2 MRI demonstrates an
There is a subacute right posterior cerebral artery (PCA) acute infarct in the right thalamus and an old left PCA
territory infarct involving the right posterior temporal lobe infarct
Much more commonly, bilateral HVFDs are visual pathway are not always aware of the
associated with consecutive vascular infarctions accompanying HVFD until they suffer a second
of the posterior cerebral circulation. Except for event in the contralateral hemisphere weeks to
the visual symptoms, occipital lobe lesions are years later, causing a more pronounced visual
generally not associated with other neurological impairment.
problems; hence patients do not always present Bilateral occipital lobe lesions either from a
in the acute setting. In such cases it may be dif- single or from consecutive events may lead to
ficult to determine the exact time of onset of complete bilateral homonymous hemianopia
bilateral ischemic lesions and to decide if they with no macular sparing, which is called cor-
were due to single or consecutive events [4]. tical or cerebral blindness. More often, how-
Patients with unilateral brain lesions of the ever, there is some degree of macular sparing
5 Types of Homonymous Visual Field Defects 85
giving rise to “keyhole” visual fields [10]. The common tumors of the occipital lobe leading to
degree of m acular sparing may be different on bilateral superior or inferior altitudinal HVFDs
each side, and reading ability depends on the are meningiomas.
residual central visual field. Affected individu- Bilateral HVFDs, especially in the form of
als are severely handicapped in their everyday incomplete hemianopias with macular sparing,
life, as they suffer from “tunnel vision” [5]. central scotomas, ring scotomas, or altitudinal
Occasionally bilateral paracentral HVFDs with defects, must be distinguished from bilateral optic
macular sparing and intact periphery present nerve or retinal disease. For example, bilateral
as “ring” scotomas. In rare instances, bilateral superior or inferior altitudinal defects may occur
HVFDs affect only the central area with preser- due to bilateral retinal or optic nerve damage, such
vation of the peripheral visual field, and manifest as anterior ischemic optic neuropathy, ischemic
as bilateral central scotomas that may resemble retinal lesions, choroiditis, choroidal colobomas,
ocular disease [4]. The presence of a vertical glaucoma, optic nerve hypoplasia, tilted discs, dru-
step on careful perimetric testing differentiates sen, and intracranial masses affecting the chiasm or
these defects from a true central scotoma due both optic nerves. Apart from the pathological fun-
to retinal or optic nerve disease. Patients with dus findings, careful observation of the perimetric
bilateral central HVFDs do not have mobility results in the above cases reveals that the pattern of
problems, because the visual field periphery is visual field loss is usually asymmetric and not
preserved. However, they have difficulties with homonymous, i.e., does not respect the vertical
reading, face recognition, and other activities meridian. True bilateral HVFDs respect the vertical
that require intact central vision. meridian; there are normal pupillary, oculomotor,
Crossed homonymous quadrantanopias and funduscopic findings, and visual acuity is usu-
(“checkerboard” visual fields) present in lesions ally normal or decreased symmetrically [10].
of the superior occipital lobe (above the calcarine
fissure) on one side, and the inferior occipital Cerebral Blindness Cerebral blindness is a
lobe (below the calcarine fissure) on the opposite general term that refers to bilateral severe vision
side [33]. Such defects are usually the result of loss from damage posterior to the lateral genicu-
sequential rather than simultaneous infarctions. late nuclei. Cerebral blindness can be persistent
In some instances, checkerboard visual fields or transient. The most common cause of persis-
occur after recovery from a single vascular event tent cerebral blindness is infarction due to
associated initially with cerebral blindness. embolic or thrombotic events. Transient cerebral
Other patterns of visual field loss in bilateral blindness can last hours to days and often
occipital disease include bilateral altitudinal recovers fully.
defects, i.e., bilateral superior or inferior homony- There are many causes of cerebral blindness,
mous quadrantanopias, which occur when such as vertebrobasilar arteritis, subclavian steal,
bilateral brain lesions are located below or above hypoxia, hemorrhage, generalized hypotension
the calcarine fissure respectively (see Fig. 5.9a). from antihypertensive medications such as nife-
Ischemic events of the posterior cerebral artery dipine, and rupture of occipital mycotic aneu-
tend to produce mainly bilateral superior altitudi- rysms with endocarditis. Cerebral blindness can
nal hemianopias. On the other hand, bilateral infe- also complicate cardiac surgery, due to hemody-
rior altitudinal defects usually result from tumors namic compromise or emboli. Causes of transient
and traumas, such as bullet wounds. As mentioned cerebral blindness include seizures (ictal or
above, the superior occipital lobe is more vulner- postictal), migraine, posterior reversible enceph-
able to traumatic injuries. Traumatic lesions of the alopathy syndrome, demyelinating disease,
inferior occipital lobe are often fatal due to their metabolic insults (hepatic encephalopathy, hypo-
location in the vicinity of the cerebral sinuses and glycemia, alcoholic ketoacidosis, adrenoleuko-
the resulting hemorrhage [4]. Finally, the most dystrophy, and acute intermittent porphyria),
86 E. Papageorgiou and E. Tsironi-Malizou
Visual fields
Left Right
Retina
1
2
3%
4%
5
3
0.6%
13% !
11%
4
22%
? ?
19% 6% 1% 19%
Fig. 5.10 (a) Projection of the visual fields onto the retina (4) Bitemporal hemianopia from central chiasmal dam-
and course of the retinal ganglion cell axons in the region age. (5) Posterior junction syndrome due to damage to the
of the chiasm. Sites of damage and corresponding visual posterior knee of Wilbrand, which carries signals from the
field defects with their frequency of occurrence are shown ipsilateral superior nasal retina that correspond to the infe-
(modified from Schiefer et al. [5] and Schiefer et al. [47]). rior temporal quadrant of the visual field. This ipsilateral
(1) Compressive optic neuropathies. (2) Partial prechias- inferior temporal scotoma is combined with a contralat-
mal lesion affecting the fibers, which carry signals from eral homonymous hemianopia. (6) Optic tract lesion. (b)
the nasal hemiretina and correspond to the temporal hemi- Coronal section of the sellar and parasellar regions sur-
field. (3) Anterior junction syndrome due to damage of the rounding the pituitary gland. (c) Sagittal section of the
ipsilateral optic nerve and the anterior Wilbrand knee. chiasmal region. (d) Bitemporal hemianopia from central
Fibers from the contralateral inferior nasal retina that chiasmal compression, i.e., pituitary adenoma. (e)
extend anteriorly into the affected ipsilateral optic nerve Anterior junction syndrome from compression at the level
form the so-called anterior Wilbrand knee, and corre- of the junction between the left optic nerve and chiasm
spond to the superior temporal quadrant of the visual field.
5 Types of Homonymous Visual Field Defects 89
Diaphragma
sellae
Third nerve
Fifth nerve
(second division)
Sphenoid sinus
m
Suprasellar m
8
cistern
Optic chiasm
4 mm
m
m
15 Infundibular
Anterior clinoid
10 mm recess
process
ON
45°
Diaphragma
sellae
Optic nerve
Pituitary
(posterior lobe)
Optic canal
Sphenoid
sinuses
Fig. 5.10 (continued)
90 E. Papageorgiou and E. Tsironi-Malizou
d Bitemporal hemianopia
e Anterior junction syndrome
LE RE LE RE
Chiasma
Lesion
Chiasma
Optic tract
Optic tract
Fig. 5.10 (continued)
5.3.2 Anterior Junction Syndrome defect in the superior temporal field. The fibers
from the contralateral eye (anterior Wilbrand
The anterior junction syndrome refers to severe knee), resulting in a contralateral defect in the
visual loss manifesting as advanced central sco- superior temporal field.
toma in one eye, and a temporal defect (initially
of the upper quadrant) in the healthier fellow
eye (Fig. 5.11). This pattern of hemianopic 5.3.3 Binasal Hemianopia
visual field loss will be evident when perimetry
is performed for both eyes, and careful analysis Binasal visual field defects usually represent
will reveal that the temporal deficit in the health- nerve fiber bundle defects in cases of bilateral
ier eye respects the vertical meridian [47]. It is glaucomatous neuropathy. Genuine binasal hemi-
usually caused by lesions located at the antero- anopia is a rare entity and may result from
lateral margin of the chiasm, such as meningio- bilateral compression of the lateral aspect of the
mas or supraclinoid aneurysms. The lesion chiasm, i.e., due to bilateral intracavernous
involves the ipsilateral optic nerve producing an carotid aneurysms or calcified carotid arteries.
ipsilateral extensive field defect or total blind- Ocular causes, such as bilateral retinoschisis and
ness, but also damages the crossed ventral fibers papilledema, have been rarely described [5].
that originate from the inferonasal retina of the Chiasmal processes are often accompanied
contralateral eye, resulting in a contralateral by early red desaturation within the temporal
5 Types of Homonymous Visual Field Defects 91
hemifields, reduction in visual acuity, and optic decompression is guarded in cases with disc pal-
atrophy of one or both eyes. The extent of optic lor. Papilledema is an uncommon presentation in
atrophy does not correlate well with visual acu- chiasmal disease unless a large mass compresses
ity loss. However, the prognosis for recovery of the third ventricle from below and obstructs the
visual field and chiasmal function after surgical foramina of Monro.
a b
Fig. 5.11 (a) Coronal T1 magnetic resonance image MRI hemianopia of the right eye due to compression at the
of a 30-year-old male demonstrates a pituitary adenoma, junction between the left optic nerve and the anterior chi-
with elevation and compression of the optic chiasm mainly asm. (d) Coronal T2 MRI after transnasal transsphenoidal
to the left side. (b) Axial fluid-attenuated inversion recover resection of the prolactinoma. (e) Postoperative sagittal T2
MRI of the above patient (sagittal view unavailable). MRI. (f) Perimetry 1 week after the operation shows par-
(c) The patient has an anterior junction syndrome with tial recovery of the scotoma of the right eye to a superior
advanced central scotoma of the left eye and temporal temporal quadrantic scotoma
92 E. Papageorgiou and E. Tsironi-Malizou
d e
Fig. 5.11 (continued)
10. Brazis PW, Masdeu JC, Biller J. Localization in clini- nance imaging and perimetric correlation. Arch
cal neurology. 6th ed. Philadelphia/Boston: Lippincott Ophthalmol. 1999;117(2):208–17.
Williams & Wilkins; 2011. 26. Mejico LJ, Bergloeff J, Miller NR. Peripheral hom-
11. Taoka T, Sakamoto M, Nakagawa H, Nakase H,
onymous scotomas from a cavernous angioma affect-
Iwasaki S, Takayama K, et al. Diffusion tensor trac- ing fibers subserving the intermediate region of the
tography of the Meyer loop in cases of temporal lobe striate cortex. Am J Ophthalmol. 2001;132(3):440–3.
resection for temporal lobe epilepsy: correlation 27. Papageorgiou E, Ticini LF, Schiefer U. Peripheral
between postsurgical visual field defect and anterior homonymous hemianopia: correlation between lesion
limit of Meyer loop on tractography. AJNR Am location and visual field defects by means of cytoar-
J Neuroradiol. 2008;29(7):1329–34. chitectonic probabilistic maps. J Neuroophthalmol.
12. Barton JJ, Hefter R, Chang B, Schomer D, Drislane 2012;32(1):5–12.
F. The field defects of anterior temporal lobectomy: a 28. Chavis PS, al-Hazmi A, Clunie D, Hoyt WF. Temporal
quantitative reassessment of Meyer’s loop. Brain. crescent syndrome with magnetic resonance correla-
2005;128(Pt 9):2123–33. tion. J Neuroophthalmol. 1997;17(3):151–5.
13. Borius PY, Roux FE, Valton L, Sol JC, Lotterie JA, Berry 29. Lepore FE. The preserved temporal crescent: the clin-
I. Can DTI fiber tracking of the optic radiations predict ical implications of an “endangered” finding.
visual deficit after surgery? Clin Neurol Neurosurg. Neurology. 2001;57(10):1918–21.
2014;122:87–91. 30. Walsh TJ. Temporal crescent or half-moon syndrome.
14. Kölmel HW. Homonymous paracentral scotomas.
Ann Ophthalmol. 1974;6(5):501–5.
J Neurol. 1987;235(1):22–5. 31. Wein F, Miller NR. An unusual homonymous visual
15. Mavrakanas NA, Dang-Burgener NP, Lorincz EN,
field defect. Surv Ophthalmol. 2000;44(4):324–8.
Landis T, Safran AB. Perceptual distortion in hom- 32. Kumral E, Bayulkem G, Ataç C, Alper Y. Spectrum of
onymous paracentral scotomas. J Neuroophthalmol. superficial posterior cerebral artery territory infarcts.
2009;29(1):37–42. Eur J Neurol. 2004;11(4):237–46.
16. Wilbrand H, Saenger A. Die Neurologie des Auges. 33. Cross SA, Smith JL. Crossed-quadrant homonymous
Wiesbaden: J Bergmann; 1904. p. 98–120. hemianopsia. The "checkerboard" field defect. J Clin
17. Safran AB, Achard O, Duret F, Landis T. The “thin Neuroophthalmol. 1982;2(3):149–58.
man” phenomenon: a sign of cortical plasticity fol- 34. Glaser JS. Neuro-ophthalmology. 3rd ed. Philadelphia:
lowing inferior homonymous paracentral scotomas. Lippincott Williams & Wilkins; 1999.
Br J Ophthalmol. 1999;83(2):137–42. 35. Aldrich M, Alessi A, Beck R, Gilman S. Cortical
18. Ganssauge M, Papageorgiou E, Schiefer U. Facial blindness: etiology, diagnosis and prognosis. Ann
dysmorphopsia: a notable variant of the “thin man” Neurol. 1987;21(2):149–58.
phenomenon? Graefes Arch Clin Exp Ophthalmol. 36. Lee AG, Martin C. Neuro-ophthalmic findings in the
2012;250(10):1491–7. visual variant of Alzheimer’s disease. Ophthalmology.
19. Margo CE, Hamed LM, McCarty J. Congenital optic 2004;111(2):376–80.. discussion 81
tract syndrome. Arch Ophthalmol. 1991;109(8): 37. McDaniel K, McDaniel L. Anton’s syndrome in a
1120–2. patient with posttraumatic optic neuropathy and
20. Newman SA, Miller NR. Optic tract syndrome. Neuro- bifrontal contusions. Arch Neurol. 1991;48(1):101–5.
ophthalmologic considerations. Arch Ophthalmol. 38. Hershenfeld SA, Sharpe JA. Monocular temporal
1983;101(8):1241–50. hemianopia. Br J Ophthalmol. 1993;77(7):424–7.
21. Goto K, Miki A, Yamashita T, Araki S, Takizawa G, 39. Smolyar A, Eggenberger ER, Kaufman DI. Monocular
Nakagawa M, et al. Sectoral analysis of the retinal temporal hemianopia associated with optic nerve
nerve fiber layer thinning and its association with hypoplasia. Arch Ophthalmol. 2005;123(8):1155.
visual field loss in homonymous hemianopia caused 40. Stacy RC, Jakobiec FA, Lessell S, Cestari DM.
by post-geniculate lesions using spectral-domain opti- Monocular nasal hemianopia from atypical sphenoid
cal coherence tomography. Graefes Arch Clin Exp wing meningioma. J Neuroophthalmol. 2010;30(2):
Ophthalmol. 2016;254(4):745–56. 160–3.
22. Jindahra P, Petrie A, Plant GT. Retrograde trans- 41. Rahman I, Nambiar A, Spencer AF. Unilateral nasal
synaptic retinal ganglion cell loss identified by optical hemianopsia secondary to posterior subcapsular cata-
coherence tomography. Brain. 2009;132(Pt 3):628–34. ract. Br J Ophthalmol. 2003;87(8):1045–6.
23. Cowey A, Alexander I, Stoerig P. Transneuronal retro- 42. Cox TA, Corbett JJ, Thompson HS, Kassell NF.
grade degeneration of retinal ganglion cells and optic Unilateral nasal hemianopia as a sign of intracranial
tract in hemianopic monkeys and humans. Brain. optic nerve compression. Am J Ophthalmol. 1981;92(2):
2011;134(Pt 7):2149–57. 230–2.
24. Horton JC, Hoyt WF. The representation of the visual 43. Manor RS, Ouaknine GE, Matz S, Shalit MN. Nasal
field in human striate cortex. A revision of the classic visual field loss with intracranial lesions of the optic
Holmes map. Arch Ophthalmol. 1991;109(6): nerve pathways. Am J Ophthalmol. 1980;90(1):1–10.
816–24. 44. Peiris JB, Ross Russell RW. Giant aneurysms of the
25. Wong AM, Sharpe JA. Representation of the visual carotid system presenting as visual field defect. J Neurol
field in the human occipital cortex: a magnetic reso- Neurosurg Psychiatry. 1980;43(12):1053–64.
94 E. Papageorgiou and E. Tsironi-Malizou
45. Gittinger Jr JW. Functional monocular temporal hemi- 47. Schiefer U, Isbert M, Mikolaschek E, Mildenberger I,
anopsia. Am J Ophthalmol. 1986;101(2):226–31. Krapp E, Schiller J, et al. Distribution of scotoma pat-
46. Humphrey PR, Moseley IF, Russell RW. Visual field tern related to chiasmal lesions with special reference
defects in obstructive hydrocephalus. J Neurol to anterior junction syndrome. Graefes Arch Clin Exp
Neurosurg Psychiatry. 1982;45(7):591–7. Ophthalmol. 2004;242(6):468–77.
Novel Imaging Techniques
and Neuroradiologic Imaging 6
Njoud Aldusary, Birgit Hartog-Keisker,
and Spyros Kollias
Abstract
The opportunity to apply various neuroimaging techniques to study the
visual system has had a strong impact on the clinical assessment and man-
agement of visual pathologies. Advancements in the technology and the
methodology of neuroimaging techniques, such as functional magnetic
resonance imaging (fMRI) and diffusion tensor imaging (DTI), allow
obtaining noninvasive structural, functional, and physiological informa-
tion on the visual pathway. FMRI and DTI allow the noninvasive investi-
gation of cognitive processes (fMRI), provide models of brain connectivity,
and reveal abnormalities in white matter fiber structure (DTI). Combining
these techniques with standard ophthalmology tests provides the clinicians
with a powerful tool for the assessment and management of patients with
visual field defects such as hemianopia.
Keywords
Magnet resonance imaging • Functional magnetic resonance imaging •
Diffusion tensor imaging • MRI • FMRI • DTI • Visual field defect •
Homonymous • Hemianopia
6.1 Introduction
whole brain T2 and T1 weighted IMAGES, sequence in a patient with 10-day history of a
without and with contrast administration, depend- shadow in her left visual field while CT and rou-
ing on the symptoms of the patient and the exact tine T2-weighted MRI failed to demonstrate the
clinical indication. Diffusion-weighted imaging lesion. The available MRI sequences constantly
is indispensable for the exclusion of vascular improve; a successful diagnosis relies on the neu-
(ischemic) lesions at the level of optic tracts or roradiological expertise to choose the most sensi-
optic radiations. If necessary, perfusion imaging, tive ones that will better demonstrate the
spectroscopic imaging, and angiographic imag- pathology, depending on the clinical symptoms of
ing will be required to further characterize the the patient [9]. In summary, considering the
type of pathology (i.e., vascular, infectious, above-mentioned aspects, MRI is the method of
tumoral, etc.) for establishing a more precise and choice in the evaluation of patients with homony-
accurate diagnosis [8]. mous hemianopia.
In patients suffering from homonymous visual
defects due to tumoral or vascular lesions, the
focus of the MRI investigation should be on the 6.3 MRI–DTI Tractography
chiasm and retrochiasmal visual pathways. In the
case of hemorrhagic stroke along the retrochias- Since the communication network of the brain
mal visual pathway in the setting of a head consist of white matter fiber pathways, any alter-
trauma, a noncontrast rapid and low cost CT, ation of these pathways will affect the signal
available in any emergency room, is suitable. transmission between brain regions and thus
However, this protocol is sufficient neither to influence functional performance. Diffusion ten-
detect nonhemorrhagic ischemia nor to identify sor imaging (DTI) is a MR method allowing non-
other lesions like tumors, demyelinating plaques, invasive mapping of large white matter tracts in
and infections. In case of such incidents, MRI is the human brain in vivo and investigation of
far more sensitive and accurate to detect the white matter axonal integrity in a quantitative
pathology at an early stage [9]. manner. It is based on the directional diffusion
A careful and targeted MRI assessment should properties of microscopic water molecules in tis-
be considered in patients with symptoms of hom- sues. The mammalian white matter is a highly
onymous visual field defects because other neu- anisotropic structure. Barriers to free water diffu-
rological conditions (e.g., dementias, metabolic sion include, among others, the myelin sheath
disorders, etc.) may mimic stroke and cause and the cell membrane, which facilitate diffusion
visual field defects. Vachalová et al. described a of the water molecules along the long axis of the
patient with the Heidenhain variant of fiber than perpendicular to its axis. The three-
Creutzfeldt–Jakob disease who was diagnosed dimensional direction and speed of maximal dif-
with symptoms mimicking acute ischemic stroke fusivity along axonal fibers in the white matter
presenting with acute inferior homonymous qua- can be estimated for each voxel of the imaged
drantanopia. Four days after symptom onset, volume.
MRI revealed a medial occipital cortex ribbon- Quantitative parameters of DTI include first,
like high signal intensity and restricted diffusion the fractional anisotropy (FA) index measuring
typical for this neurodegenerative disease [10]. the degree of anisotropic diffusion, ranging
Exact knowledge of the various MRI sequences between 1 for maximum anisotropy, along one
and their different contrasts between tissues is direction and 0 for isotropic diffusion, equal in
pivotal to achieve correct clinical diagnoses. all directions. The second quantitative parame-
Horton reported on a successful diagnosis of ter is the apparent diffusion coefficient (ADC)
infarction in the lower right calcarine sulcus using index, measuring the degree of diffusivity along
fluid-attenuated inversion recovery (FLAIR) each direction. Both measurements are used as
98 N. Aldusary et al.
an expression of structural integrity of the tis- LGN and in the subcortical white matter of the
sue structure [11]. For example, reduced diffu- occipital visual areas. For the reconstruction of
sivity of water molecules is observed when the individual fiber bundles that compose the optic
integrity of the myelin sheaths in the white mat- radiations, ROIs should be placed along the para-
ter is affected, such as in demyelinating dis- ventricular white matter in a more ventral loca-
eases, infiltration by a tumor, degenerative tion to reconstruct Meyer’s loop, in a more central
processes etc [12]. location for the central bundle, and more dorsal
Diffusion information can be represented in for the dorsal bundle [13]. It has to be noted that
the two-dimensional space as color-coded direc- individual selection and positioning of the seed
tionality images (FA maps), or three-dimensional ROI’s may result in a certain variability in the
reconstructions of specific white matter fiber reconstruction of the fibers [12].
tracts using diffusion tensor tractography (DTT) DT imaging has become an important tool for
algorithms (Fig. 6.1) [11]. To start the tracking visualization of the macroscopic fiber tract archi-
process and create 3D reconstructions of the tecture of the visual pathway in 3D [14].
visual pathway from the DTI data, an appropriate Compared to conventional imaging methods,
placement of regions of interest (ROIs) is required which do not demonstrate the anatomy of the
to selectively isolate specific white matter fiber posterior visual pathway effectively and cannot
systems that make part of the pathway. These reveal microstructural changes in early stages of
ROIs are selected based on anatomical landmarks pathology, DTI provides the possibility to study
from high-resolution MR images of the same the integrity of the entire visual pathway in
subject. For example, the ROI for starting the greater detail. The ability of DTI to visualize
tracking of the optic tract should be placed over white matter tracts, which connect different parts
the chiasm with the end point in the lateral genic- of the neural network in the brain, found an early
ulate nucleus (LGN). The optic radiation recon- application in presurgical planning and intraop-
structions are performed by placing ROIs in the erative guidance to avoid or minimize damage
a b
Fig. 6.1 (a) 2D diffusion tensor imaging (DTI) color- using the Fiber Assignment by Continuous Tracking
coded according to the orientation in the 3D space of the (FACT) algorithm. Based on the localization and the spe-
primary eigenvector within each voxel of the image (red cific main orientation, we can virtually reconstruct indi-
for left-right, green for anterior-posterior, and blue for vidual fiber systems of the white matter in great detail
superior-inferior). (b) 3D reconstruction of fiber systems (From Kollias et al. [11], with permission)
6 Novel Imaging Techniques and Neuroradiologic Imaging 99
during surgical intervention, i.e., removal of a injury. The patient had right homonymous hemi-
tumor in the vicinity of the visual pathway [15]. anopia following the brain trauma, which per-
DTI is also used in the clinical investigation of sisted for 6 months following the trauma and was
other neurological conditions and developmental diagnosed with Humphrey visual field testing.
disorders, which arise from disturbed white DTI demonstrated discontinuation in the mid-
matter connections. Recent advances in the post- portion of the left optic radiation with decreased
processing and analysis algorithms of DTI data values of fractional anisotropy and increased val-
promise increased specificity and improvements ues of the diffusion coefficient in the same region
in the localization accuracy of the method and in and the more distal parts of the radiations, as com-
relating lesion location to clinical/ophthalmolog- pared to healthy subjects [18]. Furthermore,
ical symptoms. In the case of multiple sclerosis, Romero et al. in a case study of optic tract injury
DTI imaging provides improved pathological in neuromyelitis optica demonstrated the comple-
specificity compared to conventional MRI; how- mentary role of a growing number of imaging
ever, the pathological substrates underpinning modalities, including DTI and optical coherence
alterations in brain tissue diffusivity are not yet tomography (OCT), in the investigation of
fully delineated [16]. To better reveal the poten- patients with visual field defects [19].
tial association between altered DTI diffusivity DTI has found a prominent clinical applica-
and the underlying tissue damage in multiple tion in presurgical planning including identifica-
sclerosis, Klistorner et al. segmented a single tion of the spatial/topographical relationship
tract of the optic radiation into separate groups between an intracranial mass lesion and the
based on their relationship to lesions. The calcu- visual pathway in order to avoid potential visual
lation of diffusivity in predefined regions along field defects following tumor resection [14].
the white matter tracts allowed a comparison of Since most of the anterior temporal portion of the
diffusion values with the corresponding segments optic radiation passes through the temporal lobe,
of the nonlesional fibers in the same tract. damage to the Meyer’s loop during surgery is one
According to their findings, the authors suggest of the most common causes of homonymous field
that within the orbital radiation, parallel and per- defects following temporal lobe surgery.
pendicular diffusivities are affected by tissue However, in contrast to the common structural
restructuring related to distinct pathological pro- MR sequences, which cannot identify the optic
cesses, providing evidence that DTI is a sensitive radiation, DTI tractography allows the in vivo
marker to investigate the tissue damage in demonstration of this structure and thus enables
lesioned and normal appearing white matter [16]. the surgeons to optimally plan tumor resections
Furthermore, McNulty et al. assessed the impact to preserve function with maximal possible tumor
of fiber tractography on the visualization of resection [20]. According to Powell et al., who
pathologies in the medial longitudinal fasciculus found a superior homonymous quadrantanopia as
of multiple sclerosis patients suffering from prevalent complication after anterior temporal
internuclear ophthalmoplegia (INO). They found lobe resection, the preoperative application of
that DTI tractography allows improved visualiza- DTI tractography is highly recommended for a
tion of fiber pathologies related to INO, which in better outcome [21]. In addition to the common
turn supports monitoring of disease progression application of DTI methods for presurgical plan-
with refined association between lesion type and ning, the intraoperative application of DTI allows
location and clinical symptomatology [17]. immediate evaluation of the surgical intervention
The usefulness of DTI for the assessment of by combining mapping of the efferent visual sys-
patients harboring pathologies affecting the visual tem with microsurgical neuronavigation using
pathways was shown in several studies. Yeo et al. intraoperative MRI technique [20]. This tech-
demonstrated the impact of DTI in the detection nique was applied recently in clinical practice
of a lesion in the optic radiation causing visual during resection of lesions involving, or adjacent
field defects in a patient with traumatic brain to, the optic radiation in order to maintain
100 N. Aldusary et al.
patients’ visual fields [20]. The DTI images were within this area and the pronounced bending of
incorporated into the neuronavigation intraopera- the optic radiations, which are difficult to
tively to provide the surgeon with exact resolve with existing algorithms used routinely
information on the site of the lesion and the adja- in the clinical work [22]. Recently, various data
cent optic radiation [15]. acquisition schemes (i.e., q-ball imaging and
Despite the promising applications of DTI diffusion spectral imaging) and tracking algo-
fiber tracking in studying neurological disease rithms have been proposed to overcome the
of the brain, clinical investigations are limited to existing limitations. However, they require
the assessment of major fiber tracts due to short- extensive examination times that are often pro-
comings inherited to the method. First, the voxel hibiting for routing clinical use in daily patient
size of the data, which can be achieved in a clin- care. The choice of an accurate tracking algo-
ically acceptable acquisition time, is much rithm is pivotal for the reliable reconstruction of
larger than the diameter of a single axon, which visual fiber tracks in the human brain (Fig. 6.2)
lies in the range of a few microns, so only major [22]. Despite the above-mentioned challenges
fiber bundles can be resolved. Other limitations, related to DTI, this new method can be requested
particularly relevant to the reconstruction of the by clinicians on an everyday basis in most clini-
visual pathway, are related to the increased sus- cal centers operating MRI scanners. The exis-
ceptibility artifacts in the area of the chiasm, tence of several commercially available
which result in signal loss in the DTI acquisi- postprocessing softwares and neuroradiological
tions, as well as the extensive fiber crossing expertise make it relatively easy to provide
a b
Fig. 6.2 Diffusion tensor imaging (DTI) fiber tracking. f urthermore the optic radiation onto the area striata in the
Fibers reconstructed from the center of the optic chiasm occipital lobe can be seen. The fibers emerging from the
are shown. Trajectories calculated with the aFM (advanced chiasm in the posterior direction most probably represent
fast marching algorithm) are depicted in green. In both connections following the oculomotor nerve to its nuclei
hemispheres of the brain, the optic nerve, the optic tract, in the brain stem (From Staempfli et al. [22], with
the connections to the lateral geniculate nucleus, and permission)
6 Novel Imaging Techniques and Neuroradiologic Imaging 101
quantitative measures on the visual pathway for assumed that blood flow might depend on func-
better correlation of clinical symptoms with tion [29]. Accordingly, task related neural activa-
underlying structural anomalies. tion causes a greater regional increase in blood
flow than the moderate increase in the oxygen
consumption by the neurons, implying that the
6.4 Functional Magnetic blood flow is imperfectly coupled to the increased
Resonance Imaging metabolic demands of neurons both in terms of
spatial specificity and quantity [6]. Consequently,
Functional magnetic resonance imaging (fMRI), the relative concentration of oxyhemoglobin in
as a further noninvasive imaging method next to the small venules draining the area of activated
structural and DT imaging, allows the assessment neurons increases. Oxyhemoglobin has diamag-
of brain activity associated with sensory, motor, netic properties, and thus the increased blood
and cognitive behavior [1, 6, 23–26]. The appli- oxygenation can be detected by the
cation of fMRI in studies of the visual system oxyhemoglobin-sensitive fMRI as increased sig-
provided researchers with the ability to sketch nal, serving as marker of brain function. This
out the organization and functional properties of effect became known as the blood oxygen-level
the human visual system, which in turn allowed dependent (BOLD) signal [30, 31] for published
improved understanding of altered function of reviews [1, 6]. A decade after Ogawa et al. dem-
neuro-ophthalmologic disorders [1, 27, 28]. onstrated that MR technology could detect
The introduction of MR technology in the in vivo changes of blood oxygenation, the ques-
investigation of neuronal activity was facilitated tion, whether BOLD changes are related to axo-
by the finding that the MR signal could be made nal potentials, dendritic postsynaptic potentials,
sensitive to changes in blood flow and blood oxy- both or neither, could be elucidated [31].
genation providing important physiological According to simultaneous measurements of
information related to brain function [1]. Previous fMRI BOLD signal and electrophysiological
imaging methods used for mapping brain activ- activity in primates and also humans, rather post-
ity, as positron emission tomography (PET), synaptic potentials than action potentials seem to
required the injection of radioactively labeled be the origin of the BOLD signal [6, 32].
metabolites, which limited their applicability, The peculiarity of the neurovascular coupling
whereas fMRI measures the naturally occurring lends fMRI experiments a dynamic character
blood oxygenation level dependent (BOLD) con- since signal changes after neuronal activity
trast (for details see below), which makes this occur after 1–2 s and evolve over a period of
method safe and thus well-tolerated by normal 10–12 s. Thus, the progressive evolution of the
controls and patients [1, 6, 25, 26, 28]. Further signal provides information about the dynamics
advantages, which ushered fMRI to become the of the underlying physiological changes [1].
method of choice are: (a) fMRI provides both General fMRI designs aim at answering ques-
anatomic and functional information in a subject tions either related to estimation (e.g., “How did
at the same session, allowing accurate determina- the BOLD response change over time in a par-
tion of the anatomic site of the active regions; (b) ticular brain region?”) or to the detection of neu-
due to its noninvasive nature, repetitive scans in a ronal activity (e.g., “Which brain regions showed
single subject are possible; (c) it is financially task-related neuronal activity?”) [6]. To meet
affordable for most medical centers operating these questions whole brain images or particular
clinical MRI scanners; and (d) it has better spatial brain regions (e.g., occipital lobe) are acquired at
and temporal resolution than other methods relatively high temporal (around 2–3 s) and rea-
applying the same hemodynamic phenomena to sonable spatial (3 mm) resolution using axial or
localize neuronal activity [1, 25]. coronal gradient echo-imaging sequences (e.g.,
The principle of fMRI has its origin in the echo-planar-imaging so-called EPI sequences)
famous prediction of Roy and Sherrington who [1, 24–26]. To ensure accurate localization of
102 N. Aldusary et al.
cortical brain activity, T1-weighted images with well as subzones supporting vision at the center
a spatial resolution of 1 mm are additionally of gaze, relevant for reading and other daily
acquired [1, 25, 26]. The collected fMRI data is activities [33]. To promote constant attention of
usually subjected to several preprocessing steps, the healthy individuals or patients during scan-
which measure and remove unwanted variabil- ning, a button press task, including the fixation
ity, caused, e.g., by head motion [6]. To allow of a marker, which randomly disappears can be
comparison between subjects, the data is nor- added. The application of both annulus and
malized prior to a statistical analysis that tests wedge as stimuli yield detailed retinotopic
specific predictions about the relationship maps, so-called functional field maps (FFMap)
between the applied task and the evoked BOLD with circles representing visually responsive
response. Thereby, a variant of the general linear voxels. The FFMap of healthy individuals con-
model is used to evaluate the contribution of dif- tains circles distributed throughout the visual
ferent explanatory variables to the observed acti- field while focal damage in patients will reveal
vation pattern [6]. In the case of the relatively missing or less numerous circles within the reti-
novel method of resting state analysis, the notopic zone of the affected visual field [28].
changes in correlation between (visual) areas are Thus, the FFMap visualizes the relationship
investigated while subjects are at rest, e.g., with between the focal pathology as cortical pattern
the so-called independent component analysis and the resulting effect on the field of vision of
ICA, which includes the comparison of temporal the patient [33].
patterns of activity between independent compo- Functional magnetic resonance imaging
nents to elicit correlations that suggest connec- vision mapping is predominantly applied in
tivity [23, 27]. presurgical planning in patients with pathology
Cortical responses within the visual system of the visual pathways where resection is nec-
are induced by simple stimuli like turning lights essary [27, 28, 33, 34]. In order to avoid dam-
on and off, or flashing a large checkerboard or age to visual cortex, which is critical for daily
moving dots. However, such stimuli do not vision, fMRI maps provide additional informa-
allow a distinction between cortical representa- tion for resection planning which aims at gain-
tions of peripheral versus central vision, which ing maximized therapeutic success with
is relevant for tasks like reading [28]. A sophis- minimal resulting postoperative deficits [28,
ticated paradigm, which allows the mapping of 33]. In addition, fMRI visual field mapping can
visual field eccentricity and angular position, is also reveal abnormal functional brain organiza-
described by DeYoe and Raut. It consists of a tion, which is not easily discovered by conven-
slowly expanding checkered annulus and a tional clinical tests. For example, DeYoe et al.
slowly rotating checkered wedge, which are report on an albinistic patient where FFmaps
sequentially displayed. Due to the composition revealed that the opposite halves of the visual
of the high-contrast checkerboard consisting of field projected onto the same left cortex [33].
counter-phasing black and white checks, which Furthermore, Dem Hagen et al. (2008) com-
flicker at 8 Hz, strong neural activation and also pared fMRI and visually evoked potentials
large increases in the BOLD fMRI signal in the (VEP) to assess misrouting in albinism. They
responsive brain region can be achieved. found that hemifield stimulation recorded with
Furthermore, the presentation mode of the stim- fMRI is highly sensitive and more effective
uli is such that locations in the visual field, than VEP to detect misrouting by using interoc-
which differ in eccentricity or angular position, ular comparison [35].
are stimulated at different times. The resulting A further application of FFMaps is reported
maps both delineate visual cortex and identify by Reitsma et al. in a study that investigated 25
multiple functionally distinct visual areas as patients with adult onset (stroke, tumor, or
6 Novel Imaging Techniques and Neuroradiologic Imaging 103
trauma) and two patients with congenital pathol- majority of research on visual processing and
ogy of the visual cortex. They identified atypical related disorders is performed using 3 T scan-
retinotopic organization in only three patients ners, future studies will certainly benefit from
revealing an expanded ipsilateral field represen- increased signal-to-noise ratio, since more and
tation, which was on average 3.2 greater com- more 7 T scanners can be afforded. In combina-
pared to healthy individuals. They concluded that tion with sophisticated analyses, noninvasive
under certain pathologic conditions, visual cortex recordings of neural activity will further pro-
might undergo large-scale retinotopic changes mote our understanding of the visual system and
[36]. It can be speculated whether the change in its disorders.
cortical organization is true reorganization or The application of fMRI in patients with
unmasking of so-called normal subthreshold hemianopia deepens not only our knowledge
organizational features [33]. about blindsight, plasticity, and recovery after
In another study, Hoffmann et al. assessed brain injury, but it also provides important infor-
two achiasmatic patients, with complete visual mation about the visual system [27]. With the
fields and apparent integrity of the visual system, aid of Gabor stimuli presented with a spatial fre-
using fMRI-based retinotopic mapping to obtain quency of 1 cycle/°, Ajina et al. recorded con-
visual hemifield representations on the cortical trast sensitivity in the human motion processing
surface for each visual hemifield and each eye. complex (MT+) in the damaged (primary visual
They demonstrated that the gross topography of cortex, V1) hemisphere of eight patients and
the geniculostriate and occipital callosal connec- aged-matched controls. They found that hMT+
tions remains largely unaltered. Voxels of the no longer revealed early saturation but increased
primary visual cortex were better modeled with a linear response in patients, which was compa-
bilateral receptive field rather than standard con- rable to the response of V1 in healthy controls.
tralateral representations. They concluded that They concluded that V1 is essential for the
visual function is preserved by reorganization of marked contrast sensitivity and early saturation,
intracortical connections instead of large-scale which can be observed in normal hMT+
reorganizations of the visual cortex. Thus, devel- responses [39].
opmental mechanisms of local wiring within The validity of the BOLD response can be
cortical maps compensate for the improper gross compromised when focal brain pathology dis-
wiring to preserve function [37], see also rupts the coupling cascade, without affecting the
Millington et al. [27]. It is suggested that an underlying neural activity [33]. The described
interleaved representation of ipsi- and contralat- effect, so-called neurovascular uncoupling
eral hemifields may allow visualization of the (NVU), can cause incorrect activation maps,
difference in response to the two hemifields; which can have devastating outcomes when
however, such an approach requires sufficiently applied in the context of presurgical planning.
high resolution in the range of 0.5 mm3 [27]. An Therefore, testing for NVU, e.g., with the com-
example of laminar high field fMRI (7 Tesla bined application of FFmaps and perimetric tests
scanner) is shown in the study of Kok et al., is highly recommended when applying fMRI for
which investigates the cortical response to illu- presurgical mapping [33]. Nevertheless, despite
sory figures in the human primary visual cortex its limitations, fMRI, which is offered in most
at different cortical depths. Applying a spatial clinical centers applying MRI, provides (often in
regression analysis, in an attempt to explicitly combination with other behavioral or imaging
unmix signals from the different layers, they methods, such as DTI) valuable information on
were able to extract signals with less dependence the functional consequences of pathologies
on the actual voxel volume than previous inter- affecting the visual system, which helps to
polation approaches [38]. Although, the vast improve diagnosis and treatment (Fig. 6.3) [1].
104 N. Aldusary et al.
a b
Fig. 6.3 Findings obtained in a 52-year-old-man with clearly illustrates diminished activation of the right calca-
glioblastomas: (a) Coronal, T1-weighed magnetic reso- rine cortex which is indistinguishable from the infiltrating
nance image with contrast showing the extension of the tumor. (c) Goldmann perimetry showing an almost com-
tumor above and below the parieto-occipital sulcus and plete left homonymous hemianopia with sparing of some
the involvement of the right medial occipital lobe. (b) of the upper central field on the left side (From Kollias
Functional maps acquired during binocular visual stimu- et al. [1], with permission)
lation, overlaid on the corresponding anatomical images,
productive to assess cerebral visual field 15. Coenen V, Huber K, Krings T, Weidemann J, Gilsbach
disorders in the acute phase and in the course J, Rohde V. Diffusion-weighted imaging-guided
resection of intracerebral lesions involving the optic
of the disease [40]. Furthermore, the catena- radiation. Neurosurg Rev. 2005;28(3):188–95.
tion of recent neuroimaging methods with 16. Klistorner A, Vootakuru N, Wang C, Yiannikas
clinical findings improves the prediction of C, Graham SL, Parratt J, et al. Decoding diffusiv-
clinical outcomes and helps to monitor dis- ity in multiple sclerosis: analysis of optic radiation
lesional and non-lesional white matter. PLoS One.
ease recovery [41]. 2015;10(3):e0122114.
17. McNulty J, Lonergan R, Bannigan J, O’Laoide
R, Rainford L, Tubridy N. Visualisation of the
medial longitudinal fasciculus using fibre tractog-
References raphy in multiple sclerosis patients with internu-
clear ophthalmoplegia. Ir J Med Sci. 2016;185(2):
1. Kollias SS. Investigations of the human visual sys- 393–402.
tem using functional magnetic resonance imaging 18. Yeo SS, Kim SH, Kim OL, Kim M-S, Jang SH. Optic
(FMRI). Eur J Radiol. 2004;49(1):64–75. radiation injury in a patient with traumatic brain
2. Kaeser P-F, Ghika J, Borruat F-X. Visual signs injury. Brain Inj. 2012;26(6):891–5.
and symptoms in patients with the visual variant of 19. Romero RS, Gutierrez I, Wang E, Reder AT, Bhatti
Alzheimer disease. BMC Opthalmol. 2015;15(1):65. MT, Bernard JT, et al. Homonymous hemimacu-
3. Trobe JD. The neurology of vision. 1st ed. New York: lar thinning: a unique presentation of optic tract
Oxford University Press; 2001. injury in neuromyelitis optica. J Neuroophthalmol.
4. Jacobs D, Galetta S. Neuro-ophthalmology for neuro- 2012;32(2):150–3.
radiologists. Am J Neuroradiol. 2007;28(1):3–8. 20. Cui Z, Ling Z, Pan L, Song H, Chen X, Shi W, et al.
5. Carolyn Asbury PD. Brain imaging technologies and Optic radiation mapping reduces the risk of visual
their applications in neuroscience: the dana founda- field deficits in anterior temporal lobe resection. Int
tion. 2011. [cited 8 Dec 2015]. J Clin Exp Med. 2015;8(8):14283.
6. Huettel SA. fMRI: BOLD Contrast. In: Squire 21. Powell H, Parker G, Alexander D, Symms M, Boulby
LR, editor. Encyclopedia of neuroscience. Oxford: P, Wheeler-Kingshott C, et al. MR tractography pre-
Academic Press; 2009. p. 273–81. dicts visual field defects following temporal lobe
7. Prins D, Hanekamp S, Cornelissen FW. Structural resection. Neurology. 2005;65(4):596–9.
brain MRI studies in eye diseases: are they clini- 22. Staempfli P, Rienmueller A, Reischauer C, Valavanis
cally relevant? A review of current findings. Acta A, Boesiger P, Kollias S. Reconstruction of the human
Ophthalmol. 2016;94(2):113–21. visual system based on DTI fiber tracking. J Magn
8. De Champfleur NM, De Champfleur SM, Galanaud Reson Imaging. 2007;26(4):886–93.
D, Leboucq N, Bonafé A. Imaging of the optic chi- 23. Khanna N, Altmeyer W, Zhuo J, Steven A. Functional
asm and retrochiasmal visual pathways. Diagn Interv neuroimaging: fundamental principles and clinical
Imaging. 2013;94(10):957–71. applications. Neuroradiol J. 2015;28(2):87–96.
9. Horton JC. What is the evaluation for a homonymous 24. Bick AS, Mayer A, Levin N. From research to clini-
hemianopia? In: Lee AG, Kline L, Brazis PW, edi- cal practice: implementation of functional magnetic
tors. Curbside consultations in neuro-ophthalmology. imaging and white matter tractography in the clinical
Thorofare: Slack; 2009. p. 139–44. environment. J Neurol Sci. 2012;312(1):158–65.
10. Vachalová I, Gindl V, Heckmann JG. Acute infe-
25. Miki A, Haselgrove JC, Liu GT. Functional mag-
rior homonymous quandrantanopia in a 71-year-old netic resonance imaging and its clinical utility in
woman. J Clin Neurosci. 2014;21(4):683–5. patients with visual disturbances. Surv Ophthalmol.
11. Kollias S. Parcelation of the white matter using DTI: 2002;47(6):562–79.
insights into the functional connectivity of the brain. 26. Miki A, Nakajima T, Fujita M, Takagi M, Abe
Neuroradiol J. 2009;22(Suppl 1):74–84. H. Functional magnetic resonance imaging in
12. Reinges MH, Schoth F, Coenen VA, Krings T. Imaging homonymous hemianopsia. Am J Ophthalmol.
of postthalamic visual fiber tracts by anisotropic diffu- 1996;121(3):258–66.
sion weighted MRI and diffusion tensor imaging: princi- 27. Millington RS, Ajina S, Bridge H. Novel brain imag-
ples and applications. Eur J Radiol. 2004;49(2):91–104. ing approaches to understand acquired and congeni-
13. Hofer S, Karaus A, Frahm J. Reconstruction and
tal neuro-ophthalmological conditions. Curr Opin
dissection of the entire human visual pathway using Neurol. 2014;27(1):92.
diffusion tensor MRI. Front Neuroanat. 2010;4:15. 28. DeYoe EA, Raut RV. Visual mapping using blood
doi:10.3389/fnana.2010.00015. oxygen level dependent functional magnetic
14. X-f T, Wang Z-Q, W-q G, Q-j J, Shi Z-R. A new study resonance imaging. Neuroimaging Clin N Am.
on diffusion tensor imaging of the whole visual path- 2014;24(4):573–84.
way fiber bundle and clinical application. Chin Med 29. Roy CS, Sherrington CS. On the regulation of the
J (Engl). 2009;122(2):178–82. blood-supply of the brain. J Physiol. 1890;11(1–2):85.
106 N. Aldusary et al.
30. Thulborn KR, Waterton JC, Matthews PM, Radda 36. Reitsma DC, Mathis J, Ulmer JL, Mueller W,
GK. Oxygenation dependence of the transverse Maciejewski MJ, DeYoe EA. Atypical retinotopic
relaxation time of water protons in whole blood at organization of visual cortex in patients with central
high field. Biochim Biophys Acta. 1982;714(2): brain damage: congenital and adult onset. J Neurosci.
265–70. 2013;33(32):13010–24.
31. Ogawa S, Lee T-M, Kay AR, Tank DW. Brain mag- 37. Hoffmann MB, Kaule FR, Levin N, Masuda Y,
netic resonance imaging with contrast dependent Kumar A, Gottlob I, et al. Plasticity and stability
on blood oxygenation. Proc Natl Acad Sci U S A. of the visual system in human achiasma. Neuron.
1990;87(24):9868–72. 2012;75(3):393–401.
32. Logothetis NK, Pauls J, Augath M, Trinath T,
38. Kok P, Bains LJ, van Mourik T, Norris DG, de Lange
Oeltermann A. Neurophysiological investigation of FP. Selective activation of the deep layers of the
the basis of the fMRI signal. Nature. 2001;412(6843): human primary visual cortex by top-down feedback.
150–7. Curr Biol. 2016;26(3):371–6.
33. DeYoe EA, Ulmer JL, Mueller WM, Sabsevitz DS, 39. Ajina S, Rees G, Kennard C, Bridge H. Abnormal
Reitsma DC, Pillai JJ. Imaging of the functional and contrast responses in the extrastriate cortex of blind-
dysfunctional visual system. Semin Ultrasound CT sight patients. J Neurosci. 2015;35(21):8201–13.
MR. 2015;36(3):234–48. 40. Gau M, Nestler A, Dietrich J, Faude F. A retrosellar
34. Kollias SS, Landau K, Khan N, Golay X, Bernays arachnoid cyst as a rare cause of homonymous hemi-
R, Yonekawa Y, et al. Functional evaluation using anopsia. Klin Monbl Augenheilkd. 1998;212(6):480–
magnetic resonance imaging of the visual cortex in 1. [Article in German].
patients with retrochiasmatic lesions. J Neurosurg. 41. Polonara G, Salvolini S, Fabri M, Mascioli G, Cavola
1998;89(5):780–90. GL, Neri P, et al. Unilateral visual loss due to ischaemic
35. von dem Hagen EA, Hoffmann MB, Morland AB. injury in the right calcarine region: a functional mag-
Identifying human albinism: a comparison of VEP and netic resonance imaging and diffusion tension imaging
fMRI. Invest Ophthalmol Vis Sci. 2008;49(1):238–49. follow-up study. Int Ophthalmol. 2011;31(2):129–34.
Pupillary Disorders
in Homonymous Visual Field 7
Defects
Karolína Skorkovská, Barbara Wilhelm,
and Helmut Wilhelm
Abstract
Classically, the pupil light reflex pathway is considered to be a simple reflex
arc consisting of the retinal ganglion cells, intercalated neurons in the mid-
brain, the oculomotor nerve, and short ciliary nerves. However, there are
some specialties in the structure of the afferent pupillary pathway that should
be taken into account when interpreting pupillary disorders and that can help
in the topodiagnosis of the lesion. Moreover, studies in patients with lesions
of the retrogeniculate pathway showed that the pupillary pathway is more
complex than previously assumed and the retrogeniculate visual pathway
and the visual cortex are also involved in the pupillary light reaction. Clear
anatomic evidence is still lacking but pupillographic measurements in
patients with various disorders of the visual pathway support the existence
of two pupillomotor channels that drive the pupil light reaction – the subcor-
tical (more primitive, luminance channel associated with the intrinsically
photosensitive retinal ganglion cells) and the suprageniculate (responds to
shifts in structured stimuli, is driven by the rods and cones, and receives
input from the visual cortex and extrastriate areas). The chapter summarizes
possible pupillary findings in patients with homonymous hemianopia.
Keywords
Pupil • Pupil light reflex • Relative afferent pupillary defect • Pupil
perimetry • Chromatic pupillography • Swinging flashlight test •
Hemihypokinesia • Hemiakinesia • Intrinsically photosensitive retinal
ganglion cells • Melanopsin
7.2.1 R
elative Afferent Pupillary light response is higher than the differential light
Defect and Swinging threshold in conventional perimetry, stimuli in
Flashlight Test pupil perimetry have to be brighter or larger.
Brighter stimuli increase straylight, and larger
The most frequently evaluated pupillary parame- stimuli reduce spatial resolution of pupil perime-
ter in clinical practice is the relative afferent try. This is the major problem of all systems
pupillary defect (RAPD). It is typically related to applied in pupil perimetry. To overcome this,
lesions within the anterior visual pathway and is M-sequence techniques known from multifocal
almost always present in unilateral or asymmet- electroretinography have been applied but not yet
ric bilateral diseases of the optic nerve, chiasm, tested against conventional pupil perimetry.
or the optic tract. It can be diagnosed by means of Visual field defects in pupil perimetry can be
the swinging flashlight test and is characterized recognized by a reduced or absent pupil light
by diminished pupillary constriction on direct reaction within these areas. Studies dealing with
illumination with a normal consensual response clinical applications of pupil perimetry have
to illumination of the contralateral eye. shown that most diseases affecting the retina and
Swinging flashlight test can be performed as the visual pathway caused pupil field scotomata
follows: In a darkened room ask the patient to fix- which match the defects found in standard perim-
ate an object in a few meters’ distance. Shine etry (Figs. 7.2, 7.3, and 7.4) [3–5].
with the ophthalmoscope in an angle of 45° from Pupil perimetry can be performed either by
below and from the distance of 20–40 cm into the means of a special pupillographic device or by a
eyes. Move the light quickly from one eye to the modified standard perimeter. However, most of
other and observe the direct pupil light reaction these devices serve for research purposes and only
of both pupils. Both pupils should be illuminated a few machines are available commercially. In our
for the same time (ca. 2 s) and the switch between laboratory, the pupillographic device consists of a
both eyes should be repeated at least five times. If computer, a 19-inch CRT screen for the stimulus
a relative afferent pupillary defect is present on presentation, and a third monitor for continuous
one side, then at the illumination of this eye both monitoring of fixation by observation (Fig. 7.5).
pupils will either enlarge without any previous Stimuli are displayed on the computer screen at a
contraction or this contraction will be smaller distance of 20 cm from the subject’s eye. A small
and shorter. RAPD can be quantified by means of red spot is presented for fixation. Blinds around the
neutral density filters and expressed in log units: device prevent stray light from the room disturbing
A filter is placed between light source and the the measurement. The pupil reaction is recorded by
“good eye”. If there is still a RAPD defect visi- means of an infrared-sensitive video camera. The
ble, a filter with higher density is chosen until the pupil edges can be determined by the contrast of
difference in pupillary constriction between both the dark fundus and a very light iris infrared reflex.
eyes disappears or even the RAPD switches side. During the test the examiner can observe the qual-
The density of the filter necessary to compensate ity of fixation, the stimulus sequence, as well as the
the side difference is a measure for the RAPD. continuous pupillographic curve. For the stimuli,
white light is usually used and different stimulus
intensities can be tested with a constant background
7.2.2 Pupil Perimetry luminance of 2.7 cd/m2. The stimulus is usually
presented for 200 ms every 2000 ms.
Pupil perimetry or campimetry is an objective In contrast to standard visual perimetry, pupil
visual field test that measures pupil light reaction perimetry represents a method for objective
(PLR) to focal light stimuli projected onto the visual field examination. It can be very useful
retina. Light stimuli are presented at various particularly in patients suspected of stimulation
locations in the visual field, similar as in standard [6] or in patients who do not manage standard
perimetry. However, as the threshold for the pupil perimetry well enough.
110 K. Skorkovská et al.
Visual Visual
acuity 1.25 30° visual field acuity 1.00
Left Right
(Tübinger automatik perimeter)
Relative Absolute
defect defect
Left eye
Fig. 7.2 (Top) Visual field in a patient with sphenoid value of pupil light response amplitude in millimeters at
wing meningioma causing a lower altitudinal defect in the each tested location in the visual field. Corresponding
left eye. (Bottom) Pupil field of the left eye as detected by pupil field defect in the lower hemifield can be recognized
means of pupil perimetry. The column represents the mean by a reduced pupil light reaction in this area
7 Pupillary Disorders in Homonymous Visual Field Defects 111
Relative Absolute
defect defect
Right eye
Fig. 7.3 (Top) Visual field in a patient with pituitary ade- field of the right eye showing a corresponding pupil field
noma affecting the entire visual field of the left eye and defect in the temporal hemifield
the temporal hemifield of the right eye. (Bottom) Pupil
112 K. Skorkovská et al.
Fig. 7.4 (Left) Schematic drawing of advanced concen- (Right) Corresponding pupil field with pupil light reaction
tric visual field loss in a patient with retinitis pigmentosa present only within the preserved visual field (From
as detected by kinetic perimetry (Goldmann stimulus V4). Skorkovská et al. [4], with permission)
Right
Left
RAPD N3
right
OT
RAPD
right
M
OR
no
RAPD
Fig. 7.8 Schematic representation of different findings pupillary defect (RAPD). Lesions of the brachium of the
according to the course of the pupil light reflex pathway superior colliculus cause contralateral RAPD but no
(OT optic tract, M midbrain, N3 oculomotor nerve, OR visual field defect. In suprageniculate lesions with suffi-
optic radiation). Lesions of the optic tract result in hom- cient distance from lateral geniculate body homonymous
onymous hemianopia with contralateral relative afferent hemianopia without RAPD develops
even visual cortex is involved in the pupillary measurements in patients with suprageniculate
light reaction. In patients with retrogeniculate lesions have been performed already by Harms in
damage the so-called pupillary hemihypokinesia 1949 [22] and have challenged the Wernicke’s
can be observed which differs from RAPD. description of the pupil light reflex. Harms found
Pupillary hemihypokinesia (or akinesia) reduced pupil light reaction in war veterans with
means a reduced or absent pupil light reaction to occipital lobe injuries. At that time, his results
perimetric stimuli in the blind part of the visual were called into question and the findings
field and was observed in all kinds of postchias- ascribed to the transsynaptic degeneration or to
mal lesions (Fig. 7.9). The first pupillometric an overlooked pregeniculate damage. Harm’s
Visual Visual
acuity 0.80 Left 30° visual field Right acuity 0.80
(Tübinger automatik perimeter)
Relative Absolute
defect defect
Left Right
Fig. 7.9 (Top) Visual field in a patient with superior left or absent pupil light reaction in the affected portion of the
homonymous quadrantanopia due to an ischemia. visual field (From Skorkovská et al. [4], with permission)
(Bottom) Pupil field of the same patient showing a reduced
7 Pupillary Disorders in Homonymous Visual Field Defects 117
findings were eventually many times reproduced, “luminance channel,” which connects the retina
later also with the help of modern pupillographic directly with the pretectal area and responds to dif-
equipment and sophisticated imaging methods. fuse light, and “pattern channel,” which is medi-
Still, even today we can only speculate about the ated suprageniculately and responds to shifts in
underlying cause of this phenomenon. structured stimuli, like isoluminant grating,
The findings, for example, can be explained by motion, and isoluminant color stimuli. The PLR is
the view, that in pre- and retrogeniculate lesions primarily mediated by the luminance channel and
different components of the light response may be to a smaller extent by the “weaker,” supragenicu-
involved to a different extent. The steady- state late pattern channel (Fig. 7.10). It seems that the
component of the pupillary light response regu-
lates the resting pupil diameter depending on the
SCN CG
ambient light level; it is characterized by a large
spatial summation and a wide dynamic range. This
component is represented basically by the subcor-
tical pupillary pathway. The transient component N.III
of the pupil light response is responsible for the
constriction of the pupil in response to brisk light
ON
stimuli. In the presence of this component, the
steady-state signal is largely discarded. The tran-
sient component reflects merely novel changes in
luminance contrast; it is characterized by a “lim-
ited spatial summation, band-pass temporal
response characteristics, and high contrast gain”
[30, 31]. It is obvious that the stimulus characteris- NEW
OT
tics of pupil perimetry predominantly address this
transient component. There is strong evidence IN
that – after cortical processing of specific stimulus
characteristics – projections from the extrastriate PN
Pathologie des Sehorgans. Albrecht Von Graefes Arch patients with geniculate and retrogeniculate lesions.
Ophthalmol. 1949;149:1–68. Neuro Ophthalmol. 1996;16(4):219–24.
23. Harms H. Hemianopische Pupillenstarre. Klin Monbl 29. Papageorgiou E, Ticini LF, Hardiess G, Schaeffel F,
Augenheilkd. 1951;118:133–47. (Article in German). Wiethoelter H, Mallot HA, et al. The pupillary
24. Bresky R, Charles S. Pupil motor perimetry. Am
light reflex pathway: cytoarchitectonic probabi-
J Ophthalmol. 1969;68(1):108–12. listic maps in hemianopic patients. Neurology.
25. Cibis GW, Campos EC, Aulhorn E. Pupillary hemia- 2008;70(12):956–63.
kinesia in suprageniculate lesions. Arch Ophthalmol. 30.
Barbur JL, Keenleyside MS, Thompson WD.
1975;93:1322–7. Investigations of central visual processing by means
26. Alexandridis E, Krastel H, Reuther R. of pupillometry. In: Kulikowski JJ, Dickinson CM,
Pupillenreflexstörungen bei Läsionen der oberen Murray TJ, editors. Seeing contour and colour.
Sehbahn. Albrecht Von Graefes Arch Klin Exp Oxford: Pergamon Press; 1987. p. 431–51.
Ophthalmol. 1979;209(3):199–208. (Article in German). 31. Barbur JL. Learning from the pupil – studies of basic
27. Hellner KA, Jensen W, Mueller-Jensen A. mechanisms and clinical applications. In: Chalupa
[Videoprocessing pupillographic perimetry in hemi- LM, Werner JS, editors. The visual neurosciences.
anopsia] Fernsehbildanalytische pupillographische Cambridge: MIT Press; 2004. p. 641–56.
Perimetrie bei Hemianopsie. Klin Monbl Augenheilkd. 32. Wilhelm BJ, Wilhelm H, Moo S, Barbur JL. Pupil
1978;172(5):731–5. (Article in German). response components: studies in patients with
28. Wilhelm H, Wilhelm B, Petersen D, Schmidt U,
Parinaud’s syndrome. Brain. 2002;125(Pt 10):
Schiefer U. Relative afferent pupillary defects in 2296–307.
Eye Movements and Visual Search
in Homonymous Visual Field 8
Defects
Jason J.S. Barton
Abstract
Eye movements in hemianopia can serve as an index of perceptual
changes, residual visual function, or adaptive changes to altered vision.
Central fixations are shifted towards the blind hemifield and saccades
into the seeing hemifield are prolonged and less reliable. Targets in the
blind hemifield are initially found with an inefficient series of small sac-
cades, but some patients develop a compensatory search hypermetria.
Blindsight studies have reported on the accuracy and reliability of sac-
cades or pursuit responses to targets in the blind hemifield, with variable
results. How hemianopic subjects scan visual information has been stud-
ied with search displays, line bisection and reading. The efficiency of
scanning by hemianopic subjects is an important determinant of success
in daily life activities such as driving.
Keywords
Hemifield • Dyslexia • Blindsight • Line Bisection • Visual Search •
Driving • Saccade
8.1 Introduction
e piphenomenon, eye movements may still reveal tions. The most straightforward one is that since
aspects about hemianopia that are informative half of the foveal region is lost in complete hemi-
for the researcher or critical for the patient. anopia, placing fixation slightly towards the side
For one, what we see determines where we of the hemianopia allows more of the fixated
look. In this sense, the change in visual experi- object to be seen, optimizing the perception of
ence caused by hemianopia will necessarily alter stimuli in the central field. If so, it should follow
the eye movements made in response to visual that hemianopic patients with sparing of macular
input. Studying those eye movements may allow vision would be less likely to show such a fixa-
inferences about the processing of vision after tion shift. Whether this is true is not known.
hemianopia. Furthermore, there is the interesting Alternatively, such a fixation shift may reflect
finding that what we do not see in hemianopia a perceptual shift of the hemianopic subject’s
may still influence where we look. ‘Blindsight’ estimate of the center of a visual scene. It has
rests on experimental data that show that visual long been known that hemianopic patients show
stimuli in the blind hemifield of which the patient a small contralateral deviation in line bisection
is unaware can still influence their responses to judgments [5, 6]. This may in turn have several
the world. Eye movements have had a prominent explanations. First, the representation of visual
role in such research, and indeed these were the space may be altered in hemianopia. From the
response measured by the first study that claimed retina to striate and extrastriate cortex, central
to demonstrate blindsight in humans over regions of vision are more represented than more
40 years ago [1]. peripheral regions in the visual system [7, 8]. In
The reverse is also true, however: where we someone with only one hemifield, this central
look also determines what we see. This is par- magnification means that the part of the remain-
ticularly true in the case of hemianopia. Visual ing visual field nearest to the blind field is more
stimuli located within a blind hemifield are not strongly represented, an effect which may be
seen, but can be perceived if the eyes are moved accentuated in hemianopia [9]. This can gener-
so that the stimuli now fall within the seeing ate a small contralateral bias in line bisection
hemifield. Eye movements thus play a critical judgments made by healthy subjects forced to
role in the visual experience of the hemianopic view lines with only one hemifield [10]. Thus, as
patient. Some changes in eye movements repre- they scan the environment, the contralateral
sent important strategic adaptations of behavior regions of space are emphasized, and the result
that can reduce the impact of hemianopia in may be a contralateral deviation of their estimate
daily life. Studying whether these ocular motor of where center is located. Second, there is an
changes are present, how they evolve and if they imbalance in spatial attention in hemianopia.
can be enhanced through rehabilitation are Unlike the case of hemineglect, in which there is
important and practical components of hemiano- a pathologic failure to direct attention to contra-
pia research. lateral space, patients with hemianopia direct
more attention contralaterally, into the blind
field, which is evident in fixation patterns they
8.2 Fixation make during visual search [11]. This is a strate-
gic adaptation to their deficit, and the increased
Fixation is the simple task of maintaining the emphasis of attention on contralateral space may
image of an object at the fovea, the central region lead to a deviation of the subjective estimate of
of the retina with the highest spatial resolution, center. Consistent with this adaptive hypothesis
and most often also the point at which attention is is the observation that the contralateral line
focused. A common finding in hemianopic bisection error is not seen in the acute stage of
patients is a slight shift of central fixation into hemianopia [12]. Whether any of these percep-
contralateral space [2–4]. This is likely a strategic tual explanations account for contralateral devia-
adaptation that has a number of possible explana- tions of perceptual center as seen during line
8 Eye Movements and Visual Search in Homonymous Visual Field Defects 123
bisection remains a subject of debate, with con- entire visual system, possibly reflecting damage
flicting findings from studies in which hemiano- to connection fibers both within and between
pia is simulated by virtual gaze- contingent hemispheres [16].
displays in healthy subjects [13, 14].
and to use that in lieu of direct visual input to study found a weak correlation of saccadic ampli-
generate coordinates for saccadic programming. tude with target position in only two of ten
With time some patients develop a more effi- patients [25].
cient strategy to find targets in their blind hemi- It has been suggested that blindsight saccadic
field. They make one very large contralateral localization might improve with training, as in
saccade that will place most of the previously monkeys [38]. In humans, the accuracy of sac-
unseen portions of the world into their seeing cadic search (as opposed to initial saccades) was
ipsilateral hemifield, allowing them to perceive weak or nonexistent in six subjects but improved
the target quickly and then make an ipsilateral with training [39, 40]. However, this probably
saccade to it [18]. Some healthy subjects experi- represents learning of an adaptive strategy rather
encing a computer-simulation of hemianopia than development of blindsight.
learn this strategy rapidly over minutes [13]. Hemidecorticate patients show what is possi-
However, children may not develop this adaptive ble without striate and extrastriate cortex.
strategy naturally [28] and the same may be true Braddick et al. [41] found that two hemianopic
of some adult patients: hence, some rehabilitative infants were more likely to look to their blind
approaches specifically try to foster this ‘search hemifield when a target was presented there than
hypermetria’ as a strategic compensation [29]. when there was no target at all, suggesting some
rudimentary subcortical target detection in the
hemianopic region.
8.5 Blindsight Saccades A slightly different question to that of local-
ization is whether stimuli in the blind hemifield
Early hypotheses about blindsight centered on can reliably trigger saccades. One study used
the role of the superior colliculus and thus studies blocks that included both trials on which a target
naturally focused on the ability to locate targets appeared in either the seeing or blind hemifield as
with saccades. Indeed, in monkeys with striate well as catch trials on which no target appeared
ablations, saccadic localization of contralateral [21]. Four hemianopic patients initiated more
targets is lost after a muscimol injection of the saccades when blind field stimuli appeared than
superior colliculus [30]. The first study [1] found they did on the catch trials, even though they
a weak correlation between saccadic size and tar- replied that they had not seen a stimulus. The
get position in four patients with incomplete amplitude of the saccades to the targets in the
hemianopia. Patient DB had a weak correlation blind hemifield did not correlate with target loca-
of saccades with targets, but only for targets tion, however, indicating no blindsight ability to
between 5° and 25° [31, 32], a result mainly locate the target.
driven by saccades to the target at 5°, which was
a portion of the visual field that later recovered
[33]. Interestingly, a later study of DB did not 8.6 Blindsight Modulation
find evidence of saccadic localization when the of Saccades
targets varied in both horizontal and vertical
position [34]. Nevertheless, another study [35] An alternate blindsight strategy is to see how sac-
found some saccadic localization in two hemi- cades to seen stimuli are affected by additional
anopic patients, for targets with eccentricity of stimuli in the blind hemifield. Saccades show
less than 30°. small curved deviations away from distractors
Other studies found that only patients with placed along their trajectory [42, 43]. In two of
conscious residual vision – who perceived the five hemianopic patients, vertical saccades devi-
flashes as ‘dark shadows’ – reliably located tar- ated away from distractors even though they were
gets in their blind hemifield with saccades [36, in their blind hemifield [44]. In the global effect,
37]. Another did not find any saccadic localiza- if the distractor is close to the target, the saccade
tion in three hemianopic patients [18]. A larger lands at a position between the distractor and the
8 Eye Movements and Visual Search in Homonymous Visual Field Defects 125
target [45]. This is attributed to spatial averaging directly to extrastriate regions such as area V5,
of the neural activity generated by the distractor which is involved in motion perception. Striate
and target in some neural map, most likely in the lesions do not abolish motion responses in area
superior colliculus [46, 47]. Curiously, two hemi- V5 [54–56] or area V3A [57] unless accompa-
anopic patients displayed a paradoxical global nied by lesions of the superior colliculus [54, 58],
effect, with saccadic endpoints that deviated though another study using optical imaging
away from rather than towards distractors in their found that deactivation of striate cortex with
blind hemifield [48]. muscimol abolished activity in area V5 [59]. In
Another study took a different modulatory normal human subjects, physiologic studies
approach. The antisaccade task requires subjects using evoked potentials [60] or transcranial mag-
to make a saccade to a position in the direction netic stimulation [61] have suggested that visual
opposite to the location of the target [49, 50]. The motion signals may arrive in area V5 before and
amplitudes of these antisaccades are more vari- independent of signals in striate cortex, though
able and inaccurate than those of saccades made again this is not replicated by all studies [62].
to the location of visible targets [50]. One blind- In hemianopia, such evidence has spurred
sight study showed subjects target in their seeing investigation of blindsight by studying eye move-
ipsilateral hemifield, which required them to ments to moving stimuli. For saccades, one study
make an antisaccade towards their blind contra- found that their accuracy was better to oscillating
lateral hemifield. Antisaccades were more accu- rather than stationary spots in one patient [63].
rate and faster when the stimulus in the seeing This contrasts with mixed results in manual
hemifield was accompanied by a probe that was pointing accuracy in two other studies: this was
simultaneously flashed at the goal location in the more accurate to moving than stationary gratings
blind hemifield [51]. in only two of six patients [35], and no different
Other studies using a modulatory approach for moving versus stationary squares in four
have produced negative findings. In the ocular hemispherectomized patients [64]. However, the
motor distractor effect, distractors far away from more classic ocular motor responses to motion
the target or saccadic trajectory tend to increase are the optokinetic response and smooth pursuit.
the latency of saccades rather than modifying the Four children with cortical blindness were shown
saccadic trajectory or endpoint. Blind distractors to have optokinetic nystagmus [65], though this
did not have any influence in hemianopic sub- is tempered by the fact that two may have had
jects in one study [52]. Another study examined some residual vision. In the two children with
the integration of visual and auditory information congenital cortical blindness, the optokinetic
in hemianopic patients. While the presence of responses elicited by monocular stimulation
concurrent visual stimuli improves the accuracy showed a temporo-nasal asymmetry, which is
of saccades to auditory targets by healthy sub- characteristic of the components of the optoki-
jects, seven hemianopic patients did not show netic system located in the brainstem, namely the
any benefit from visual stimuli in their blind con- nucleus of the optic tract [66]. An adult with cor-
tralateral hemifield [53]. tical blindness recovered some optokinetic
responses after 5 months [67], but two other cor-
tically blind patients did not [68, 69], nor were
8.7 Blindsight Optokinetic these elicited by motion stimuli in the blind
and Pursuit Responses hemifield of hemianopic patients [70]. The speed
of the ocular pursuit of small moving targets
Responses to moving stimuli have been a fre- weakly correlated with target speed in only one
quent choice in blindsight studies. This has been of ten hemianopic patients studied [25], and this
driven primarily by observations in monkeys that did not depend on the integrity of the lateral
support the existence of a retino-tecto-pulvinar occipitotemporal cortex, the location of the
relay that bypasses striate cortex to project human homologue of area V5 [71].
126 J.J.S. Barton
One study also looked at whether moving allow hemianopic patients to attain a perfor-
stimuli in the blind hemifield can influence the mance accuracy on complex tasks that is similar
pursuit of a moving target in the seeing hemifield to that of controls [76]. With time, the efficiency
[72]. This built on observations that, first, humans of search can improve, in part through making
can generate smooth pursuit eye of an imaginary larger and earlier saccades into the hemianopic
target whose location is indicated by moving side [77, 80].
flanking stimuli situated in parafoveal vision and, In addition to these general effects, the pattern
second, that pursuit is better when there are two of search is also altered in hemianopic patients.
such stimuli, one on either side, than if there is For one, numerous studies have shown that hemi-
just one [73]. The natural question in blindsight anopic patients spend more time scanning the
is whether this is true even if the second parafo- contralateral side of visual displays. This has been
veal stimulus is located in the blind hemifield. In observed in numerous visual paradigms, such as
subject JS, the addition of the blind stimulus did dot-counting [75, 76, 78, 81, 82], uninstructed
not increase pursuit gain [72]. viewing of natural and degraded images [83],
the search for randomly located targets [76] and
virtual driving displays [84]. A more fine-grained
8.8 Scanning Patterns analysis found that this contralateral emphasis of
in Hemianopia hemianopic search forms a gradient of fixations
that increases towards contralateral space (Fig. 8.1),
Beyond the metrics of saccades and pursuit eye the opposite of what is seen in hemineglect [11].
movements, the distribution of ocular fixations This may reflect an adaptive gradient of attention
made by hemianopic patients can reveal how that increases the visual exploration by hemi-
these subjects sample their visual environment. anopic patients on their blind side, which some
Early studies concluded that chronic hemiano- considered a marker of efficient strategic com-
pia does not affect the scanning of drawings [26, pensation for hemifield loss [85]. Studies with
74], but these used fairly coarse parameters. simulated hemianopia in healthy subjects show
Subsequent work has since revealed several that this adaptive gradient develops very quickly,
interesting findings. First, these have confirmed within about five trials after onset of hemianopia
the expected, that the visual search of hemiano- (Fig. 8.2), and then is slowly refined over many
pic patients is less efficient in general. Their trials to become more efficient [86].
overall search times, total number of fixations Altered scanning patterns can also be shown
made and scanpath lengths (the sum of the with other paradigms besides visual search. Line
amplitude of all saccades made during search) bisection is a classic task used to diagnose
are all increased, and their fixation durations are hemineglect. It can differentiate hemianopia
longer and fixations more repetitive [75, 76]. from hemineglect: while patients with hemine-
Such findings are already evident in patients glect tend to place bisection points too far towards
studied in the first few days following the onset the side of their lesion, as if they were unaware of
of hemianopia after a stroke [77] and may be the contralateral extent of the line, hemianopic
more severe in those with right hemianopia [75, patients tend to make a small error in the opposite
77]. Comparisons with the experimental effects direction [2]. During line bisection, healthy sub-
of virtual hemianopia created in healthy sub- jects concentrate fixations around the center of
jects suggest that most of these effects are the line and rarely look at the peripheral ends of
directly attributable to the hemianopia [78]; the line [2, 27, 82]. This may reflect the fact that
however, others suggest that some of the the center of the line is most relevant during a
increased search duration and certain effects bisection task, and hence attention is deployed to
such as repetitive fixations may be due to dam- this region. In contrast, hemianopic patients have
age to other brain circuitry [79]. Also, some of two peaks of fixations (Figs. 8.3 and 8.4), one at
these changes may represent adaptations that the end of the line on their blind side and a central
8 Eye Movements and Visual Search in Homonymous Visual Field Defects 127
0.06
Proportion fixations
0.04
0.02
A
N L F
A
S Q
B P
T Bins of 2° of visual angle
M U R
N P A R
A C W A
V A
J T B
A I L A W X
W C D
P K
F E U R A
L A H A
M
D F A B S
A G Y N H
R K J F
O B A B A
Z A
F A P M K J S A
U M A T
G C P C A
Fig. 8.1 Horizontal distribution of scanning during visual left hemianopia. While control subjects distribute their
search. Subjects count the number of ‘A’s in the display fixations evenly across the display, patients with hemine-
shown at the lower left. The proportion of fixations allo- glect show a gradient that emphasizes the right side of
cated to each 2° bin of horizontal space is shown in the space and relatively ignores the left. Patients with left
graphs, with 0 representing the bin at center, and positive hemianopia, however, show the reverse gradient, empha-
values indicating bins to the right. Left graph shows data sizing the left side of space (Adapted from Behrmann
for healthy control subjects, middle graph for patients et al. [11] with permission)
with left hemineglect, and right graph for patients with
10
Right hemianopia
Left hemianopia
Fig. 8.2 Scanning during visual search in virtual
5
hemianopia. Healthy subjects are made virtually
hemianopic by using a gaze-contingent display with
Horizontal position(°)
0 0 0
Normal subject Left hemianopic patient Left
hemineglect
1750
4000 4000
Time (ms)
3500
8000 8000
5250
Left Right
12000 7000 12000
–17 –8.5 0 8.5 17 –17 –8.5 0 8.5 17 –17 –8.5 0 8.5 17
Fig. 8.3 Examples of scanning during line bisection. In subject. The black arrow at the bottom shows the bisec-
each graph, the x-axis plots the horizontal position of tion judgment made by the subject. The healthy subject
fixations against the time point in the trial on the y-axis. places most fixations near the line center, with occa-
The trial begins at the top (time point 0 ms). Left graph sional forays to the right or left periphery. The left hemi-
shows the scanning of a healthy subject, middle graph anopic subject scans the left end of the line and a region
that of a subject with left hemianopia, and the right near line center but offset towards the left side. The left
graph that of a subject with left hemineglect. The dotted hemineglect subject spends almost all their fixation on
vertical line shows the center of the line being bisected, the right side of the line (Adapted from Barton et al. [2],
while the solid gray line tracks the eye movements of the with permission)
Long line
1500.00
Normal
L hemineglect
Fixation index (ms/°)
1000.00
Fig. 8.4 Horizontal
distribution of fixations 500.00
during a line bisection task.
The line being bisected is
shown above the bottom
graph as a gray shaded
bar. The fixation index 0.00
reflects the density of –25.00 –12.50 0.00 12.50 25.00
fixations in a particular
region of space; 0 is the L hemianopia
midline of both the line
and space. In the top 1000.00 R hemianopia
graph, controls scan
Fixation index (ms/°)
2 2
2 One
line
3 3
3
4 4
4
5 5
5
Fig. 8.5 Examples of scanning during reading of a para- before returning with a single saccade to the start of the
graph. On the Y-axis is time, while the X-axis shows the next line. The subject with left HVFD is slightly slowed,
horizontal position of the eye: vertical segments are fixa- but their main difficulty is finding the start of the next line,
tions while dotted horizontal transitions are saccades. Left having to hunt for it with several small saccades. The
trace is from a healthy subject, middle trace is from a patient with right HVFD takes a long time to read a single
patient with left homonymous visual field defect [HVFD], line, using a series of very small rightward saccades to
and right trace is from a patient with right HVFD. The move along the line (From Trauzettel-Klosinski and
healthy subject makes about four fixations for each line, Brendler [88], with permission)
peak [2, 82, 87]. Furthermore, the central peak is d ifficulty because that start is now in their blind
offset slightly contralaterally, which parallels the region. Healthy subjects simply make one large
observation of a contralateral line bisection bias saccade to the beginning of the next line, with at
in hemianopia [5, 6]. most one small secondary corrective saccade.
Hemifield defects that involve the central 5° Patients with left hemianopia make a series of
impair reading, leading to hemianopic dyslexia leftward saccades instead (Fig. 8.5), hunting for
[88]. Reading is a classic example of the inter- the beginning of the line, and sometimes ending
play of perception and eye movements, as it is on the wrong line [88, 90]. Right hemianopia is
accomplished by a series of fixations that each more problematic for two reasons. First, the span
sample a portion of the line on a page followed of information processed during a reading fixa-
by saccades that shift fixation further down the tion is asymmetric and emphasizes the right field:
line. The direction of reading in a particular lan- it extends 15 letters or about 5° to the right but
guage and the side of the hemianopia interact to only four letters or about 1.3° to the left [91, 92].
determine the effect of the field loss in a given Second, the inability to see what is coming up
subject. With languages written left to right, read- next on the line impairs the ability to plan the
ing speed is more prolonged for patients with optimum location for the next fixation [93]. The
right hemianopia than for those with left hemi- end result is that subjects with right hemianopia
anopia [88, 89]. Left hemianopia does not affect read with a series of many, very small saccades
the rightward reading of a line much, but when (see Fig. 8.5), and their rightward progress along
the subject reaches the end and must move left to the line is often interrupted by small leftward
find the start of the next line, they experience ‘regressive’ saccades, as if they need to check
130 J.J.S. Barton
what they are reading [88–90]. Despite these dif- 8.9 Summary
ficulties, hemianopic dyslexia can improve with
practice [88, 94]. Also, some report that hemi- Eye movements can reveal many pathologic and
anopic subjects who can improve the accuracy of adaptive effects in hemianopia. Fixation is dis-
saccades when targets are predictably located placed slightly towards the blind field, which
have better adapted reading behavior [27]. may reflect either a distortion of perceptual space
In addition to these alterations in shifting fixa- or a visual or attentional adaptive change.
tion along the line by saccades, one might ques- Saccades to targets in the remaining ipsilateral
tion whether the efficiency of information hemifield are less reliably triggered, being
acquisition during a fixation is reduced by hemi- delayed or sometimes omitted. To targets in the
anopia. If so, this might be reflected in longer blind hemifield, subjects usually make a series of
fixation durations. This was indeed found in one small searching saccades, and this is also true if
study [90], but another reported that this was seen the target is a sound rather than a visual stimulus.
only if the remaining vision also showed reduced They can use prediction to improve accuracy if
contrast sensitivity [89]. the target has a consistent location, and with time
Although most of the data on ocular motor they can learn a compensatory strategy to make a
scanning in hemianopia are obtained in experi- large saccade that will place the potential target
mental settings, there is emerging evidence that locations in a seeing part of their field. Whether
scanning matters in real life. In addition to read- their eye movements reveal unconscious process-
ing, driving is an important activity that is ing of unseen targets (blindsight) remains uncer-
impacted by hemianopia. An on-road driving tain. Targets in the blind field may trigger
study using a video-camera to capture head and saccades, but the accuracy of these is debatable;
eye movements found that hemianopic drivers distractors in the blind field may modify the tra-
rated as safe, with better stability of their posi- jectory of saccades to visible targets but not to
tion in driving lanes and fewer episodes of sud- auditory ones. Scanning studies show that hemi-
den braking, make 50% more small head anopic patients are less efficient in searching dis-
movements towards the blind hemifield than plays and rapidly develop an adaptive gradient of
hemianopic drivers considered to be unsafe [95]. fixations that emphasizes the contralateral space.
This was followed by observations with virtual Right hemianopia involving the central field can
driving simulators. Hemianopic subjects who have a pronounced effect on reading, which is
show better ability to avoid collisions scan the accomplished with a series of small saccades and
display more, with larger saccades and more many regressions. Driving studies show that the
gaze shifts, and explore moving objects on their horizontal span of space explored with fixations
blind side more [84, 85]. Similarly, the detection is an important determinant of the performance
of moving obstacles on a driving simulation is of hemianopic subjects. Training of ocular motor
better in hemianopic subjects who explore a behavior may enhance the strategic adaptation of
wider horizontal range of space with their gaze these subjects to their perceptual deficit.
and show a greater shift of fixations towards the
hemianopic side [96]. Hence, scanning with
gaze shifts represents an important adaptation References
that can mitigate the effects of hemianopia on
detection of critical objects during driving. There 1. Pöppel E, Held R, Frost D. Letter: residual visual func-
tion after brain wounds involving the central visual
are promising results that training of search and pathways in man. Nature. 1973;243(5405):295–6.
reading can make the eye movements during 2. Barton JJ, Behrmann M, Black S. Ocular search dur-
these processes more efficient [80, 94, 97], and it ing line bisection. The effects of hemi-neglect and
may be that driving performance may show sim- hemianopia. Brain. 1998;121(Pt 6):1117–31.
3. Gassel MM, Williams D. Visual function in patients
ilar rehabilitative potential through training of with homonymous hemianopia II Oculomotor mecha-
scanning. nisms. Brain. 1963;86:1–36.
8 Eye Movements and Visual Search in Homonymous Visual Field Defects 131
4. Reinhard JI, Damm I, Ivanov IV, Trauzettel-Klosinski tralesional and ipsilesional saccades in hemianopic
S. Eye movements during saccadic and fixation patients. Exp Brain Res. 2014;232(3):903–17.
tasks in patients with homonymous hemianopia. 22. Girotti F, Casazza M, Musicco M, Avanzini G.
J Neuroophthalmol. 2014;34(4):354–61. Oculomotor disorders in cortical lesions in man:
5. Liepmann H, Kalmus E. Über einer Augenmaßstörung the role of unilateral neglect. Neuropsychologia.
beu Hemianopikern. Berlin Klin Wochenschr. 1983;21(5):543–53.
1900;38:838–42. 23. Meienberg O. Clinical examination of saccadic eye
6. Barton JJ, Black SE. Line bisection in hemianopia. movements in hemianopia. Neurology. 1983;33(10):
J Neurol Neurosurg Psychiatry. 1998;64(5):660–2. 1311–5.
7. Rovamo J, Virsu V. An estimation and application of 24. Meienberg O, Harrer M, Wehren C. Oculographic
the human cortical magnification factor. Exp Brain diagnosis of hemineglect in patients with homony-
Res. 1979;37(3):495–510. mous hemianopia. J Neurol. 1986;233(2):97–101.
8. Tolhurst DJ, Ling L. Magnification factors and 25. Barton JJ, Sharpe JA. Smooth pursuit and saccades to
the organization of the human striate cortex. Hum moving targets in blind hemifields. A comparison of
Neurobiol. 1988;6(4):247–54. medial occipital, lateral occipital, and optic radiation
9. Fortenbaugh FC, VanVleet TM, Silver MA, Robertson lesions. Brain. 1997;120(Pt 4):681–99.
LC. Spatial distortions in localization and midline 26. Rizzo M, Hurtig R. Visual search in hemi-neglect:
estimation in hemianopia and normal vision. Vision what stirs idle eyes ? Clin Vis Sci. 1992;7:39–52.
Res. 2015;111(Pt A):1–12. 27. Schoepf D, Zangemeister WH. Target predictability
10. Nielsen KE, Intriligator J, Barton JJ. Spatial represen- influences the distribution of coordinated eye-head
tation in the normal visual field. A study of hemifield gaze saccades in patients with homonymous hemi-
line bisection. Neuropsychologia. 1999;37(3):267–77. anopia. Neurol Res. 1996;18(5):425–39.
11. Behrmann M, Watt S, Black SE, Barton JJ. Impaired 28. Mezey LE, Harris CM, Shawkat FS, Timms C,
visual search in patients with unilateral neglect: an ocu- Kriss A, West P, Taylor DS. Saccadic strategies in
lographic analysis. Neuropsychologia. 1997;35(11): children with hemianopia. Dev Med Child Neurol.
1445–58. 1998;40(9):626–30.
12. Machner B, Sprenger A, Hansen U, Heide W,
29. Kerkhoff G, Münssinger U, Meier EK. Neurovisual
Helmchen C. Acute hemianopic patients do not show rehabilitation in cerebral blindness. Arch Neurol.
a contralesional deviation in the line bisection task. 1994;51(5):474–81.
J Neurol. 2009;256(2):289–90. 30. Kato R, Takaura K, Ikeda T, Yoshida M, Isa T.
13. Schuett S, Kentridge RW, Zihl J, Heywood CA. Is the Contribution of the retino-tectal pathway to visually
origin of the hemianopic line bisection error purely guided saccades after lesion of the primary visual cor-
visual? Evidence from eye movements in simulated tex in monkeys. Eur J Neurosci. 2011;33(11):1952–60.
hemianopia. Vision Res. 2009;49(13):1668–80. 31. Sanders MD, Warrington EK, Marshall J, Weiskrantz
14. Mitra AR, Abegg M, Viswanathan J, Barton JJ. Line L. "Blindsight": vision in a field defect. Lancet.
bisection in simulated homonymous hemianopia. 1974;1(7860):707–8.
Neuropsychologia. 2010;48(6):1742–9. 32.
Weiskrantz L, Warrington EK, Sanders MD,
15. Hess RF, Pointer JS. Spatial and temporal contrast Marshall J. Visual capacity in the hemianopic field
sensitivity in hemianopia. A comparative study of the following a restricted occipital ablation. Brain.
sighted and blind hemifields. Brain. 1989;112(Pt 4): 1974;97(4):709–28.
871–94. 33. Weiskrantz L. Residual vision in a scotoma: a follow-up
16. Rizzo M, Robin DA. Bilateral effects of unilateral study of ‘form’ discrimination. Brain. 1987;110(Pt 1):
occipital lobe lesions in humans. Brain. 1996;119(Pt 3): 77–92.
951–63. 34. Carey DP, Sahraie A, Trevethan CT, Weiskrantz L. Does
17. Barton JJ, Sharpe JA. Ocular tracking of step-ramp localisation blindsight extend to two-dimensional tar-
targets by patients with unilateral cerebral lesions. gets? Neuropsychologia. 2008;46(13):3053–60.
Brain. 1998;121(Pt 6):1165–83. 35. Perenin MT, Jeannerod M. Visual functions within
18. Meienberg O, Zangemeister WH, Rosenberg M,
the hemianopic field following early cerebral
Hoyt WF, Stark L. Saccadic eye movements in hemidecortication in man – I. Spatial localization.
patients with homonymous hemianopia. Ann Neurol. Neuropsychologia. 1978;16(1):1–13.
1981;9(6):537–44. 36. Blythe IM, Bromley JM, Kennard C, Ruddock KH.
19. Sharpe JA, Lo AW, Rabinovitch HE. Control of the Visual discrimination of target displacement remains
saccadic and smooth pursuit systems after cerebral after damage to the striate cortex in humans. Nature.
hemidecortication. Brain. 1979;102(2):387–403. 1986;320(6063):619–21.
20. Traccis S, Puliga MV, Ruiu MC, Marras MA, Rosati 37. Blythe IM, Kennard C, Ruddock KH. Residual vision
G. Unilateral occipital lesion causing hemianopia in patients with retrogeniculate lesions of the visual
affects acoustic saccadic programming. Neurology. pathways. Brain. 1987;110(Pt 4):887–905.
1991;41(10):1633–8. 38. Mohler CW, Wurtz RH. Role of striate sortex and supe-
21. Fayel A, Chokron S, Cavézian C, Vergilino-Perez D, rior colliculus in visual guidance of saccadic eye move-
Lemoine C, Doré-Mazars K. Characteristics of con- ments in monkey. J Neurophysiol. 1977;40(1):74–94.
132 J.J.S. Barton
39. Zihl J. “Blindsight”: improvement of visually guided 56. Rosa MG, Tweedale R, Elston GN. Visual responses
eye movements by systematic practice in patients with of neurons in the middle temporal area of new world
cerebral blindness. Neuropsychologia. 1980;18(1): monkeys after lesions of striate cortex. J Neurosci.
71–7. 2000;20(14):5552–63.
40. Zihl J, Werth R. Contributions to the study of "blind- 57. Girard P, Salin PA, Bullier J. Visual activity in
sight" - II. the role of specific practice for saccadic areas V3a and V3 during reversible inactivation of
localization in patients with postgeniculate visual area V1 in the macaque monkey. J Neurophysiol.
field defects. Neuropsychologia. 1984;22(1):13–22. 1991;66(5):1493–503.
41. Braddick O, Atkinson J, Hood B, Harkness W,
58. Gross CG. Contributions of striate cortex and the
Jackson G, Vargha-Khadem F. Possible blindsight superior colliculus to visual functions in area MT, the
in infants lacking one cerebral hemisphere. Nature. superior temporal polysensory area and inferior tem-
1992;360(6403):461–3. poral cortex. Neuropsychologia. 1991;29(6):497–515.
42. Doyle M, Walker R. Curved saccade trajectories: vol- 59. Collins CE, Xu X, Khaytin I, Kaskan PM, Casagrande
untary and reflexive saccades curve away from irrele- VA, Kaas JH. Optical imaging of visually evoked
vant distractors. Exp Brain Res. 2001;139(3):333–44. responses in the middle temporal area after deactiva-
43. Van der Stigchel S, Theeuwes J. Relation between tion of primary visual cortex in adult primates. Proc
saccade trajectories and spatial distractor locations. Natl Acad Sci U S A. 2005;102(15):5594–9.
Brain Res Cogn Brain Res. 2005;25(2):579–82. 60. ffytche D, Guy CN, Zeki S. The parallel visual motion
44. Van der Stigchel S, van Zoest W, Theeuwes J, Barton inputs into areas V1 and V5 of human cerebral cortex.
JJ. The influence of "blind" distractors on eye move- Brain. 1995;118(Pt 6):1375–94.
ment trajectories in visual hemifield defects. J Cogn 61. Beckers G, Zeki S. The consequences of inactivating
Neurosci. 2008;20(11):2025–36. areas V1 and V5 in visual motion perception. Brain.
45. Findlay JM. Global visual processing for saccadic eye 1995;118(Pt 1):49–60.
movements. Vision Res. 1982;22(8):1033–45. 62. Hotson J, Braun D, Herzberg W, Boman D.
46. Glimcher PW, Sparks DL. Representation of averag- Transcranial magnetic stimulation of extrastriate cor-
ing saccades in the superior colliculus of the monkey. tex degrades human motion direction discrimination.
Exp Brain Res. 1993;95(3):429–35. Vision Res. 1994;34(16):2115–23.
47. Viswanathan J, Barton JJ. The global effect for anti- 63. Bridgeman B, Staggs D. Plasticity in human blind-
saccades. Exp Brain Res. 2013;225(2):247–59. sight. Vision Res. 1982;22(9):1199–203.
48. Van der Stigchel S, Nijboer TC, Bergsma DP, Abegg 64. Ptito A, Lepore F, Ptiito M, Lassonde M. Target detec-
M, Barton JJ. Anomalous global effects induced by tion and movement discrimination in the blind field
‘blind’ distractors in visual hemifield defects. Brain of hemispherectomized patients. Brain. 1991;114(Pt
Cogn. 2010;74(1):66–73. 1B):497–512.
49. Hallett PE. Primary and secondary saccades to goals 65. van Hof-van Duin J, Mohn G. Optokinetic and spon-
defined by instructions. Vision Res. 1978;18(10): taneous nystagmus in children with neurological dis-
1279–96. orders. Behav Brain Res. 1983;10(1):163–75.
50. Munoz DP, Everling S. Look away: the anti-saccade 66. Simpson JI. The accessory optic system. Annu Rev
task and the voluntary control of eye movement. Nat Neurosci. 1984;7:13–41.
Rev Neurosci. 2004;5(3):218–28. 67. ter Braak JW, Schenk VW, van Vliet AG. Visual
51. Savina O, Bergeron A, Guitton D. Blindsight after reactions in a case of long-lasting cortical blindness.
hemidecortication: visual stimuli in blind hemi- J Neurol Neurosurg Psychiatry. 1971;34(2):140–7.
field influence anti-saccades directed there. Cortex. 68. Perenin MT, Ruel J, Hécaen H. Residual visual
2013;49(3):861–76. capacities in a case of cortical blindness. Cortex.
52. Walker R, Mannan S, Maurer D, Pambakian AL,
1980;16(4):605–12.
Kennard C. The oculomotor distractor effect in normal 69. Verhagen WI, Huygen PL, Mulleners WM. Lack of
and hemianopic vision. Proc Biol Sci. 2000;267(1442): optokinetic nystagmus and visual motion perception
431–8. in acquired cortical blindness. Neuro Ophthalmol.
53. Ten Brink AF, Nijboer TC, Bergsma DP, Barton JJ, 1997;17(4):211–8.
Van der Stigchel S. Lack of multisensory integra- 70. Perenin MT. Discrimination of motion direction in peri-
tion in hemianopia: no influence of visual stimuli on metrically blind fields. Neuroreport. 1991;2(7):397–400.
aurally guided saccades to the blind hemifield. PLoS 71. Barton JJ, Simpson T, Kiriakopoulos E, Stewart C,
One. 2015;10(4):e0122054. Guthrie B, Wood M, et al. Functional MRI of lateral
54. Rodman HR, Gross CG, Albright TD. Afferent
occipitotemporal cortex during pursuit and motion
basis of visual response properties in area MT of perception. Ann Neurol. 1996;40:387–98.
the macaque. I. Effects of striate cortex removal. 72. Intriligator JM, Xie R, Barton JJ. Blindsight modu-
J Neurosci. 1989;9(6):2033–50. lation of motion perception. J Cogn Neurosci.
55. Girard P, Salin PA, Bullier J. Response selectivity of 2002;14(8):1174–83.
neurons in area MT of the macaque monkey during 73. Wyatt HJ, Pola J, Fortune B, Posner M. Smooth pur-
reversible inactivation of area V1. J Neurophysiol. suit eye movements with imaginary targets defined by
1992;67(6):1437–46. extrafoveal cues. Vision Res. 1994;34(6):803–20.
8 Eye Movements and Visual Search in Homonymous Visual Field Defects 133
74. Chédru F, Leblanc M, Lhermitte F. Visual searching avoidance: a traffic intersection task. Front Behav
in normal and brain-damaged subjects (contribu- Neurosci. 2013;7:62.
tion to the study of unilateral inattention). Cortex. 86. Simpson SA, Abegg M, Barton JJ. Rapid adapta-
1973;9(1):94–111. tion of visual search in simulated hemianopia. Cereb
75. Zihl J. Visual scanning behavior in patients with hom- Cortex. 2011;21(7):1593–601.
onymous hemianopia. Neuropsychologia. 1995;33(3): 87. Ishiai S, Furukawa T, Tsukagoshi H. Visuo-spatial
287–303. processes of line bisection and the mechanisms under-
76. Hardiess G, Papageorgiou E, Schiefer U, Mallot
lying spatial neglect. Brain. 1989;112(Pt 6):1485–502.
HA. Functional compensation of visual field deficits 88. Trauzettel-Klosinski S, Brendler K. Eye movements
in hemianopic patients under the influence of different in reading with hemianopic field defects: the signifi-
task demands. Vision Res. 2010;50(12):1158–72. cance of clinical parameters. Graefes Arch Clin Exp
77. Machner B, Sprenger A, Sander T, Heide W, Kimmig Ophthalmol. 1998;236(2):91–102.
H, Helmchen C, Kömpf D. Visual search disorders in 89. de Luca M, Spinelli D, Zoccolotti P. Eye move-
acute and chronic homonymous hemianopia: lesion ment patterns in reading as a function of visual
effects and adaptive strategies. Ann N Y Acad Sci. field defects and contrast sensitivity loss. Cortex.
2009;1164:419–26. 1996;32(3):491–502.
78. Tant ML, Cornelissen FW, Kooijman AC, Brouwer 90. Zihl J. Eye movement patterns in hemianopic dys-
WH. Hemianopic visual field defects elicit hemiano- lexia. Brain. 1995;118(Pt 4):891–912.
pic scanning. Vision Res. 2002;42(10):1339–48. 91. Schuett S, Heywood CA, Kentridge RW, Zihl J. The sig-
79. Machner B, Sprenger A, Kompf D, Sander T, Heide nificance of visual information processing in reading:
W, Kimmig H, et al. Visual search disorders beyond insights from hemianopic dyslexia. Neuropsychologia.
pure sensory failure in patients with acute hom- 2008;46(10):2445–62.
onymous visual field defects. Neuropsychologia. 92. Rayner K, Slattery TJ, Belanger NN. Eye movements,
2009;47(13):2704–11. the perceptual span, and reading speed. Psychon Bull
80. Mannan SK, Pambakian AL, Kennard C. Compensatory Rev. 2010;17(6):834–9.
strategies following visual search training in patients 93. Leff AP, Scott SK, Crewes H, Hodgson TL, Cowey A,
with homonymous hemianopia: an eye movement Howard D, Wise RJ. Impaired reading in patients with
study. J Neurol. 2010;257(11):1812–21. right hemianopia. Ann Neurol. 2000;47(2):171–8.
81. Martin T, Riley ME, Kelly KN, Hayhoe M, Huxlin 94. Spitzyna GA, Wise RJ, McDonald SA, Plant GT, Kidd
KR. Visually-guided behavior of homonymous hemi- D, Crewes H, Leff AP. Optokinetic therapy improves
anopes in a naturalistic task. Vision Res. 2007;47(28): text reading in patients with hemianopic alexia: a con-
3434–46. trolled trial. Neurology. 2007;68(22):1922–30.
82. Ishiai S, Furukawa T, Tsukagoshi H. Eye-fixation pat- 95. Wood JM, McGwin Jr G, Elgin J, Vaphiades MS,
terns in homonymous hemianopia and unilateral spa- Braswell RA, DeCarlo DK, et al. Hemianopic and qua-
tial neglect. Neuropsychologia. 1987;25(4):675–9. drantanopic field loss, eye and head movements, and
83. Pambakian AL, Wooding DS, Patel N, Morland AB, driving. Invest Ophthalmol Vis Sci. 2011;52(3):1220–5.
Kennard C, Mannan SK. Scanning the visual world: 96. Bahnemann M, Hamel J, De Beukelaer S, Ohl S,
a study of patients with homonymous hemianopia. Kehrer S, Audebert H, et al. Compensatory eye and
J Neurol Neurosurg Psychiatry. 2000;69(6):751–9. head movements of patients with homonymous hemi-
84. Papageorgiou E, Hardiess G, Mallot HA, Schiefer U. anopia in the naturalistic setting of a driving simula-
Gaze patterns predicting successful collision avoid- tion. J Neurol. 2015;262(2):316–25.
ance in patients with homonymous visual field defects. 97. Ong YH, Jacquin-Courtois S, Gorgoraptis N, Bays
Vision Res. 2012;65:25–37. PM, Husain M, Leff AP. Eye-Search: a web-based
85. Hardiess G, Hansmann-Roth S, Mallot HA. Gaze
therapy that improves visual search in hemianopia.
movements and spatial working memory in collision Ann Clin Transl Neurol. 2015;2(1):74–8.
Driving with Homonymous Visual
Field Defects 9
Enkelejda Kasneci and Gregor Hardiess
Abstract
Driving vehicles are part of an ultimate technology in modern societies
allowing the user to travel and navigate short distances within urban regions
as well as long routes within large-scale environments. Thereby, the ability to
drive enables us to enlarge our own, biologically defined and restricted range
of mobility in a nearly unlimited manner. Driving, that is, controlling a vehi-
cle in a visually cluttered environment, involves the simultaneous use of cen-
tral and peripheral vision and the execution of primary and secondary tasks
(both visual and non-visual). As a vehicle moves through the environment,
the visual input is rapidly changing and the driver is therefore often uncertain
as to when and where a critical visual event will occur. Consequently, appro-
priate gaze behavior is a necessary cognitive tool for a safe drive in order to
maximize information acquisition together with adequate interpretations and
predictions of environmental situations based on memories.
In this chapter, the overall demands of driving are summarized and dis-
cussed in relationship with sensory, motor, and cognitive functions.
Furthermore, several options to assess driving fitness in real (on-road) and
virtual (simulator) environments together with the present regulations
concerning the permission to drive are discussed respecting healthy driv-
ers as well as visually impaired hemianopic patients. Finally, conclusions
are provided by illustrating the complexity of the task of driving that leads
to an overall high variability of behavioral strategies, which is in cause
manifested in large individual differences.
Keywords
Hemianopia • Driving • Eye movements • Head movements • Compensatory
patterns • Visual search • Brain lesions
E. Kasneci, PhD
Perception Engineering Group, G. Hardiess, PhD (*)
Department of Computer Science, Cognitive Neuroscience, Department of Biology,
University of Tübingen, 72076 Tübingen, Germany University of Tübingen, 72076 Tübingen, Germany
e-mail: enkelejda.kasneci@uni-tuebingen.de e-mail: gregor.hardiess@uni-tuebingen.de
9.1 Driving Demands (see Fig. 9.1). On the environmental side, driving
complexity is defined by road design (e.g., motor-
The process of driving can be defined as a human- ways, rural roads, city roads), road layout
machine-environment interaction. Controlling and (e.g., street curvature, inclination, junctions), traffic
steering an automobile demands the driver with a flow (high density vs. low density), and the overall
variety of tasks and processes to handle in a proper potential-of-collision (i.e., the probability to inter-
way. Obviously, driving is a highly interactive as cept with obstacles). Concerning the capability of
well as reactive task, demanding the interplay the driver, three main processes are crucial and
between perception and action behaviors capable linked to driver safety and driving performance and
to analyze and feedback the outer spatial environ- will be discussed in the following: sensory (percep-
ment with the appropriate driving operations tion), motor (action), and cognitive functions.
Organism (driver)
Cognition
(thinking, decisions,
planning)
Reflexes (stereotype)
Perception
(vision) (Inter-) Action
(behavior)
Environment
Fig. 9.1 The action-perception cycle related to the task of representations (i.e., cognition) which drive task related
driving. Successful driving depends on effective measure- and efficient behavior (i.e., inter-action). Stereotyped
ment (i.e., perception), evaluation, and integration of infor- stimulus response connections (i.e., reflexes) can bypass
mation from the external environment to form internal cognition but restrict behavior to an inflexible action
9 Driving with Homonymous Visual Field Defects 137
is a wide variation regarding the driving fitness of Furthermore, the setting was quite simple,
patients with hemianopia. although using a high number of events. In 1993,
In the following, we provide an overview on Szlyk et al. [21] assessed the driving fitness of six
individualized assessment of driving fitness in patients with hemianopic visual field defects
driving simulators as well as on-road studies. (three out of these patients with neglect). The
authors reported that the driving performance of
the patients was either worse than, or similar to,
9.2.1 Individual Assessment that of the older control [21]. Despite the small
of Driving Fitness patient group, different etiology might explain
in Simulated Environments the high variability of the results with regard to
the driving fitness. In a later study by Schulte
Driving simulators and other computerized et al. [28], the driving performance (i.e., driving
assessments using virtual reality provide a safe, speed, reaction time, and driving error rate) of
controlled environment in which many poten- nine patients with homonymous binocular defects
tially hazardous events can be presented to the was compared with that of a control group of ten
participants to investigate their driving abilities in subjects. The authors reported that they found no
an experimental way. differences regarding the tested parameters
Simulator studies conducted so far reported between the patients and the healthy-sighted sub-
various results regarding the driving fitness of jects [28]. This confirms the hypothesis that
patients with hemianopia. More specifically, individuals with hemianopic visual field defects
there is a high variability in the pass rates reported may show safe driving behavior.
by these studies, which is associated with differ- A relatively large patient group was included
ent designs of the simulator environment (fixed- in a study by Lundqvist et al. [29], who investi-
base, moving-base, advanced virtual reality gated neuropsychological aspects of driving after
expose, screen-based tasks), the stimuli design, a stroke both in a driving simulator and on road.
patient inclusion criteria (etiology), other study The authors included 30 patients and 30 control
parameters, and the number of patients. subjects in the study. For both tests, it was reported
Most of these simulator studies [23–27] that that patients performed significantly worse than
have investigated the driving ability of subjects control subjects and that they had significantly
with binocular visual field loss (including hemi- greater difficulties in allocating processing
anopic patients) have either reported the percent- resources to a secondary information processing
age of patients who were fit-to-drive without task during driving in both settings [29].
recording their eye and head movements or have In a later simulator study by Bowers et al. [3],
assessed various driving behavior parameters the authors investigated the impact of HH for 12
(e.g., hazard detection performance, lane keeping patients on the detection of pedestrians appearing
ability, steering steadiness) without linking them in several hazardous situations. As reported by
to a driving test outcome measure. Furthermore, the authors, most of the HH drivers were rated as
hemianopic patients have been usually consid- not fit-to-drive with regard to the blind-side
ered as one group and compared to healthy- detection rates. However, some HH subjects
sighted subjects. showed detection rates similar to the control
In an early simulator study by Lövsund et al. group. Furthermore, the authors reported that
[23], the authors looked at the driver’s detection most of the HH drivers took a lane position that
performance for stimuli of different sizes appear- increases the safety margin on their blind side
ing in 24 different positions on a screen. Six par- [30]. Despite the limitations in the study design,
ticipants with HH were included in the study, for example, restricted field-of-view in the simu-
from which one showed good detection abilities. lated environment, virtual pedestrian figures
However, the experimental setting, that is, the appeared abruptly and remained stationary with-
detection task, is a rather unrealistic one. out representing a collision risk, no involvement
9 Driving with Homonymous Visual Field Defects 139
of eye- or head-tracking, results from this study movements, that is, more fixations on vehicles
underscore the importance of individualized and fewer fixations on the intersection, an overall
assessments. In a later work from the same author increased gaze eccentricity, and, particularly,
group, the detection performance of HH subjects more fixations towards moving objects of interest
was examined in a more realistic approaching on the blind side. This compensatory behavior
pedestrian paradigm for both stationary and became especially evident during the more
approaching pedestrian figures that appeared at demanding task, that is, the high traffic density
small (4°) and large (14°) eccentricities [25]. The condition. Thus, the compensatory pattern of the
results from this study confirm previous findings ‘adequate’ patients brought more visual elements
from [3], namely, that most of the HH subjects into their seeing hemifield and, hence, enabled
had deficits with regard to blind-side detection or them to analyze almost all vehicles regarding the
delayed response that could potentially result in a potential-of-collision.
collision in real-world driving [25]. Besides appropriate gaze strategies, ‘adequate’
In recent publications examining a large patients were also found to differ with respect to
cohort of patients with homonymous visual field the affected brain areas identified by lesion map-
defects (i.e., 20 with hemianopia and 10 with ping. Such mapping revealed that right-hemi-
quadrantanopsia) together with (healthy-sighted) spheric damage in ‘adequate’ patients was more
control subjects, a group of authors investigated frequent in the parieto-occipital region and poste-
factors causally affecting the performance in a rior cingulate gyrus, while left-hemispheric dam-
dynamic collision avoidance task [6, 9, 11, 31]. age in ‘inadequate’ patients was more likely to
In this task, using virtual reality, gaze tracking, involve the inferior occipital cortex and the fusi-
and a natural field-of-view stimulation, subjects form (occipito-temporal) gyrus [31]. These and
had to actively approach an intersection (driving other brain lesion results [7, 32] indicate that
distance to the intersection was about 200 m) ‘inadequate’ patients might lack (at least partly) in
where a varying number of cars crossed with a their cognitive competence related to working
constant speed of 50 km/h. The traffic densities memory functioning (i.e., object recognition, con-
included two difficulty levels that would generate trol of attention, memory storage or retrieval, and
collisions in 50% or 75% of the trials should sub- memory guided saccades). To conclude, the strat-
jects not adjust their own approaching speed egy of increasing gaze exploration allows suffi-
properly. Here, subjects could adjust their own cient uptake of visual information by ‘adequate’
speed between 18 and 61.2 km/h by means of a patients. Subsequent integration of information in
joystick in order to avoid a collision with any an intact working memory enables successful
vehicle of the cross traffic. In summary, the compensation. On the other hand, reduced work-
authors found in patients and controls, traffic ing memory availability and lack of gaze move-
density as primary factor for an increased colli- ments in the ‘inadequate’ patients are associated
sion rate as well as a positive correlation between with ineffective visual adaptation (see Fig. 9.2).
collision rate and age [6]. Furthermore, patients In a recent study, Kübler et al. [5, 33] investi-
with a higher extend of visual field loss showed gated exploratory gaze patterns and driving per-
an enhanced potential-of-collision. Interestingly, formance measures that are associated with
by splitting up the collective of patients concern- successful driving performance in a simulated
ing their performance (i.e., the number of colli- driving test. The authors included in their study
sions), the authors identified the group of three patients with complete HH, two patients
‘adequate’ patients (in contrast to the ‘inadequate’ with incomplete HH, and three patients with
patients) as performing within the range of incomplete homonymous quadrantanopsia.
healthy subjects, since they adapted successfully Driving performance and visual search behavior
to their visual deficit by achieving compensatory were compared to that of (healthy-sighted) con-
gaze patterns [9]. Such distinct gaze patterns trol subjects who were gender and age-matched
were characterized by increased exploratory gaze to the patients. The study was conducted in a
140 E. Kasneci and G. Hardiess
Mechanisms
Perception
Visual
field (size)
Spatio-
temporal
integration
Inadequate Adequate
patients Cognition patients
(Working
memory)
Fig. 9.2 Mechanisms and functions enabling dynamic (cognition). By definition, all patients with homonymous
collision detection compared between ‘adequately’ and visual field defects lack in visual field size. The group of
‘inadequately’ performing patients with homonymous ‘inadequately’ rated ones have difficulties in building
visual field defects. Three mechanisms are crucial: (1) spatio-temporal representations and, hence, fail in utiliz-
The size of the (intact/usable) visual field determines ing compensatory eye and head movements. Whereas
stimulus acquisition (perception). (2) Eye and head move- ‘adequate’ patients show widely unimpaired working
ments enable the relocation of the intact visual field memory functions enabling them to compensate the visual
(action). (3) The spatio-temporal integration in working field defect by gaze compensation
memory allows to establish adequate task representations
most advanced moving-base driving simulator at inadequate driving responses in hazardous situa-
the Mercedes-Benz Technology Center in tions compared to control subjects. However, the
Sindelfingen, Germany. This driving simulator individual assessment of the driving behavior
includes a 360° virtual environment, contains a showed that 50% of the patients were rated as fit-
whole car body, and simulates acceleration to-drive. The authors further reported that hemi-
effects, which enable a very realistic driving anopic drivers who were rated as fit-to-drive did
experience. In fact, the Kübler et al.’s study [5] is not differ from the control subjects in their driv-
to date the first study that combines a most realis- ing behavior regarding speed management and
tic driving experience (since the subjects experi- lane keeping capability. Furthermore, no associa-
ence full inertial characteristics of an actual tion between the side of the visual impairment
motor vehicle) with the advantage of having and the side of the hazardous event that caused a
identical, controlled experimental settings. Each failure of the driving test was found.
subject absolved a driving route of 37.5 km Kübler et al. [5] found increased head move-
length facing nine hazardous situations during ments and longer saccades in patients who were
the course. The authors compared the study judged as fit-to-drive. Indeed, compensation by
parameters across control subjects, patients who increased saccadic amplitudes in hemianopic
passed, and patients who failed the test. By means drivers has been reported by various authors [9,
of eye- and head-tracking technology, the explicit 32]. In the Bowers et al. study [24], the authors
visual exploratory behavior could be assessed also quantified head scanning and found that HH
throughout the drive. drivers had impaired detection of blind side sta-
Kübler et al. [5] reported that when all patients tionary pedestrians at simulated intersections,
were analyzed as a group, they showed more either due to not scanning or an insufficient scan
9 Driving with Homonymous Visual Field Defects 141
magnitude. The same authors found that success- and operational handling during driving.
ful detection of a pedestrian moving on a colli- However, they found no indication for a cut-off
sion course in the blind field was associated with point below which all participants were unfit to
a saccadic eye movement towards the target [3]. drive. The majority of the on-road studies includ-
Similarly, in another simulator study, more fre- ing hemianopic and quadrantanopic patients have
quent compensatory saccades to the affected in contrast reported that the extent of hemianopic
side, but no head movements were found for one visual field loss is of minor importance with
patient who had no collisions [34]. Other studies regard to driving performance [4, 20, 38, 40].
investigating visual search behavior of patients Therefore, the extent of visual field per se cannot
with homonymous visual field defects in every- predict fitness to drive. On-road studies that have
day tasks have confirmed the use of compensa- analyzed the gaze behavior of patients with hom-
tory eye and head movements [35]. onymous visual field defects have reported that
some patients are able to compensate for the
visual impairment by means of gaze scanning.
9.2.2 Individual Assessment Indeed, Wood et al. [20] and Kasneci et al. [4]
of Driving Fitness in On-Road reported that patients who passed the on-road
Studies driving test showed a higher percentage of gaze
towards their visual field defect than patients who
The most realistic assessment of individual driv- failed. Furthermore, patients who were rated as
ing fitness can be obtained by means of on-road fit-to-drive demonstrated increased exploration
studies. As summarized in [36], several settings in terms of head and shoulder movements and
have been employed in a variety of research stud- received superior ratings regarding scanning
ies to measure driving performance on open activity. By means of sophisticated eye tracking,
roads (i.e., public roads with natural traffic envi- the authors [4] showed that effective visual scan-
ronment) or closed-road circuits. Here, we focus ning into the area of visual field defect is associ-
on individual assessment of driving fitness in ated with superior driving performance.
patients with homonymous visual field defects. Additionally, in the study of Kasneci et al. [4],
Due to the high effort associated with on-road patients rated as fit-to-drive focused longer on the
studies with patients with homonymous visual central area of the visual field than patients who
field defects, the number of studies conducted so failed the test. This interesting result is in agree-
far is quite low compared to simulated driving ment with a recent study, suggesting a significant
tests. In the following, we will summarize the bias of fixations and viewing time towards the
main outcomes from such studies. center of the screen for both healthy subjects and
Several on-road studies with patients with hemianopic patients during a visual search task
homonymous visual field defects have found in a static display [41]. The authors suggested
poor steering control, incorrect lane position, dif- that this central bias could be related to func-
ficulties in gap judgment, and inadequate detec- tional specialization of the human visual field.
tion of potential hazards to be the primary reasons Saccadic eye movements are performed in order
for failing the on-road driving tests [4, 20, 21, 26, to overcome acuity limitations of the visual field
37, 38, 39]. With regard to the different patient and shift its center to new objects of interest [41].
etiology and experimental setting, the pass rates Several explanations have been suggested for the
reported by these studies show a high variation. tendency to fixate the center of the scene when
However, a consensus is achieved, as all studies freely viewing images. First, the central bias may
consistently report of a subgroup of patients that result from motor biases that favor small ampli-
show driving performance similar to that of con- tude saccades over large amplitude saccades.
trol subjects despite the visual impairment. De Second, the bias may arise from the distribution
Haan et al. [37] reported a positive correlation of image features. In addition, the center of the
between visual field size and viewing behavior screen may be an optimal location for early
142 E. Kasneci and G. Hardiess
i nformation processing of the scene. Alternatively, including a pedestrian detection task in simulated
the center of the screen may be a convenient loca- driving and evaluated 12 patients with homony-
tion from which to start oculomotor exploration mous visual field defects and 12 control subjects.
of the scene. Finally, the central bias may reflect Based on their visual search performance, two
a tendency to re-center the eye in its orbit [42]. subgroups of patients were identified: patients
who were able to ‘adequately’ compensate for
their visual deficit and others who were not. It is
9.3 Conclusion and Future Work noticeable that the ‘adequately’ compensated
group showed better performance than the ‘inad-
In conclusion, the discussed body of work indi- equately’ compensated patient group, although
cates that some patients with hemianopic visual reaction times were slightly slower than controls
field loss, who do not meet the legal requirements [45]. Thus, such a search task [45] can be used to
for driving, are nevertheless judged as fit-to-drive predict the compensation capability of hemiano-
in simulated or on-road tests. This confirms the pic persons to a certain extent.
frequently stated hypotheses that visual field Another promising approach of evaluating the
related parameters per se are inadequate for assess- ability of HH patients to functionally compensate
ment of driving fitness and more individualized for their visual field defect should include work-
approaches are required. Similarly, the prediction ing memory tests. Here, the ability to represent
of driving safety in patients with hemianopic and generate spatial coordinates to plan effective
visual field defects by evaluating the causative saccadic behavior by using memory functions
brain lesion on clinical neuroimaging has not been (i.e., memory guided saccades) would need to be
successful [43]. While imaging studies do suggest analyzed. Indeed, some studies point out that
that certain brain regions are linked with specific memory-guided saccades may serve as essential
parameters of driving performance, this may not compensation strategy in patients with HH [8, 9].
necessarily result in an unsafe driver [43]. Finally, and in light of the above finding, we
Therefore, individualized approaches are required think that standardized tests in driving simula-
to assess the driving fitness of an HH patient. tors, including the detection of potential hazards
Although individualized on-road driving while recording gaze patterns, should be contin-
assessment can be a good means to keep the driv- ued and expanded, since they provide ecologi-
ing license, such tests are quite costly, and there- cally valid measures to individually assess the
fore, not always practical. More research needs to driving as well as the compensation capabilities
be conducted to design simple tests that can pre- of hemianopic drivers [46, 47].
dict the driving performance of individuals. For
example, a new test procedure allowing the
assessment of the so-called exploratory field-of- References
view (EFOV) (i.e, the field-of-view of a person
when eye movements are allowed) was intro- 1. Owsley C, McGwin Jr G. Vision and driving. Vision
Res. 2010;50(23):2348–61.
duced in [44]. In contrast to perimetric proce- 2. Land MF. Eye movements and the control of actions in
dures, during EFOV testing, the subject is everyday life. Prog Retin Eye Res. 2006;25(3):296–324.
encouraged to move the eyes towards the pre- 3. Bowers A, Mandel A, Goldstein R, Peli E. Driving
sented stimulus in order to fixate it. Thus, EFOV with hemianopia, I: detection performance in a driv-
ing simulator. Invest Ophthalmol Vis Sci. 2009;50(11):
testing can capture the visual exploration capa- 5137–47.
bility of a subject and reveal the real impact of a 4. Kasneci E, Sippel K, Aehling K, Heister M, Rosenstiel
visual field defect on daily tasks. Although a W, Schiefer U, et al. Driving with binocular visual
promising approach, detection performance in field loss? A study on a supervised on-road parcours
with simultaneous eye and head tracking. PLoS One.
EFOV and driving performance have not been 2014;9(2):e87470.
linked yet. In a recent work by Smith et al. [45], 5. Kübler T, Kasneci E, Rosenstiel W, Aehling K,
the authors introduced two computerized tests Heister M, Nagel K, Schiefer U, Papageorgiou E.
9 Driving with Homonymous Visual Field Defects 143
Driving with homonymous visual field defects: driv- 20. Wood JM, McGwin Jr G, Elgin J, Vaphiades MS,
ing performance and compensatory gaze movements. Braswell RA, DeCarlo DK, et al. On-road driving
J Eye Mov Res. 2015;8(5):1–11. performance by persons with hemianopia and quadran-
6. Papageorgiou E, Hardiess G, Ackermann H, tanopia. Invest Ophthalmol Vis Sci. 2009;50(2):577–85.
Wiethoelter H, Dietz K, Mallot H, et al. Collision 21. Szlyk JP, Brigell M, Seiple W. Effects of age and
avoidance in persons with homonymous visual field hemianopic visual field loss on driving. Optom Vis
defects un-der virtual reality conditions. Vision Res. Sci. 1993;70(12):1031–7.
2012;52(1):20–30. 22. Tant M, Cornelissen F, Kooijman A, Brouwer WH.
7. Machner B, Sprenger A, Kömpf D, Sander T, Heide W, Hemianopic visual field defects elicit hemianopic
Kimmig H, et al. Visual search disorders beyond pure scanning. Vision Res. 2002;42(10):1339–48.
sensory failure in patients with acute homonymous 23. Lövsund P, Hedin A, Törnros J. Effects on driving
visual field defects. Neuropsychologia. 2009;47(13): performance of visual field defects: a driving simula-
2704–11. tor study. Accid Anal Prev. 1991;23(4):331–42.
8. Martin T, Riley M, Kelly K, Hayhoe M, Huxlin K. 24. Bowers AR, Ananyev E, Mandel AJ, Goldstein RB,
Visually-guided behavior of homonymous hemi- Peli E. Driving with hemianopia: IV. Head scanning
anopes in a naturalistic task. Vision Res. 2007;47(28): and detection at intersections in a simulator. Invest
3434–46. Ophthalmol Vis Sci. 2014;55(3):1540–8.
9. Papageorgiou E, Hardiess G, Mallot H, Schiefer U.
25. Alberti CF, Peli E, Bowers A. Driving with
Gaze patterns predicting successful collision avoid- Hemianopia: III. Detection of stationary and approach-
ance in patients with homonymous visual field defects. ing pedestrians in a simulator. Invest Ophthalmol Vis
Vision Res. 2012;65:25–37. Sci. 2014;55(1):368–74.
10. Hardiess G, Mallot H. Task-dependent representation
26. Wood JM, McGwin Jr G, Elgin J, Vaphiades MS,
of moving objects within working memory in obstacle Braswell RA, DeCarlo DK, et al. Hemianopic and qua-
avoidance. Strabismus. 2010;18(3):78–82. drantanopic field loss, eye and head movements, and
11. Hardiess G, Hansmann-Roth S, Mallot H. Gaze
driving. Invest Ophthalmol Vis Sci. 2011;52(3):1220–5.
movements and spatial working memory in collision 27. Coeckelbergh TR, Brouwer WH, Cornelissen FW, Van
avoidance: a traffic intersection task. Front Behav Wolffelaar P, Kooijman AC. The effect of visual field
Neurosci. 2013;7:62. defects on driving performance: a driving simulator
12. Henderson J. Human gaze control during real-world study. Arch Ophthalmol. 2002;120(11):1509–16.
scene perception. Trends Cogn Sci. 2003;7(11): 28. Schulte T, Strasburger H, Muller-Oehring EM, Kasten
498–504. E, Sabel BA. Automobile driving performance of
13. Kiyonaga A, Egner T. Working memory as internal brain-injured patients with visual field defects. Am
attention: toward an integrative account of internal J Phys Med Rehabil. 1999;78:136–42.
and external selection processes. Psychon Bull Rev. 29. Lundqvist A, Gerdle B, Ronnberg J. Neuropsychological
2013;20(2):228–42. aspects of driving after a stroke – in the simulator and
14. Droll J, Hayhoe M. Trade-offs between gaze and
on the road. Appl Cogn Psychol. 2000;14(2):135–50.
working memory use. J Exp Psychol Hum Percept 30.
Bowers AR, Mandel AJ, Goldstein RB, Peli
Perform. 2007;33(6):1352–65. E. Driving with hemianopia, II: lane position and
15. Hardiess G, Gillner S, Mallot H. Head and eye move- steering in a driving simulator. Invest Ophthalmol Vis
ments and the role of memory limitations in a visual Sci. 2010;
search paradigm. J Vis. 2008;8(1):1–13. 51(12):6605–13.
16. Casson EJ, Racette L. Vision standards for driving in 31. Papageorgiou E, Hardiess G, Wiethölter H, Ackermann
Canada and the United states. A review for the H, Dietz K, Mallot HA, Schiefer U. The neural corre-
Canadian ophthalmological society. Can J Ophthalmol. lates of impaired collision avoidance in hemianopic
2000;35(4):192–203. patients. Acta Ophthalmol. 2012;90(3):e198–205.
17. Silveira S, Jolly N, Heard R, Clunas NJ, Kay L.
32. Hardiess G, Papageorgiou E, Schiefer U, Mallot
Current licensing authority standards for peripheral HA. Functional compensation of visual field deficits
visual field and safe on-road senior aged automobile in hemianopic patients under the influence of different
driving performance. Clin Experiment Ophthalmol. task demands. Vision Res. 2010;50(12):1158–72.
2007;35(7):612–20. 33. Kübler TC, Kasneci E, Rosenstiel W, Schiefer U,
18. International Council of Ophthalmology. Visual stan- Nagel K, Papageorgiou E. Stress-indicators and
dards: vision requirements for driving safety. exploratory gaze for the analysis of hazard perception
International Council of Ophthalmology. 2006. in patients with visual field loss. Transportation
Retrieved from http://www.icoph.org/pdf/visionfor- Research Part F: Traffic Psychology and Behaviour,
driving.pdf. Accessed 4 Mar 2016. 2014;24:231–43.
19. Tant M, Brouwer W, Cornelissen F, Kooijman A. 34. Hamel J, Kraft A, Ohl S, De Beukelaer S, Audebert
Driving and visuospatial performance in people with HJ, Brandt SA. Driving simulation in the clinic: test-
hemianopia. Neuropsychol Rehabil. ing visual exploratory behavior in daily life activities
2002;12(5):419–37. in patients with visual field defects. J Vis Exp.
2012;67:e4427.
144 E. Kasneci and G. Hardiess
35. Kasneci E, Sippel K, Heister M, Aehling K, Rosenstiel visual field is reflected in gaze patterns during visual
W, Schiefer U, Papageorgiou E. Homonymous visual search. Vision Res. 2009;49(2):237–48.
field loss and its impact on visual exploration: A 42. Tatler BW. The central fixation bias in scene viewing:
supermarket study. Translational vision science & selecting an optimal viewing position independently
technology. 2014;3(6):2–2. of motor biases and image feature distributions. J Vis.
36. Owsley C, Wood JM, McGwin Jr G. A roadmap for 2007;7(14):4.1–17.
interpreting the literature on vision and driving. Surv 43. Vaphiades MS, Kline LB, McGwin Jr G, Owsley C,
Ophthalmol. 2015;60(3):250–62. Shah R, Wood JM. Prediction of driving safety in
37. de Haan GA, Melis-Dankers BJ, Brouwer WH,
individuals with homonymous hemianopia and
Bredewoud RA, Tucha O, Heutink J. Car driving per- quadrantanopia
from clinical neuroimaging.
formance in hemianopia: an on-road driving study. J Ophthalmol. 2014;2014:754042.
Invest Ophthalmol Vis Sci. 2014;55(10):6482–9. 44. Tafaj E, Hempel S, Heister M, Aehling K, Rosenstiel
38. Elgin J, McGwin G, Wood JM, Vaphiades MS,
W, Schaeffel F, et al. A new method for assessing the
Braswell RA, et al. Evaluation of on-road driving in exploratory field of view (EFOV). In: Proceedings of
people with evaluation of on-road driving in people the international conference on health informatics
with hemianopia and quadrantanopia. Am J Occup (BIOSTEC 2013). 2013. p. 5–11.
Ther. 2010;64(2):268–78. 45. Smith M, Mole CD, Kountouriotis GK, Chisholm C,
39. Bowers AR, Tant M, Peli E. A pilot evaluation of on- Bhakta B, Wilkie RM. Driving with homonymous
road detection performance by drivers with hemiano- visual field loss: Does visual search performance predict
pia using oblique peripheral prisms. Stroke Res Treat. hazard detection? Br J Occup Ther. 2015;78(2):85–95.
2012;2012:176806. 46. Bowers AR, Alberti CF, Hwang AD, Goldstein R,
40. Racette L, Casson EJ. The impact of visual field loss Peli E. Pilot study of gaze scanning and intersection
on driving performance: evidence from on-road driv- detection failures by drivers with hemianopia. In:
ing assessments. Optom Vis Sci. 2005;82(8):668–74. Proceedings of the eighth international driving
41. Pflugshaupt T, von Wartburg R, Wurtz P, Chaves S, symposium on human factors in driver assessment,
Déruaz A, Nyffeler T, von Arx S, et al. Linking physi- training and vehicle design. New York: Bolton
ology with behaviour: functional specialisation of the Landing; 2015. p. 240–6.
Neurological
and Neuropsychological 10
Investigation in Patients
with Homonymous Visual Field
Defects
Martin Pail, Sabina Goldemundová,
Karolína Skorkovská, and Milan Brázdil
Abstract
Visual information is transferred via the optic pathway and processed by an
interaction of the striate cortex with visual associative areas. These areas
evaluate different properties of the signal and contribute to an overall per-
ception of the visual environment. Lesions of associative cortices in humans
cause the so-called central visual disorders that may accompany homony-
mous visual field defects. An understanding of the functions of these areas
is important, as many of these patients will first contact an ophthalmologist,
usually with vague complaints that may be difficult to specifically define.
This chapter gives an overview of central visual disorders that can be found
in patients with homonymous visual field defects and of the principles of
neurological and neuropsychological examination in these patients.
Keywords
Central visual disorder • Visual cortex • Associative area • Agraphia • Alexia •
Agnosia • Achromatopsia • Neglect • Allesthesia • Akinetopsia • Anomia •
Blindsight • Visual attention • Visual hallucination • Visual aura migraine •
Neuropsychology
Fig. 10.1 Parallel visual processing pathways in the geniculate nucleus, pars dorsalis, Pulv pulvinar, SC superior
human. Diagram of the major routes passing from the colliculus) (Artist: David Fisher. From Goodale et al. [1],
retina into the dorsal and ventral streams (LGNd lateral with permission)
10 Neurological and Neuropsychological Investigation in Patients with Homonymous Visual Field Defects 147
the inferior temporal lobe (object identification), fluid, electroencephalography (EEG), electro-
and limbic structures. The ventral pathway is myography (EMG), evoked potentials (EP)
often called the “what” pathway because it serves studies, etc. Moreover, in certain cases specific
object recognition. The dorsal pathway projects auxiliary diagnostic methods (clinical neuro-
to the posterior parietal cortex and superior tem- physiology, biochemistry or molecular genetics)
poral cortex (visuospatial analysis) and then may be helpful.
continues forward to the premotor frontal cortex [2] The basic task is to detect whether a given patient
This dorsal or “where” pathway serves the local- has suffered damage to the peripheral or central ner-
ization of objects in visual space and their move- vous system and to determine the location of the
ment. Naming the what and where pathway of damage as precisely as possible. Associated focal
visual processing is an oversimplification of how neurological signs and symptoms, as well as visual
these cortical areas function as there is a lot of field characteristics, may help to determine the site
interaction among the individual regions as well. of the causative brain lesion. Also, the “general”
However, it serves as a good clinical model of examination of the patient is as important as the
cortical visual processing. Damage to the ventral neurological part and may help to reveal the diagno-
stream results, for example, in visual object sis. Only with all necessary data can a differential
agnosia, pure alexia, anomia, prosopagnosia, and diagnosis, diagnostic evaluation, and treatment plan
achromatopsia. Bálint syndrome, visual inatten- be generated. However, quite frequently the situa-
tion, and hemispatial neglect are examples of tion may be impeded by the fact that the clinical
dorsal stream disorders [2]. picture in a given patient is expressed incompletely,
or combines symptoms of impairment at both the
peripheral and central levels.
Taking the patient’s history is the most impor-
10.3 P
rinciples of Neurological tant part of a neurological examination and should
Examination be performed prior to all other procedures unless it
proves impossible (i.e., the patient is unconscious).
The diagnostic balance sheet in neurology The time profile of visual problems (temporary or
proceeds from the detection of symptoms to a permanent) and mono- and binocular nature of the
syndromological, topical, and nosological diag- disorder are essential for the localization and etiol-
nosis, or ideally to the disclosure of the etiology ogy of the disability. Acute and subacute disorders
of the disorder. Syndromological diagnosis usually have a vascular and inflammatory origin,
means a summary of the various manifestations while chronic and progressive disorders are usu-
of the disease (subjective symptoms and objec- ally caused by compression, infiltration, or a
tive signs revealed during a neurological and degenerative process. Understanding the neuro-
neuropsychological examination) grouped in a logical condition of the patient at the time of ques-
combination characteristic for a particular syn- tioning is important to get an idea as to which
drome. Topical diagnosis defines the level of neurological systems could be affected. Then it is
impairment within the peripheral or central ner- necessary to focus on the neurological examina-
vous system. Nosological diagnosis describes tion and to investigate in detail the functions whose
the disease by means of the detected neurologi- disability is suspected.
cal syndrome, clinical course of the disease,
response to treatment, etc. Usually, the cause of
the disease cannot be determined with certainty 10.3.1 Specific Tests of Visual
only from the patient’s history and clinical Function
examination. To verify a clinical suspicion, it is
often necessary to perform other tests such as Visual field test Visual field testing is used to
computed tomography (CT), magnetic reso- detect signs of pre- and postchiasmatic damage to
nance imaging (MRI), examination of cerebrospinal the visual pathways. Visual field defects may be
148 M. Pail et al.
Fig. 10.2 Visual evoked potentials wave patterns recording is 200 ms, average responses number in one
(R-VEP – pattern reversal VEP) of a healthy volunteer recording is 200, 2 runs)
(bipolar montage Oz-Cz, O1-Cz, O2-Cz, length of the
plotted in a number of ways: confrontation visual visual cortex. The light-evoked signal, small in
field, tangent screen, automated static, or amplitude and hidden within the normal elec-
Goldmann kinetic perimetry [3, 4]. Simple, bed- troencephalographic (EEG) signal, is amplified
side confrontational testing of the visual field is by repetitive stimulation and time-locked, sig-
even nowadays the most important component of a nal-averaging techniques, separating it from the
basic neurological examination. Confrontational background EEG readings. The VEP comprises
visual field examination should be performed by a series of negative and positive waves, of which
an experienced neuro-ophthalmologist or neurolo- the most reliable and useful is the P100 wave
gist using hand movement, finger counting, and elicited usually with a latency of close to 100 ms
color comparison (red saturation across the verti- after the stimulus presentation (Fig. 10.2). VEPs
cal meridian). Each eye should be tested separately are used to measure the functional integrity of the
as the patient looks at the examiner’s eye, and visual pathways from retina to the visual cortex of
stimuli should be presented in each quadrant. This the brain. Any abnormality that affects the visual
method will, however, detect only absolute visual pathways or visual cortex can affect the VEP
field defects. Subtle visual field defects such as (increase the latency or decrease the amplitude of
small central scotomas are likely to be missed. the individual peaks). Flash stimulus VEP is more
Double simultaneous stimulation may reveal useful in patients with substantial visual loss.
visual neglect, which may or may not be accompa-
nied by a homonymous visual field defect.
well-recognized tests used in everyday clinical in terms of the Rey Complex Figure Test).
practice can help reveal central visual disorders. Observing the patient in such situations can pro-
The horizontal line bisection task is a common vide a lot of information. We should also ask
clinical test introduced in 1894 by the German about the patient’s left-right orientation.
physician Axenfeld, who published a case report Homonymous hemianopia may occur
about line bisection as a “simple method to diag- together with visual inattention (visual neglect),
nose hemianopia” [5]. The examiner draws a line but these conditions are not necessarily linked.
on a paper in front of the patient asking him to Observing the patient in daily life can help us to
make a mark right in the middle of the line. The distinguish the two entities: a woman with
score is the length by which the patient’s esti- neglect would comb just half of her hair, in case
mated center deviates from the actual center. It of hemianopia she would turn her head (and
should be pointed out that even normal subjects eyes) during combing so that she can see better.
tend to make the mark a little to the left (1 or There may also be other symptoms of hemispa-
2 mm). It also depends on the length of the line – tial inattention apart from visual deficit; for
the longer it is, the more deviation from the cen- example, patients may not react to sound stim-
ter there may be [6]. uli coming from the ignored side, etc. Patients
The symbolic functions should be examined with neglect are not able to compensate their
by asking the patient to read a text aloud and to deficit (in contrast to those with hemianopia).
write something spontaneously or by dictation. Again, there are tests used to diagnose different
The patient should describe a complex picture symptoms associated with unilateral visual
like the cookie theft picture from the Boston neglect like the star cancelation, key cancel-
diagnostic aphasia examination (Fig. 10.3) [7], ation, line crossing, and apples cancelation
draw a picture (a human figure, a flower, a tree, a (Fig. 10.4) [8]. Another test used for the evalu-
house, do the Clock Test, or copy a figure, e.g., ation of neglect is the Bells Test. First, the
patient becomes familiar with the target figure
(the bell) and all the distractors. Then, on the
task sheet the patient is asked to circle all the
bells in the picture. The scoring sheet (Fig.
10.5) [9] is divided into seven parts (one cen-
tral, three to the left, three to the right). The
score consists of the number of circled bells
and the time necessary for completion of the
test [9]. The use of distractors instead of line
crossing enables detection of mild and moder-
ate neglect more readily because the patient has
to develop a consistent search strategy [10].
Sometimes in a patient with visual problems a
possible psychogenic cause or simulation may be
Fig. 10.3 The “cookie theft picture” from the Boston suspected. One of the most common psychogenic
Diagnostic Aphasia Examination. The information in the
picture is scattered into four visual field quadrants. disturbances in ophthalmology is decreased
A patient is asked to describe the events in the picture. visual acuity [11]. Psychogenic visual field
A person with simultanagnosia is able to describe only defects may present as homonymous hemiano-
disconnected fragments of the scene such as the cookie jar pia, though this is rather rare. After excluding an
or the faucet and is not able to describe the events related
to the scene (From Goodglass et al. [7], with permission) organic cause, we should calm the patient with
150 M. Pail et al.
a b
G TEN
E DAY GET
J
L HER E N
C READ E
MAN
U O N
LEG M LEG
ARE
c d
Fig. 10.4 Examples of tests frequently used to diagnose sheet of paper – deficits are measured by target omis-
heterogeneous symptoms associated with unilateral sions on either left or right side of space. Gap detection
visual neglect, which can provide measure of deficits tests: (d) apples cancelation, administered by asking
associated with impaired control of attention either (a–c) patients to cross only full targets (full circles or full
across space, i.e., egocentric frame of reference and/or apples, respectively) evenly distributed on the centrally
(d) within objects, i.e., allocentric frame of reference. placed sheet of paper – deficits are measured by counting
Common cancelation tests: (a) star cancelation, (b) key missing targets on either left or right side of space as
cancelation, and (c) line crossing, all administered by well as false-positive responses, i.e., crossing objects
asking patients to cross targets (small stars, keys, or lines with either left or right openings (From Chechlacz et al.
respectively) evenly distributed on the centrally placed [8], with permission)
reassurance that the situation will eventually Last but not least, homonymous hemianopia
improve. Most patients report they are able to may be associated with the impairment of daily
manage daily life activities despite their visual activities, particularly with difficult orientation
problems; therefore there is no need to intervene. to surroundings. Patients may fear falling, slip-
If the patient’s situation is complicated, we ping, or running into objects. Consequently,
should recommend a more thorough psychologi- they may prefer to stay isolated at home, not
cal examination and follow-up psychotherapy. In risking the danger outside. This can cause seri-
case of simulation, mostly it is not necessary to ous mental problems like panic attacks, pho-
confront the patient, but the situation should be bias, and depression that should not be
clarified. underestimated.
10 Neurological and Neuropsychological Investigation in Patients with Homonymous Visual Field Defects 151
10.5 Neuropsychological injured optic tract, and a temporal optic disc p allor
Symptoms and Syndromes is seen ipsilateral to the lesion. Either a complete
Associated with or an incomplete homonymous h emianopia can
Homonymous Visual Field be seen in patients with optic tract lesion, and
Defects about half of the optic tract lesions manifest by
congruous homonymous hemianopia [13].
Homonymous visual field defects may be accom- Involvement of the hypothalamus, pituitary infun-
panied by different neuropsychological symp- dibulum, or pituitary gland (structures adjacent to
toms and syndromes that depend on the location the optic tract) may lead to autonomic or endo-
of the lesion. Lesions of the associative areas crine dysfunction. If cerebral peduncles are
often go unnoticed and, unlike the HVFDs, may affected, contralateral hemiparesis may be pres-
be only temporary. Still, they can significantly ent. Disturbances of memory and seizures may
affect patient’s life. occur with impairment of the temporal lobe [3].
LGN by the anterior choroidal artery and the lat- in dementia caused by Creutzfeldt-Jakob disease,
eral posterior choroidal artery. The impairment of progressive multifocal leukoencephalopathy, or
nearby structures may result in superimposed Alzheimer disease [2, 3, 12, 14].
neurological deficit like contralateral hemipare-
sis, hemianesthesia, central pain, etc. [3].
10.5.4 Lesions of the Occipital Lobe
(Area V1-V5)
10.5.3 Lesions of the Optic Radiation
Unilateral lesions of the occipital lobe typically
Lesions of the optic radiation in the temporal lobe lead to congruent contralateral homonymous
lead to contralateral upper homonymous quadran- hemianopia with a typical central (macular) spar-
tanopia, while lesions located in the parietal lobe ing. Incomplete homonymous hemianopia is
result in contralateral lower homonymous more common than complete hemianopia, and
quadrantanopia. In case of a complete lesion of homonymous quadrantanopia is the most com-
the optic radiation a contralateral homonymous mon type of incomplete homonymous hemiano-
hemianopia is present. A varying degree of
pia [14]. Explanation of the preserved central
hemiparesis or hemianesthesia may also be found vision is twofold: (1) the macular visual cortex is
due to damage to the internal capsule [2, 3]. a “watershed zone” receiving dual blood supply
Hemianopia caused by lesions of the optic from the middle and posterior cerebral arteries,
radiation may be accompanied by other hemi- and (2) double-sided representation of the mac-
spheric symptoms that may be revealed with ula in the cortex [3]. Bilateral cortical lesions
detailed neurological testing. Temporal lobe above or below the calcarine fissure lead to altitu-
lesions lead to defects of personality, temporal dinal hemianopia respecting the horizontal dis-
epilepsy, memory disturbances, Wernicke apha- secting line.
sia, or Klüver-Bucy syndrome (hypersexuality, Contralateral homonymous hemianopic cen-
hyperorality, visual and auditory agnosia and tral scotoma is indicative of a unilateral lesion of
apathy). Dominant parietal lobe lesions manifest the visual cortex with preserved visual acuity;
with conductive aphasia, Wernicke aphasia, nevertheless, vision is reduced in bilateral affec-
alexia with or without agraphia, Gerstmann syn- tions of the pole. Generally, both-sided retrochi-
drome (finger agnosia, agraphia, acalculia, fail- asmal lesions are associated with a varying
ure of left-right orientation), or tactile agnosia. degree of decreased visual acuity; however, the
Nondominant parietal lesions manifest by a left- decrease is symmetrical. In case of an asymmet-
hand syndrome, neglect, or constructional apraxia ric decrease of visual acuity, the presence of pre-
and apraxia of dressing in association with left- chiasmatic lesions can be suspected. Patients
sided hemianopia. Patients with parietal lobe with a hemianopia due to occipital ischemia usu-
lesions involving the optic radiation are often ally have normal optokinetic nystagmus, whereas
unaware of their visual field defects. This condi- in occipital tumors with edema extending to the
tion is called anosognosia and is most often seen parietal lobe, optokinetic nystagmus is missing.
with nondominant parietal lobe lesions. Cortical blindness is the result of a bilateral
Moreover, dysfunctions of higher sensory inte- lesion of the visual cortex. Patients do not react
gration may be discovered, including astereogno- with blinking upon a rapid approach of an object
sis, agraphesthesia, and impaired two-point or person, and optokinetic nystagmus cannot be
discrimination. Deep parietal lesions may lead to elicited. Some patients may experience photop-
homonymous hemianopia and abnormalities in sias or other visual hallucinations in the blind
smooth pursuit eye movements to the ipsilateral hemifield [12]. Charles Bonnet syndrome is the
side. Visual disturbances in lesions of the parieto- name for visual hallucinations that frequently
occipital border or the occipital lobe itself – occur in patients who lose vision in both eyes,
Bálint syndrome or cortical blindness – can occur regardless of the location of the causative lesion.
10 Neurological and Neuropsychological Investigation in Patients with Homonymous Visual Field Defects 153
The hallucinations may be simple (e.g., brief visual pathway (see Fig. 10.1) involving particu-
flashes of light, phosphenes, various shapes etc.), larly the superior colliculus and pulvinar [19, 20]
or complex (specific objects, people, country- or, alternatively, by a pathway from the LGN to
side, animals, etc.). Patients are generally aware the associative visual areas that bypasses the stri-
that the images are not real, but they can cause ate cortex [22, 23]. Considerable controversy still
significant anxiety, and thus patients should be surrounds the existence of blindsight in humans
educated regarding the benign nature of this syn- and further studies are needed to explain this
drome [12]. phenomenon.
Some patients may confabulate visual percep- Cerebral achromatopsia is a rare acquired
tion to mask their visual loss or deny their blind- defect of color perception, usually as a result of
ness. This is called Anton syndrome or visual damage to the ventromedial visual cortex. It may
anosognosia. The patient cannot see and bumps be complete (which is rare) or affect only one
into objects while walking, but he adamantly hemifield [24]. These patients cannot match up
behaves as if he could see. It is an uncommon the colors of the surrounding world but see the
form of anosognosia that may follow extensive environment completely devoid of color, only
damage to the striate cortex [15]. Although Anton black and white, in shades of gray, or washed out
syndrome usually accompanies geniculostrate [23]. If discrimination of the main colors is pre-
lesions, it may occur from any etiology, including served, but the ability to discriminate subtle col-
blindness from prechiasmal disorders such as ors is reduced, then it is referred to as cerebral
optic neuropathies and retinal detachment [2]. (hemi)dyschromatopsia [25]. Most often, the
There are several theories regarding the etiology pathology in patients with achromatopsia is
of Anton syndrome. Denial of blindness may be localized in the posterior fusiform and lingual
due to damage to higher cognitive areas or due to gyri [23]. Cerebral achromatopsia may be associ-
lesions of the geniculostriate pathway that dis- ated with a superior homonymous visual field
rupt input to and also interfere with output from defect from damage to the inferior striate cortex.
the visual cortex to areas involved in the con- In such cases, the residual inferior field on that
scious awareness of visual perception [16]. side is achromatopic [2]. Achromatopsia may be
Further, patients with Anton syndrome often have accompanied by other symptoms like prosopag-
altered emotional reactivity similar to patients nosia, topographagnosia, visual object agnosia,
with frontal lobe lesions [17]. pure alexia, and defects of visual memory [2],
Other symptoms of impaired visual cortex resulting from a lesion of the ventral occipitofu-
function include the covert vision sign [18], gal pathway. If the lesion extends to the temporal
unaware vision (blindsight), or impaired percep- lobe, global amnesia may be present as well [2].
tion of static objects with preserved perception of Due to intact chromatic pathways from the retina
moving stimuli in the blind hemifield (Riddoch to the striate cortex, patients with acquired cere-
phenomenon) [19, 20]. Blindsight refers to a con- bral achromatopsia show, in comparison with
dition sometimes observed in humans who expe- congenital achromatopsia, preserved trichromacy
rience severe damage to one or both occipital and intact cortical responses to chromatic visual
lobes. Under experimental conditions the direc- evoked potentials [26]. Because of preserved
tion of moving objects in the blind hemifield is function of the wavelength-selective cells in the
correctly given by the patients, colors recognized, striate cortex, achromatic patients may perform
or a target object and its displacement is detected. well in tests with pseudoisochromatic plates [27].
The significance of this phenomenon is topically Akinetopsia (also mentioned under the dorsal
low [2]. Some authors attribute these perceptions pathway disorders) is a rare disorder usually
to preserved islands of striate cortex [21] or mul- associated with damage to the ventrolateral
tifocal damages [18]. Blindsight may also be occipital gyri or attributed to a bilateral lesion of
explained by the transmission of visual signals the parieto-occipito-temporal transition of area
through the primitive extrastriate subcortical V5. Patients with akinetopsia are unable to detect
154 M. Pail et al.
motion. They perceive moving objects as s yndrome is the sign of damage to the right pari-
stationary objects that suddenly appear, disap- etal lobe. Additionally, bilateral lesions of the
pear, and jump from one place to another and so parieto-occipital area (important brain regions
change their position [28]. Also in this case, pos- for foveal fixation and visual attention) cause the
sible hemiakinetopsia may be associated with so-called Bálint syndrome. It is usually accompa-
incomplete homonymous field defects and so be nied by lower altitudinal hemianopia due to the
obscured. currently affected area above the calcarine
Visual aura migraine is localized in one hemi- fissure.
field (corresponding to the impairment of the pri-
mary visual cortex of one occipital lobe) and is Lesions of the Dorsal Occipito-Parietal
characterized by a scotoma and irritative symp- Pathway The dorsal or “where” pathway begins
toms like scintillations, zig-zag lines, and phos- in area V1 and projects through V2 and V3 to
phenes. These types of visual disturbances tend area V5 (area MT). The information is conveyed
to start in the center of the visual field and move along the dorsal longitudinal fascicles to the pos-
outward, or spread. Quite typical is a gradually terior parietal cortex, frontal motor areas, and
expanding, scintillating scotoma with flickering frontal eye fields (FEF) [1]. The posterior parietal
light on the edge. Visual aura migraine usually cortex combines characteristics of both motor
occurs within an hour before the onset of head- and sensory areas and serves as a junction
ache and lasts less than 60 min. Unlike classical between multimodal sensory input and motor
migraine, retinal migraine occurs as a transient output [1]. This pathway is associated with spa-
visual loss in the visual field of the affected eye. tial localization, visuomotor search, guidance
Other symptoms of central visual disorders (guidance of eye, head and arm movements), and
include polyopia (multiple vision, perception of visuospatial synthesis [29]. Lesions of the
one object as few), palinopsia (persistence of occipito-parietal pathway cause visual hallucina-
visual perceptions), and optical allesthesia tions and disturbances of visual attention and of
(abnormal orientation of objects in space). the processing of objects in space [30].
Hemianopia caused by occipital lobes ischemia Bálint syndrome is defined as a combination
due to vertebrobasilar thrombosis may be associ- of acquired oculomotor apraxia, simultanagno-
ated with brainstem (especially oculomotor nerve sia, and optic ataxia. These individual symptoms
lesions) and cerebellar symptoms. do not necessarily occur together, but may be
detected in isolation or in association with other
disorders of visuospatial perception [31, 32].
10.5.5 Lesions of the Associative Bálint syndrome is caused by a bilateral lesion
Visual Areas of the parieto-occipital regions, which are impor-
tant for attention and visual foveal fixation [32].
In the case of lesions of the associative visual Symptoms are usually accompanied by lower
areas, symptoms occur that cannot be explained altitudinal hemianopia due to the simultaneously
by failure of the primary visual cortex only. For affected area above the calcarine fissure. Acquired
example, when a lesion affects the splenium of oculomotor apraxia or spasm of fixation is char-
the corpus callosum or the adjacent periventricu- acterized by the loss of voluntary eye movements
lar white matter of the dominant hemisphere, with persistence of fixation on a target [2]. In con-
then pure alexia as part of the dysconnection syn- trast to congenital oculomotor apraxia, saccades
drome may occur (the inability to recall vocabu- are easily made to peripheral targets in the absence
lary for visual information processed in the intact of a fixation target. Other oculomotor symptoms
right occipital lobe). A bilateral lesion of the include defective smooth pursuit eye movements
medial temporo-occipital area (impaired lower and impaired optokinetic nystagmus in both
longitudinal fascicle) results in visual agnosia as directions, while volitional saccades are relatively
well as in prosopagnosia. Left-sided neglect preserved, but are hypermetric and inaccurate.
10 Neurological and Neuropsychological Investigation in Patients with Homonymous Visual Field Defects 155
Patients with simultanagnosia can perceive whole and left side of the visual field, but only the
shapes, but they cannot recognize whole scenes right stimulus is p erceived if both stimuli are
because they are unable to shift visual attention. presented at the same time (i.e., extinction sen-
Patients with this condition require multiple sation). Severe forms of neglect are usually
fixation, and they complain of tunnel vision or caused by ischemia in the territory of the right
of the sudden appearance or disappearance of middle cerebral artery and damage to two-
objects. They have problems when reading or thirds of the parietal lobe. It disturbs all percep-
interpreting images while performing an action tion of left-sided stimuli, including body
[31, 33, 34]. They thus behave as if they were sensations (hemiasomatognosia); head and eyes
blind even though they have intact visual fields. are twisted to the right, and while looking left,
Simultanagnosia can be diagnosed, for example, the eyeballs do not cross the medial line. Unlike
by the “cookie theft picture” (see Fig. 10.3). This hemianopic patients, patients with neglect syn-
scene requires higher-order synthesis of multiple drome are usually capable of capturing elemen-
objects scattered throughout four quadrants of the tary visual stimuli from the left half of the
picture to achieve a global understanding of the visual field (light stimuli during a perimetric
image. Optic ataxia means the inability to grasp an examination), and visual evoked potentials
object during visual inspection or, in other words, show normal results. Left-sided neglect mani-
to perform accurate limb movements under visual fests with mistakes when drawing a picture
control. Thus, patients reach for targets within an (Fig. 10.6) – e.g. in the clock test the patients
intact field as if they were blind. tends to place all digits or most of them into the
Unilateral (or hemispatial) neglect is a neu- right half of the dial [39]. When the patient is
ropsychological disorder of attention, percep- asked to split a horizontal line in half, the
tion, and orientation in one half of the space, patient places a hyphen to the right of the cen-
usually left, without breaking the primary ter. Similarly, in the cancellation test the patient
motor and sensory functions [2, 35–37]. crosses out certain characters only in the right
Hemispatial neglect may affect multiple sen- half of the paper. Neglect syndrome often
sory modalities, but visual inattention is often remains undiagnosed, and affected patients are
the most prominent feature. Left-sided neglect not recognized. Past studies, however, show
syndrome is a sign of the right nondominant that the marginalization of half of the space and
hemispheric lesion that may occur without left- other difficulties associated with the reported
sided hemianopia. However, extensive parietal diagnosis often limit the patient more seriously
lobe lesions usually lead to both neglect syn- than, for instance, disorders of speech and
drome and hemianopia. In these cases, it is right-sided hemiparesis [36].
rather difficult to prove neglect. Besides right Hemiasomatognosia is part of the neglect syn-
parietal lesions, neglect syndrome may also be drome in which the patient does not recognize the
due to the damage of the left parietal lobe, the left (rarely, the right) half of his body or its parts.
right prefrontal areas, the cingulate gyrus, stria- The patient, e.g., shaves only the right half of his
tum, thalamus, and posterior arm of the internal face, his left extremity seems foreign (belonging
capsule [38]. Hemianopic patients have diffi- to another person lying in the bed), etc. He also
culties on the side of the visual field defect due generally neglects the left half of the space and
to impaired exploration, while neglect patients may suffer from a simultaneous visual hemifield
seem to lose awareness of space on the affected disturbance. The syndrome is a manifestation of
side. The least serious form of disability, a right parietal lobe lesion and usually is accom-
reflecting a focal lesion of the right parietal panied by a left-sided hemiparesis.
lobe, shows in diagnostic tests often only dur- Visual allesthesia is a disorder of visuospatial
ing double simultaneous stimulation. That perception in which the retinotopic visual field is
means that a patient with left-sided neglect is rotated, flipped, or even inverted. The lesion is
able to perceive individual stimuli in the right usually localized in the right occipito-parietal
156 M. Pail et al.
12 1
11
10 2
9 3
8 4
7 5
6
Fig. 10.6 Neglect syndrome: the drawings are missing their left halves, which is due to damage to the right parietal
lobe (From Brown [39])
10 Neurological and Neuropsychological Investigation in Patients with Homonymous Visual Field Defects 157
area. An explanation for this phenomenon is not agnosia have impaired object recognition due to
clear; there may be an error in the integration of perceptual difficulties in which elementary visual
visual information with signals from the vestibu- function remains intact [2]. Patients are unable to
lar system [2]. integrate visual information to form an internal
image of an object and have difficulty matching,
Lesions of the Ventral Occipito-Temporal copying, and recognizing even simple shapes.
Pathway The ventral occipitofugal or “what” These patients often have visual field defects, but
pathway is conducted mainly via the inferior lon- their perceptual deficit cannot be explained by
gitudinal fascicles. It originates in V1 and proj- the field loss. Apperceptive agnosia usually
ects through V2 and V4 to specific inferior develops in association with diffuse lesions to the
temporal cortical areas, the angular gyrus, and posterior parts of brain. In contrast to appercep-
limbic structures. Lesions of the occipito- tive agnosia, patients with associative agnosia are
temporal pathway cause primarily visuoassocia- unable to identify objects or categories of objects
tive deficits and hallucinations [30]. Lesions of visually; however, they have intact perception
this pathway are often divided into three types of and can draw and match objects [40].
disconnection syndromes: (1) visual-visual dis- Patients with dorsal simultanagnosia can per-
connection (agnosia), (2) visual-verbal discon- ceive whole shapes, but their perception of these
nection (alexia, anomia), and (3) visual-limbic shapes is restricted to a single visual area because
disconnection (deficits in visual memory and of their inability to shift visual attention. Patients
emotion) [2]. However, the separation between fail to integrate information into a global image;
these cerebral processes involved in visual per- they describe only fragments of a scene.
ception, object identification, and naming is not Peripheral visual field defects may simulate
distinct and patients seldom display completely simultanagnosia and must be excluded [2]. This
isolated manifestations of these syndromes [2]. symptom results from bilateral lesions to the dor-
Also, all lesions are often associated with upper sal occipitofugal pathway and was initially con-
homonymous unilateral or bilateral visual field sidered as part of Bálint syndrome (see above).
defects from extension of the damage to the infe- Ventral simultanagnosia may be another conse-
rior striate cortex. quence of a lesion to the ventral occipitofugal
Visual agnosia is a disorder of object recog- pathway, particularly to the left inferior temporal
nition that cannot be explained by a sensory or region of the brain. In this case patients are able
speech disorder or global cognitive dysfunction. to shift attention to multiple objects, in contrast
A patient with visual agnosia cannot recognize to dorsal simultanagnosia; however, they cannot
seen objects, and can provide neither the name integrate single components into a whole object
nor the associative features of an object, like [36]. For example, they are unable to recognize a
describing its function. However, patients can complex object like a car, even though they can
recognize these objects if they can touch them identify the components of the car, such as the
or hear them, for example, the characteristic tires.
sound of a bunch of keys. Visual agnosia is a Prosopagnosia (“face blindness”) is a special
sign of bilateral affection of the medial occipito- form of agnosia, manifesting by the inability to
temporal areas involving both lower longitudi- identify familiar faces or to memorize new ones
nal fascicles connecting the primary visual [41]. However, the affected person is able to
cortex with visual associative areas within the identify the appropriate person by other distin-
temporal lobe. guishing marks, such as by voice. Prosopagnosia
The general mechanism of agnosia is a disor- is usually due to bilateral damage to the inferior
der of association (associative agnosia) or inte- portions of the occipitotemporal cortex, notably
gration (apperceptive agnosia) of sensory stimuli the lingual and fusiform gyri. As with all lesions
ensued from loss, breach, or unavailability of the in the ventral stream, prosopagnosia is often
relevant subject image (memory trace) in the associated with superior homonymous unilateral
association cortex [40]. Patients with apperceptive or bilateral visual field defects from extension of
158 M. Pail et al.
damage to the inferior striate cortex. Often a left extensive lesions of the left angular gyrus may also
homonymous hemianopia is present [2]. cause—apart from alexia with agraphia—the so-
Visual-spatial agnosia manifests by impaired called Gerstmann syndrome: finger agnosia, acal-
orientation in space, loss of topographical culia, and failure of left-right orientation [2, 33].
memory, and constructional apraxia. The patient Lesions of the splenium (callosal disconnection)
is unable to trace images, especially if they show may cause a left hemialexia associated with other
spatial relationships, and usually suffers from signs such as tactile anomia and agraphia with the
other disorders, such as finger agnosia, apraxia of left hand [3, 33]. Additionally, depending on the
dressing, etc. The underlying lesion is usually in extension of the lesion, other properties conveyed
the posterior part of the right (nondominant) pari- by the ventral occipitofugal pathway may be
etal lobe. affected, resulting in agnosia and memory deficits
Object anomia should be distinguished from that may be easily confused with anomia [2]. If
object agnosia and is characterized by a general- disturbances of spontaneous speech; speech
ized defect in visual naming. The patient is able understanding; and impaired repetition of speech,
to describe objects and their function but is writing, and spelling are noted, it is necessary to
unable to recall the names of objects, as long as consider aphasic alexia, where reading is second-
he sees them only. Only the touch or a character- arily involved [2, 33].
istic noise leads to the recall of the object name
[33]. Object matching and recognition are intact.
Color anomia is the inability to name colors. 10.5.6 Functional Visual
There is no achromatopsia (isochromatic tests are Disturbances
recognized successfully) and no agnostic deficit.
Color anomia may occur in conjunction with an Functional visual disorders are relatively com-
aphasic disorder and pure alexia. The semantic mon, representing up to 5% of all visual difficul-
recall of colors remains intact, and so the patient ties for which patients visit an ophthalmologist or
can identify colors by the colors of known objects a neurologist. They can be either unconscious
(the color, for example, of an orange, banana, or (dissociative disorder) or deliberate (simulation).
tomato). The most commonly reported problems include
Patients with pure alexia (alexia without bilateral or unilateral blindness, visual field
agraphia) suffer from the loss of reading ability defects, and monocular diplopia. The most com-
(even words they have just written) with preserved mon pretended visual field loss is concentric
language, retained speech and writing skills [42]. visual field loss and hemifield defects. Several
Some patients are completely unable to identify tests may help distinguish functional from
words, letters, or symbols. Other patients are able organic visual loss:
to read letters and identify words by tracing and
letter-by-letter reading strategy [33]. Affected • Absence of a linear improvement or deteriora-
patients usually have a right homonymous hemi- tion in vision by changing the test distance;
anopia, or the defect is limited to the upper quad- e.g. in organic concentric visual field loss the
rant. Many cases of pure alexia are overlooked or visual field will enlarge if the test distance is
wrongly attributed to the hemianopic defects fre- doubled. In feigned concentric visual field
quently seen in these patients. However, the alexia loss, the borders of the visual field will be
is not caused by the visual field defect only, but given by the patient as previously so that the
rather by disruption of visual inputs to higher order so-called tunnel vision can be observed.
linguistic centers. Pure alexia, similarly to color • Normal color and spatial vision (stereopsis).
anomia or object anomia, results from the discon- • Normal defensive response (blink reflex) to
nection of visual inputs to the dominant angular quickly approaching hands.
gyrus from damage to the left striate cortex and the • Normal optokinetic nystagmus.
splenium of the corpus callosum usually due to • Normal VEP and mfVEP (however, abnormal
ischemia of the left posterior cerebral artery. More VEP does not confirm an organic cause, since
10 Neurological and Neuropsychological Investigation in Patients with Homonymous Visual Field Defects 159
abnormalities can be achieved on purpose 6. Lezak M, Howieson DB, Loring DW. Neuropsychological
assessment. New York: Oxford University Press; 2004.
by eccentric fixation or convergence and
p. 377.
accommodation). 7. Goodglass H, Kaplan E, Barresi B. BDAE-3 Long
• Normal electroretinography and electro- form stimulus cards picture book 11853. In: Boston
encephalography. diagnostic aphasia examination. 3rd ed. (BDAE-3).
Austin: PRO-ED; 2000.
• Absence of RAPD in unilateral vision loss.
8. Chechlacz M, Rotshtein P, Humphreys GW.
Neuroanatomical dissections of unilateral visual
Conclusion neglect symptoms: ALE meta-analysis of lesion-
Homonymous hemianopia is an indicative of symptom mapping. Front Hum Neurosci. 2012;6:230.
doi:10.3389/fnhum.2012.00230.
a retrochiasmal disease, and is one of the
9. Gauthier L, DeHaut F, Joanette Y. The Bells test: a
most common features of central nervous quantitative and qualitative test for visual neglect. Int
system damage. However, this visual disabil- J Clin Neuropsychol. 1989;11(23):49–54.
ity may be accompanied by changes in the 10. Strauss E, Sherman EM, Spreen O. A compendium of
neuropsychological tests, administration, norms and
perception of the visual environment that
commentary. 3rd ed. Oxford/New York: Oxford
cannot be explained by the visual field defect University Press; 2006.
only. They are caused by concurrent damage 11. Miller NR. Neuro-ophthalmologic manifestations of
to associative visual areas of the brain and psychogenic disease. Semin Neurol. 2006;26(3):
310–20.
may dispose the patient to severe handicap.
12. Goodwin D. Homonymous hemianopia: challenges
Neurological and neuropsychological exami- and solutions. Clin Ophthalmol. 2014;22(8):
nation is crucial in the investigation of 1919–27.
patients with homonymous hemianopia. 13. Kedar S, Zhang X, Lynn MJ, Newman NJ, Biousse V.
Congruency in homonymous hemianopia. Am
Associated focal neurological signs and
J Ophthalmol. 2007;143(5):772–80.
symptoms, as well as visual field characteris- 14. Zhang X, Kedar S, Lynn MJ, Newman NJ, Biousse V.
tics such as their location and congruity, may Homonymous hemianopias: clinical-anatomic corre-
help to more precisely determine the site of lations in 904 cases. Neurology. 2006;66(6):906.
15. Misra M, Rath S, Mohanty AB. Anton syndrome and
the causative brain lesion. Understanding
cortical blindness due to bilateral occipital infarction.
neurological syndromes will enable ophthal- Indian J Ophthalmol. 1989;37(4):196.
mologists and neurologists to identify 16. Joseph R. Confabulation and delusional denial: fron-
patients with central visual disorders and tal lobe and lateralized influences. J Clin Psychol.
1986;42(3):507–20.
perform appropriate testing. Only at that
17. Lessel S. Higher diorders of visual function: negative
point can a differential diagnosis, diagnostic phenomena. In: Glaser J, Smith JM, editors. Neuro-
evaluation, and treatment plan be generated. opthalmology, vol. 8. St. Louis: Mosby; 1975. p. 3–4.
18. Brázdil M, Kuba R, Daniel P, Sochůrková D, Dobsík M,
Rektor I. Covert vision sign. Eur J Neurol. 2002;9(3):
316–9.
References 19.
Weiskrantz L. Blindsight revisited. Curr Opin
Neurobiol. 1996;6(2):215–20.
1. Goodale MA, Meenan JP, Bülthoff HH, Nicolle DA, 20. Stoerig P. Blindsight, conscious vision, and the role of
Murphy KJ, Racicot CI. Separate neural pathways for primary visual cortex. Prog Brain Res. 2006;155:
the visual analysis of object shape in perception and 217–34.
prehension. Curr Biol. 1994;4(7):604–10. 21. Fendrich R, Wessinger CM, Gazzaniga MS. Residual
2. Girkin CA, Miller NR. Central disorders of vision in vision in a scotoma: implications for blindsight.
humans. Surv Ophthalmol. 2001;45(5):379–405. Science. 1992;258(5087):1489–91.
3. Fraser JA, Newman NJ, Biousse V. Disorders of the 22. Cowey A, Stoerig P. The neurobiology of blindsight.
optic tract, radiation, and occipital lobe. Handb Clin Trends Neurosci. 1991;14(4):140–5.
Neurol. 2011;102:205–21. 23. Zeki S. A century of cerebral achromatopsia. Brain.
4. Spector RH. Visual fields. In: Walker HK, Hall WD, 1990;113(Pt 6):1721–77. (Review).
Hurst JW, editors. Clinical methods: the history, 24. Paulson HL, Galetta SL, Grossman M, Alavi A.
physical, and laboratory examinations. 3rd ed.
Hemiachromatopsia of unilateral occipitotemporal
Boston: Butterworth-Heinemann; 1990. infarcts. Am J Ophthalmol. 1994;118(4):518–23.
5. Kerkhoff G, Bucher L. Line bisection as an early 25. Miller NR, Newman NJ. Central disorders of
method to assess homonymous hemianopia. Cortex. visual function. In: Miller NR, Newman NJ, edi-
2008;44(2):200–5. tors. The essentials: Walsh & Hoytʼs clinical
160 M. Pail et al.
neuro-ophthalmology. Baltimore: Williams & Wilkins; 33. Trobe JD. The neurology of vision. New York: Oxford
1999. p. 369–408. University Press; 2001.
26. Cowey A, Heywood CA. There’s more to colour than 34. Andersen RA, Andersen KN, Hwang EJ, Hauschild
meets the eye. Behav Brain Res. 1995;71(1–2):89– M. Optic ataxia: from Bálint’s syndrome to the pari-
100. Review. etal reach region. Neuron. 2014;81(5):967–83.
27. Meadows JC. Disturbed perception of colours associ- 35. Kinsbourne M. A model for the mechanism of unilat-
ated with localized cerebral lesions. Brain. 1974;97(4): eral neglect of space. Trans Am Neurol Assoc. 1970;
615–32. 95:143–6.
28. Zihl J, von Cramon D, Mai N, Schmid C. Disturbance 36. Heilman KM, Valenstein E, Watson RT. Neglect
of movement vision after bilateral posterior brain and related disorders. Semin Neurol. 2000;20(4):
damage. Further evidence and follow up observations. 463–70.
Brain. 1919;114(Pt 5):2235–52. 37. Corbetta M, Shulman GL. Spatial neglect and atten-
29. Haxby JV, Grady CL, Horwitz B, Ungerleider LG, tion networks. Annu Rev Neurosci. 2011;34:569–99.
Mishkin M, Carson RE, et al. Dissociation of object 38. Verdon V, Schwartz S, Lovblad KO, Hauert CA,
and spatial visual processing pathways in human Vuilleumier P. Neuroanatomy of hemispatial neglect
extrastriate cortex. Proc Natl Acad Sci U S A. and its functional components: a study using voxel-
1991;88(5):1621–5. based lesion-symptom mapping. Brain. 2010;133(Pt 3):
30.
van Essen DC, Anderson CH, Felleman DJ. 880–94.
Information processing in the primate visual system: 39. Brown AC. Professor A.C. Brown physiology & neu-
an integrated systems perspective. Science. 1992; roscience web sites. http://www.acbrown.com/neuro-
255(5043):419–23. science. 2011. Accessed 6 Mar 2016.
31. Bálint R. [Paralysis of visual perception]. Seelenlähmung 40. Devinsky O, Farah MJ, Barr WB. Chapter 21 Visual
des “Schauens,” optische Ataxie, räumliche Störung der agnosia. Handb Clin Neurol. 2008;88:417–27.
Aufmerksamkeit. Monatszeitschrift Psychiatr Neurol. 41.
Damasio AR, Damasio H, Van Hoesen GW.
1909;25:51–81. [Article in German]. Prosopagnosia: anatomic basis and behavioral mecha-
32.
Vallar G. Spatial neglect, Bálint-Homes’ and nisms. Neurology. 1982;32(4):331–41.
Gerstmann’s syndrome, and other spatial disorders. 42. Damasio AR, Damasio H. The anatomic basis of pure
CNS Spectr. 2007;12(7):527–36. alexia. Neurology. 1983;33(12):1573–83.
Adaptation and Rehabilitation
in Patients with Homonymous 11
Visual Field Defects
Susanne Trauzettel-Klosinski
Abstract
Hemianopia leads to severe impairment of spatial orientation and mobil-
ity. In cases without macular sparing an additional reading disorder occurs.
Persistent visual deficits require rehabilitation. The goal is to compensate
for the deficits to regain independence and to maintain the patient’s quality
of life. Spontaneous adaptive mechanisms, such as shifting the field defect
towards the hemianopic side by eye movements or eccentric fixation,
are beneficial, but often insufficient. They can be enhanced by training,
e.g., saccadic training to utilize the full field of gaze in order to improve
mobility and by special training methods to improve reading performance.
At present only compensatory interventions are evidence-based.
Keywords
Hemianopia • Orientation disorder • Reading disorder • Rehabilitation •
Compensatory methods • Training • Quality of life
Fig. 11.1 World Health Organization (WHO) classification applied to the visual system (International Classification of
Functioning, Disability and Health; WHO 2004) (Modified from Trauzettel-Klosinski [5], with permission)
11 Adaptation and Rehabilitation in Patients with Homonymous Visual Field Defects 163
probability during the first 6 months after linguistic (grammatical, contextual) mechanisms.
onset [6]. Additionally, a placebo effect Recordings of reading eye movements in healthy
should be excluded by using a control group. subjects show a typical staircase pattern, a
2. Quality of testing methods: sequence of saccades and holding positions. The
(a) Reliability (e.g., repeatability, precision) significance of the central visual field is indicated
(b) Objectivity by the high cortical magnification: the central
(c) Validity (does the test show causal 10°, which account for approximately 2% of the
relations) total visual field, cover more than 50% of the
3. Aim of the intervention: primary visual cortex [13].
(a) Clinical relevance of the effect
(b) Persistence of the effect after training
11.3.2 The Clinical Picture
11.3 T
he Hemianopic Reading Reading performance in HH depends strongly on
Disorder the configuration of the field defect and its dis-
tance to the visual field centre, i.e., the size of the
Reading is a key function in developed societies. reading visual field (Fig. 11.2) [14]. In macular
It means independence, participation, mental splitting, half of the reading visual field is
agility, and quality of life. obscured (see Fig. 11.2a). In macular sparing, the
reading visual field is preserved and reading can
be normal dependent on the amount of sparing
11.3.1 Normal Reading (see Fig. 11.2b). On the other hand, a small para-
central homonymous scotoma causes severe
The visual preconditions for reading are [5, 7]: reading problems because it covers half of the
reading visual field (see Fig. 11.2c). Such small
(a) Sufficient resolution of the retinal area used paracentral scotomas can easily be overlooked in
for reading (for reading common newspaper automated perimetry if the grid of test points is
print at a distance of 25 cm a visual acuity of not dense enough. Therefore, an especially dense
at least 0.4 or 20/50 is required) grid in automated perimetry should be chosen, or
(b) Sufficient size of the retinal area during one manual perimetry should be performed, which
fixation, which requires a minimum of 2° allows the examiner to search for small scotomas
right and left of fixation [8, 9] in a goal-directed manner.
The degree of the hemianopic reading disorder
In skilled readers, the total “perceptual span” is also influenced by the side of the field defect. If
or “reading visual field” during one fixation can the hemianopic field defect is in the reading direc-
be extended in the reading direction. For fluent tion, reading is extremely impaired. Figure 11.3
reading, a total perceptual span of 5° (or 15 let- shows the eye movement recordings during read-
ters) right and 1.3–2° (or 4–6 letters) left of fixa- ing of one line of text: In Fig. 11.3a for a healthy
tion is necessary, which was shown in scrolling subject; in Fig. 11.3b for a patient with left
window experiments in normal subjects [10], and HH. The patient gets through the line quite well,
in patients with HH [11]. The perceptual span but needs additional steps during the return sweep,
offers a preview benefit by providing information indicating the difficulties in finding the beginning
about word length, word shape, etc., which is of the next line. Figure 11.3c displays the record-
useful to guide the next saccade to the appropri- ing of a patient with right HH who needs many
ate landing position [12]. The perceptual span is more forward saccades per line and makes several
a dynamic parameter that is also influenced by backward saccades to get through the line.
164 S. Trauzettel-Klosinski
a b
c d
Fig. 11.2 The impact of a homonymous field defect on c ausing spatial orientation problems. (c) A small paracen-
reading performance: Right homonymous hemianopia tral homonymous scotoma causes severe reading prob-
related to the text: (a) In macular splitting, half of the lems. (d) Eccentric fixation shifts the field defect towards
reading visual field is functionless and reading ability is the blind side, which creates a small perceptual area along
severely impaired. (b) In macular sparing, reading ability the midline (Modified from Trauzettel-Klosinski [14],
is preserved, even though there is a large field defect with permission)
a 0 c
0
2
2
4
4
6
–2° 0° 2° 4° 6° 8° 10° 12° 14° 16° 16° 20° 6
b 0 8
2 10
4 12
6 14
–2° 0° 2° 4° 6° 8° 10° 12° 14° 16° 18° 20° –2° 0° 2° 4° 6° 8° 10° 12° 14° 16° 18° 20°
Fig. 11.3 Eye movements while reading one line of text. finding the beginning of the next line, indicated by several
(a) The normally-sighted subject makes eight saccades and additional steps during the return sweep. (c) A patient with
needs approximately 1.5 s to get through the line. An accu- right HH shows an increased number of saccades and several
rate return sweep follows to the beginning of the next line. regressions per line, has a markedly prolonged reading time,
(b) A patient with left homonymous hemianopia (HH) has no but has no problems with the return sweep (Modified from
major problems getting through the line, but has d ifficulties Trauzettel-Klosinski [5], with permission)
11 Adaptation and Rehabilitation in Patients with Homonymous Visual Field Defects 165
a b
SLO-Fundus Field defect right
*Mean: 2.6°
400
0s
300
5s
frequency
200
10s
100
15s
20s
–2° 0° 2°
0
–15 –10 –5 0 5 10 15
Horizontal fixational eye movements
Fig. 11.4 (a) (left) SLO-image of the fundus with a (20 s) in 25 patients with right homonymous hemiano-
fixation cross. The image shows the absolute position of pia and with sparing of <4°. A positive mean value cor-
the fovea related to the stimulus, which allows direct responds to a shift of the distribution to the right: here
fixation control without calibration. (right) Asymmetric centered at +2.6° (mean). The distributions are signifi-
fixational eye movements towards the hemianopic side cantly different from a normal distribution centered at
(individual example). (b) Distribution of fixational eye 0° (P < 0.0001) (Modified from Reinhard et al. [16],
movements during continuous fixation of a single cross with permission)
166 S. Trauzettel-Klosinski
a b
Fig. 11.5 When looking straight ahead (a), information from the blind side is missing. The obstacle, here, the baby
carriage, becomes visible by making scanning eye movements (b)
11 Adaptation and Rehabilitation in Patients with Homonymous Visual Field Defects 167
gaze can be utilized to gather information from predictive saccades after training to find a unique
the blind side and to avoid collisions, here with target. Such short-term adaptation was reported by
the baby stroller (Fig. 11.5b). This mechanism Meienberg et al. [26]. On the other hand, Reinhard
normally is not sufficient for complete compensa- et al. did not find a correlation between the number
tion, but can be enhanced by saccade training. of dysmetric saccades and the duration of the dis-
ease, which indicates that long-term adaptation
Saccadic Tasks Dysmetric saccades often occur with more effective strategies did not, or only
while performing tasks that require saccades, so insufficiently, occur [16]. As a consequence, for
that the accuracy of “landing places” as well as rehabilitation simple saccade training in addition
fixation stability after landing is decreased [16]. to using search tasks can be considered.
Using mainly hypometric saccades is an ineffec-
tive, time-consuming strategy. Some patients learn Head Turn Turning the head in yaw is a com-
to switch from hypometric saccades at onset to mon habit in patients with HH. However, Fig. 11.6
a b c
d e f
Fig. 11.6 Visual field seen in normal conditions (a) and without eye movements no information from the blind
in patients with right homonymous hemianopia. Upper side, (e) scanning eye movements utilize the field of gaze
row Head turn: (b) looking straight, no information from and enlarge the “functional visual field”, (f) scanning eye
the blind side; (c) head turn alone does not change the movements plus head turn enhance this effect (Modified
visual field. Lower row Eye movements and head turn: (d) from Paysse and Coats [27], with permission)
168 S. Trauzettel-Klosinski
shows that head turn alone does not change the into blind areas of the visual field [32], i.e., into
visual field. On the contrary, the disadvantage of the blind side in HH. Therefore, a consciously
continuous head turn is torticollis without any controlled saccade can be performed in order to
functional improvement. If head turn is combined bring the stimulus onto the seeing hemifield.
with scanning eye movements, the “functional” After that, transient attentional mechanism can
visual field is extended by using the full field take over again.
of gaze [27].
a b c
17°
17°
50°
90°
P P
FL FR
Fig. 11.7 Exotropia of the eye on the hemianopic side dus (bottom; FL fovea left eye, FR fovea RE) and to the
(right homonymous hemianopia, HH) with anomalous monocular visual fields (top). (c) Exotropia of RE of 17°
retinal correspondence leads to extension of the binocular extends the binocular visual field towards the hemianopic
visual field. (a) Binocular visual field in right HH with side (The marked section represents the area of double
normal binocular vision. (b) Exotropia of the right eye vision in the case of normal correspondence) (Modified
(RE) with anomalous correspondence related to the fun- from van Waveren et al. [30], with permission)
11 Adaptation and Rehabilitation in Patients with Homonymous Visual Field Defects 169
of life scores in the social domain. The newly During the rehabilitative consultation, it is
learnt saccadic strategy could be applied to generally important to explain the special nature
everyday life. The training effect remained sta- of the visual impairment to patients and their
ble after the end of the training. Even patients relatives in detail. The goal is to make them aware
with long-standing HH for many years of their impairment and to make them understand
improved, which indicates that the spontaneous that driving a car is illegal in Europe. There are
adaptation had not been sufficient. In a recent as some states in the United States, where, depen-
yet unpublished study with age-matched nor- dent on local legal regulations, a restricted driv-
mally-sighted subjects, we found that the aver- ing license can be given limited to a familiar
age improvement of the reaction times of the environment. In the future, it might be an option
HH patients even reaches normal levels [unpub- that HH patients may regain a restricted driving
lished data]. license after compensative saccadic training,
The control group that received visual field when their reaction times during scanning tasks
stimulation training (a flickering letter at 22° normalize and they pass a special driving test.
eccentricity) showed neither a change in explora-
tion behavior nor in the extent of the visual field. Substitutive Interventions: Optical Devices
The EST software (www.visiocoach.de) was Binocular sector prisms have been shown to be
designed to be easy to handle and can be used ineffective in HH, but helpful in neglect [43].
independently by the patient at home, even with- Most patients find monocular prisms and mirrors
out previous personal computer experience. too unpleasant because of the resulting confusion
Other software is also available with different and diplopia in the centre (see Fig. 11.6). A newer
saccadic or search strategies [19, 34, 35]. approach applied monocular sector prisms placed
Meanwhile, more RCTs have been conducted, across the whole width of the lens, but only in the
showing audio-visual stimulation to be more peripheral part of the glasses. This was reported
effective than visual training alone [36], and ocu- to be beneficial by expanding the visual field
lomotor and attentional training to have the same without central diplopia, based on the finding that
benefit [37]. A recent study compared a com- after 1 year 47% of these patients were still wear-
bined reading and exploration training with a ing the prisms [44]. However, these studies were
control training (attentional tasks) and found performed without control group. A recent ran-
enlargement of the field of gaze, and improve- domized crossover trial found higher acceptance
ment of activities of daily living and reading and greater improvement of mobility in the group
speed [25]. In another RCT, a horizontal saccadic with real prisms, based on subjective reports
training improved mobility [38]. [45]. In order to come to a conclusive judgment
Other approaches do not yet allow final con- about the benefits, future studies should employ
clusions: Studies with a very small sample and this method using objective outcome variables.
without patient control group reported that a
ramp-step paradigm improved visual search [39], Restitutive Training The aim of restitutive
which was then applied in a larger patient group training is to reactivate incompletely damaged
as a Web-based training [40]. Regarding multi- neurons in the blind hemifield and to enlarge the
sensory stimulation, a review of 21 studies cited visual fields by visual stimulation. Earlier studies
beneficial effects in 20 of them; however, the performed visual stimulation along the vertical
quality of these studies was judged as insufficient border of the field defect, and reported visual
for a valid conclusion that this is an effective field enlargement [46, 47]. However, these effects
intervention [41]. could not be confirmed in studies using conven-
Antisaccade training by stimulation of con- tional perimetry [48, 49], nor by fundus perime-
sciously controlled attention in combination with try with simultaneous fixation control [50].
a saccadic adaptation procedure yielded positive Fixational eye movements shift the field defect
effects on different tasks [42]. towards the blind side [16, 51, 52] and can be
170 S. Trauzettel-Klosinski
misinterpreted as an enlargement of the visual On the other hand, compensatory plasticity after
field [50, 53–55]. V1-damage is indicated by changes in gaze strat-
Studies that applied stimulation of the visual egy: “a compensatory, higher-level, integrative plas-
field in a more peripheral area, thus reducing the ticity, which supports interaction between the
risk of eye movements towards the stimulus, did person and the environment” [63]. This view is sup-
not yield enlargement of the visual fields [3, 56], ported by a functional magnetic resonance imaging
but rather increased contrast sensitivity in two study that yielded bilateral activation of extrastriate
patients [56], which might be an effect of an cortex after eye movement training [66].
extrastriate activation (see below).
In summary, at present, there are no evidence-
based studies in the literature that could show the 11.5 Associated Symptoms
effect of restitutive training on improvement of
visual fields [57]. However, regeneration of neu- Patients with brain damage often show rather
rons in V1 has to be distinguished from the blind- unspecific ophthalmological symptoms, which
sight phenomenon, which is based on extrastriate can be very impairing and that interfere with reha-
activation (see below). bilitation efforts. These symptoms can be related
to crowding, glare, reduced contrast sensitivity,
Plasticity of the Adult Visual Cortex Perceptual oculomotor disorders, and asthenopia due to
learning in the normal visual cortex has been accommodation deficits and diplopia. Diplopia
shown in many studies (see the review by Sasaki (14.6% in one study [5]) and hypo-accommodation
et al. [58]). It is characterized by an improvement can particularly be very disturbing and require
in different functions such as motion direction, early treatment with prisms and appropriate near
orientation, Vernier acuity, and texture discrimi- addition lenses, respectively. Furthermore, HH is
nation. The underlying mechanisms have been frequently associated with other neurological def-
described as an increase in response strength, icits such as hemiplegia and cognitive and atten-
tuning of individual neurons, changes in contex- tional deficits. Hemineglect can be present in
tual modulation (rather than large-scale spatial combination with HH. The line dissection test, for
reorganization [59]), or long-term potentiation of example, can help to assess the additional effect
synaptic responses [60]. of neglect [67, 68], but not in acute cases [69]. In
In cases of bilateral retinal lesions, reorgani- isolated HH the line is slightly shifted to the blind
zation in the occipital cortex (V1) has been found side, but in hemineglect, to the seeing side. The
to be very limited, and the interpretation of ear- rehabilitation of these patients requires an inter-
lier data regarding cortical remapping has been disciplinary approach.
seriously questioned [61, 62].
In cases of damage to the visual cortex, percep-
tual relearning for specific motion direction stim- 11.6 Diagnostics
uli has been reported [63–65]. This finding is
based on visual training of extrastriate cortex [63]. The following test battery is important for spe-
The “blindsight” phenomenon is mediated by cific rehabilitative measures:
mostly unconscious perception of visual stimuli
via the superior colliculus to extrastriate regions • Assessment of visual acuity for far and near
bypassing V1 [65]. These “phylogenetically old” distance, refraction, accommodation, and
pathways can be reactivated by intensive train- adaptation of optimal corrections are essen-
ing, which can lead to mainly unconscious per- tial. Appropriate glasses are a precondition for
ception in some patients [64]. Whether this kind an effective visual rehabilitation.
of residual vision can be improved by the blind- • Neuro-ophthalmological/orthoptic examination
sight training to a level of everyday-life relevance assesses binocular vision, eye position, motility,
is still an open question. saccades, pursuit, optokinetic nystagmus.
11 Adaptation and Rehabilitation in Patients with Homonymous Visual Field Defects 171
• Visual field examination is crucial. If standard scrolled text for right HH [22], search task in a
perimetry (see Chap. 4) cannot be performed, line [24, 25], and single word reading [23].
confrontational perimetry or tangent screen For orientation: Visual search tasks [3], audio-
campimetry (focusing on the central part of visual search tasks [36], saccadic tasks [25, 38],
the field) are semiquantitative methods to be and attentional training [37].
applied. When looking for small paracentral Remark: The author has no personal financial
defects, a specially dense grid of test points or interest in any products mentioned.
a thorough manual strategy should be used.
• Contrast sensitivity can be important for read- Acknowledgment The author thanks Manfred MacKeben
ing and for orientation. These activities can be PhD, The Smith Kettlewell Eye Research Institute,
San Francisco, CA, USA, for critical comments and edito-
improved by augmented illumination and/or rial help.
by marking the patient’s environment with
special high contrast landmarks.
• Reading speed is an important measure. A suit- References
able tool are the International Reading Speed
Texts (IReST), which are standardized para- 1. Papageorgiou E, Hardiess G, Schaeffel F, Wiethoelter
graphs to be read aloud. A set of ten equivalent H, Karnath HO, Mallot H, et al. Assessment of
texts is linguistically adapted for contents, vision-related quality of life in patients with hom-
onymous visual field defects. Graefes Arch Clin Exp
length, difficulty, and linguistic complexity in Ophthalmol. 2007;245(12):1749–58.
order to be used for repeated measurements. 2. Truelsen T, Piechowski-Jóźwiak B, Bonita R, Mathers
They were developed in 17 languages and are C, Bogousslavsky J, Boysen G. Stroke incidence and
therefore comparable, not only within one lan- prevalence in Europe: a review of available data. Eur
J Neurol. 2006;13(6):581–98.
guage, but also between different languages 3. Roth T, Sokolov AN, Messias A, Roth P, Weller M,
(www.amd-read.net.) [70, 71]. Trauzettel-Klosinski S. Comparing explorative sac-
• Recording eye movements during reading is a cade and flicker training in hemianopia: a randomized
valuable method for detailed analysis of read- controlled study. Neurology. 2009;72(4):324–31.
4. World Health Organisation (WHO). International
ing performance that allows quantitative classification of functioning, disability and health
assessment of reading parameters, such as (ICF). Geneva: World Health Organization; 2004.
number and amplitude of forward and back- 5. Trauzettel-Klosinski S. Rehabilitation for visual dis-
ward saccades, fixation durations, and return orders. J Neuroophthalmol. 2010;30(1):73–84.
6. Zhang X, Kedar S, Lynn MJ, Newman NJ, Biousse
sweep. V. Natural history of homonymous hemianopia.
Neurology. 2006;66(6):901–5.
7. Whittaker SG, Lovie-Kitchin J. Visual requirements
11.7 Summary and Conclusions for reading. Optom Vis Sci. 1993;70(a):54–65.
8. Aulhorn E. [Fixation width and fixation fre-
quency of the contours presented in reading]. Über
The main effects of HH are impairment of read- Fixationsbreite und Fixationsfrequenz beim Lesen
ing and spatial orientation. Reading performance gerichteter Strukturen. Pflügers Arch Physiol.
depends on the side of the field defect (unfavor- 1953;257(4):318–28. (Article in German).
9. Legge GE, Ahn SJ, Klitz TS, Luebker A. Psychophysics
able if in the reading direction), the size of a of reading. XVI. The visual span in normal and low
macular sparing, and spontaneous adaptation. vision. Vision Res. 1997;37(14):1999–2010.
Spontaneous adaptive mechanisms are mostly 10. McConkie GW, Rayner K. The span of the effec-
beneficial, but often insufficient. However, they tive stimulus during a fixation in reading. Percept
Psychophys. 1975;17(6):578–86.
can be supported by compensatory training. They 11. Trauzettel-Klosinski S, Brendler K. Eye movements
aim to enlarge the “functional visual field” by in reading with hemianopic field defects: the signifi-
utilizing the field of gaze. cance of clinical parameters. Graefe’s Arch Clin Exp
Ophthalmol. 1998;236(2):91–102.
12. Hohenstein S, Kliegl R. Semantic preview benefit
For rehabilitation, at present only compensa- during reading. J Exp Psychol Learn Mem Cogn.
tory methods are evidence-based For reading: 2014;40(1):166–90.
172 S. Trauzettel-Klosinski
13. Horton JC, Hoyt WF. The representation of the visual in congenita homonymous hemianopia. J Pediatr
field in human striate cortex: a revision of the classic Ophthalmol Strabismus. 1995;32(4):236–8.
Holmes map. Arch Ophthalmol. 1991;109(6):816–24. 29.
Donahue SP, Haun AK. Exotropia and face
14.
Trauzettel-Klosinski S. Reading disorders. In: turn in children with homonymous hemianopia.
Schiefer U, Wilhelm H, Hart W, editors. Clinical J Neuroophthalmol. 2007;27(4):304–7.
neuro-ophthalmology. Heidelberg: Springer; 2007. 30. Van Waveren M, Jägle H, Besch D. Management
p. 303–8. of strabismus with hemianopic visual field
15. Trauzettel-Klosinski S. Eccentric fixation in hemi- defects. Graefe’s Arch Clin Exp Ophthalmol.
anopic field defects - a valuable strategy to improve 2013;251(2):575–84.
reading ability and an indication for cortical plasticity. 31. Herzau V, Bleher I, Joos-Kratsch E. Infantile exotro-
Neuro Ophthalmol. 1997;18(3):117–31. pia with homonymous hemianopia: a rare contraindi-
16.
Reinhard J, Damm I, Ivanov IV, Trauzettel- cation for strabismus surgery. Graefes Arch Clin Exp
Klosinski S. Eye movements during saccadic Ophthalmol. 1988;226(2):148–9.
and fixation tasks in patients with hemianopia. 32. Nakayama K, Mackeben M. Sustained and transient
J Neuroophthalmol. 2014;34(4):354–61. components of focal visual attention. Vision Res.
17. Schmidt D, Ullrich D, Roßner R. Horizontal and
1989;29(11):1631–47.
vertical reading: a comparative investigation of eye 33. Hoffman JE, Subramaniam B. The role of visual atten-
movements. Ger J Ophthalmol. 1993;2(4–5):251–5. tion in saccadic eye movements. Percept Psychophys.
18. Subramanian A, Legge GE, Wagoner GH, Yu D.
1995;57(6):787–95.
Learning to read vertical text in peripheral vision. 34.
Pambakian ALM, Mannan SK, Hodgson TL,
Optom Vis Sci. 2014;91(9):1097–105. Kennard C. Saccadic visual search training: a treat-
19. Kerkhoff G, Münßinger U, Haaf E, Eberle-Strauss G, ment for patients with homonymous hemianopia.
Stögerer E. Rehabilitation of homonymous scotoma J Neurol Neurosurg Psychiatry. 2004;75(10):1443–8.
in patients with postgeniculate damage of the visual 35.
Mannan SK, Pambakian ALM, Kennard C.
system: saccadic compensation training. Restor Compensatory strategies following visual search train-
Neurol Neurosci. 1992;4(4):245–54. ing in patients with homonymous hemianopia: an eye
20. Zihl J, Krischer C, Meissen Z. [Hemianopic dyslexia movement study. J Neurol. 2010;257(11):1812–21.
and its treatment.] Die hemianopische Lesestörung 36. Keller I, Lefin-Rank G. Improvement of visual
und ihre Behandlung. Nervenarzt. 1984;55(6):317– search after audiovisual exploration training in
23. (Article in German). hemianopic patients. Neurorehabil Neural Repair.
21. Zihl J. Visual scanning behaviour in patients with 2010;24(7):666–73.
homonymous hemianopia. Neuropsychologia. 37. Lane AR, Smith DT, Ellison A, Schenk T. Visual
1995;33(3):287–303. exploration training is no better than attention train-
22. Spitzyna GA, Wise RJS, McDonald SA, Plant GT, ing for treating hemianopia. Brain. 2010;133(Pt
Kidd D, Crewes H, et al. Optokinetic therapy improves 6):1717–28.
text reading in patients with hemianopic alexia: a con- 38. de Haan GA, Melis-Dankers BJM, Brouwer WH,
trolled trial. Neurology. 2007;68(22):1922–30. Tucha O, Heutink J. The effects of compensatory
23. Schuett S, Heywood CA, Kentridge RW, Dauner R, scanning training on mobility in patients with hom-
Zihl J. Rehabilitation of reading and visual explora- onymous visual field defects: a randomized controlled
tion in visual field disorders: transfer or specificity? trial. PLoS ONE. 2015;10(8):e0134459.
Brain. 2012;135(3):912–21. 39. Jacquin-Courtois S, Bays PM, Salemm R, Leff AP,
24. Schuett S, Heywood CA, Kentridge RW, Zihl
Husain M. Rapid compensation of visual search
J. Rehabilitation of hemianopic dyslexia: are words strategy in patients with chronic visual field defects.
necessary for re-learning oculomotor control? Brain. Cortex. 2013;49(4):994–1000.
2008;131(Pt 12):3156–68. 40. Ong YH, Jacquin-Courtois S, Gorgoraptis N, Bays
25. Aimola L, Lane AR, Smith DT, Kerkhoff G, Ford PM, Husain M, Leff AP. Eye-search: a web-based
GA, Schenk T. Efficacy and feasibility of home- therapy that improves visual search in hemianopia.
based training for individuals with homonymous Ann Clin Transl Neurol. 2015;2(1):74–8.
visual field defects. Neurorehabil Neural Repair. 41. Tinga AM, Visser-Meily JM, van der Smagt MJ, van
2014;28(3):207–18. der Stigchel S, van Ee R, Nijboer TCW. Multisensory
26. Meienberg O, Zangemeister WH, Rosenberg M, Hoyt stimulation to improve low- and higher-level sen-
WF, Stark L. Saccadic eye movement strategies in sory deficits after stroke: a systematic review.
patients with homonymous hemianopia. Ann Neurol. Neuropsychol Rev. 2016;26(1):73–91.
1981;9(6):537–44. 42. Lévy-Bencheton D, Pélisson D, Prost M, Jacquin-
27. Paysse EA, Coats DK. Anomalous head posture with Courtois S, Salemme R, Pisella L, et al. The effects
earlyonset homonymous hemianopia. J AAPOS. of short-lasting anti-saccade training in homonymous
1997;1(4):209–13. hemianopia with and without saccadic adaptation.
28. Levy Y, Turetz J, Krakowski D, Hartmann B, Nemet P. Front Behav Neurosci. 2016;9:332.
Development of compensating exotropia with anom- 43. Rosetti Y, Rode G, Pisella L, Farné A, Li L, Boisson D,
alous retinal correspondence after early infancy Perenin MT. Prism adaptation to a rightward o ptical
11 Adaptation and Rehabilitation in Patients with Homonymous Visual Field Defects 173
deviation rehabilitates left hemispatial neglect. Nature. 58. Sasaki Y, Nanez JE, Watanabe T. Advances in visual
1998;395(6698):166–9. perceptual learning and plasticity. Nat Rev Neurosci.
44. Bowers AR, Keeney K, Peli E. Community-based trial 2010;11(1):53–60.
of a peripheral prism visual field expansion device for 59. Karmakar U, Dan Y. Experience-dependent plasticity
hemianopia. Arch Ophthalmol. 2008;126(5):657–64. in adult visual cortex. Neuron. 2006;52(4):577–85..
45. Bowers AR, Keeny K, Peli E. Randomized cross- (Review).
over clinical trial of real and sham peripheral 60. Sale A, de Pasquale R, Bonaccorsi J, Pietra G,
prism glasses for hemianopia. JAMA Ophthalmol. Olivieri D, Berardi N, et al. Visual perceptual learning
2014;132(2):214–22. includes long-term potentiation in the visual cortex.
46. Zihl J, von Cramon D. Restitution of visual function in Neuroscience. 2011;172:219–25.
patients with cerebral blindness. J Neurol Neurosurg 61. Haak KV, Morland AB, Engel SA. Plasticity, and
Psychiatry. 1979;42(4):312–22. its limits, in adult human primary visual cortex.
47. Kasten E, Wüst S, Behrens-Baumann W. Computer- Multisens Res. 2015;28(3–4):297–307.
based training for the treatment of partial blindness. 62. Wandell BA, Smirnakis SM. Plasticity and stability
Nat Med. 1998;4(9):1083–7. of visual field maps in adult primary cortex. Nat Rev
48. Balliet R, Blood KM, Bach-Y-Rita P. Visual field reha- Neurosci. 2009;10(12):873–84.
bilitation in the cortically blind? J Neurol Neurosurg 63. Huxlin K, Martin T, Kelly K, Riley M, Friedman DI,
Psychiatry. 1985;48(11):113–24. Burgin WS, et al. Perceptual relearning of complex
49. Schreiber A, Vonthein R, Reinhard J, Trauettel-
visual motion after V1 damage in humans. J Neurosci.
Klosinski S, Connert C, Schiefer U. Effect of visual 2009;29(13):3981–91.
restitution training on absolute homonymous scoto- 64. Das A, Tadin D, Huxlin KR. Beyond blindsight: prop-
mas. Neurology. 2006;67(1):143–5. erties of visual relearning in cortically blind fields.
50. Reinhard J, Schreiber A, Schiefer U, Kasten E,
J Neurosci. 2014;34(35):11652–64.
Sabel BA, Kenkel S, et al. Does visual restitution 65. Weiskrantz L. Roots of blindsight. Prog Brain Res.
training change absolute homonymous scotoma? Br 2004;144:229–41.
J Ophthalmol. 2005;89(1):30–5. 66. Nelles G, Pscherer A, de Greiff A, Gerhard H,
51. Bischoff P, Lang J, Huber A. Macular sparing as a peri- Forsting M, Esser J, et al. Eye-movement training-
metric artifact. Am J Ophthalmol. 1995;119(1):72–80. induced changes of visual field representation in
52. Trauzettel-Klosinski S, Reinhard J. The vertical field patients with post-stroke hemianopia. J Neurol.
border in human hemianopia and its significance for 2010;257(11):1832–40.
fixation behavior and reading. Invest Ophthalmol Vis 67. Schenkenberg T, Bradford DC, Ajax ET. Line bisec-
Sci. 1998;39(11):2177–86. tion and unilateral visual neglect in patients with neu-
53.
Horton JC. Disappointing results from Nova rologic impairment. Neurology. 1980;30(5):509–17.
Vision’s visual restoration therapy. Br J Ophthalmol. 68. Lanyon LJ, Barton JJ. Visual search and line bisec-
2005;89(1):1–2. tion in hemianopia: computational modelling of corti-
54. Horton JC. Vision restoration therapy: confounded by cal compensatory mechanisms and comparison with
eye movements. Br J Ophthalmol. 2005;89(7):792–4. hemineglect. PLoS One. 2013;8(2):e54919.
55. McFadzean R. NovaVision: vision restoration ther- 69.
Sperber C, Karnath HO. Diagnostic valid-
apy. Curr Opin Ophthalmol. 2006;17(6):498–503.. ity of line bisection in the acute phase of stroke.
(Review). Neuropsychologia. 2016;82:200–4.
56.
Raninen A, Vanni S, Hyvärinen L, Näsänen 70. Hahn GA, Penka D, Gehrlich C, Messias A, Weismann
R. Temporal sensitivity in a hemianopic visual M, Hyvärinen L, et al. New standardised texts for
field can be improved by long-term training using assessing reading performance in four European lan-
flicker stimulation. J Neurol Neurosurg Psychiatry. guages. Br J Ophthalmol. 2006;90(4):480–4.
2007;78(1):66–73. 71.
Trauzettel-Klosinski S, Dietz K, IReST Study
57. Pollock A, Hazelton C, Henderson CA, Angilley
Group. Standardized assessment of reading per-
J, Dhillon B, Langhorne P et al. Interventions for formance: the New International Standardized
visual field defects in patients with stroke. Cochrane Reading Texts IReST. Invest Ophthalmol Vis Sci.
Database Syst Rev. 2011;(10):CD008388. 2012;53(9):5452–61.
Index
A Blindsight, 153
Achromatopsia, 25, 27, 153 optokinetic and pursuit responses, 125–126
Acute cerebral artery occlusion, endovascular saccades, 124–125
treatment, 33–35 Blind spots, 18–19
Acute ischemic stroke. See also Ischemic stroke Blobs, 13
brain computed tomography, 32 Blood supply
intravenous thrombolysis, 33 of lateral geniculate body, 10
magnetic resonance imaging of optic chiasm, 9
with angiography, 32 of optic nerve, 8
in posterior cerebral artery, 35, 37 of optic radiation, 10–11
Agnosia of retina, 7
apperceptive, 157 of visual cortex, 14
associative agnosia, 157 Boston diagnostic aphasia examination, 149
visual, 154, 157 Bow-tie atrophy, 56, 59, 60, 81, 88
visual-spatial, 158
Agraphia, 146, 152, 158
Akinetopsia, 153 C
Alzheimer disease, 28 Campimetry. See Pupil perimetry
Amacrine cells, 6 Carbon monoxide (CO) poisoning, 23, 27
Amsler grid testing, 45–46, 88 Central 30° threshold automated perimetry, 52
Anosognosia, 152 Central visual disorders, neuropsychological examination
Anterior junction syndrome, 90–92 bells test, 149, 151
Antiplatelet therapy, 35 Boston diagnostic aphasia examination, 149
Anton syndrome, 86, 152, 153 decreased visual acuity, 149
horizontal line bisection task, 149
scoring sheet, 149, 151
B symbolic functions, 149
Bálint syndrome, 15, 154 unilateral visual neglect, 149, 150
Bells test, 149, 151 Cerebral achromatopsia, 25, 27, 153
Bilateral homonymous visual field defects Cerebral ischemia, 32
bilateral superior homonymous quadrantanopia, 84 Cerebral stroke, 26
causes, 83 Charles Bonnet syndrome, 152
cerebral blindness, 83–85 Chromatic pupillography, 108, 112, 113
checkerboard visual fields, 85 Color anomia, 158
cortical blindness, 86 Color desaturation, 45
occipital lobe lesions, 83, 84 Color vision, 22–23
superior/inferior altitudinal defects, 84–85 Confrontation perimetry
visual field loss patterns, 85 in children, 55–56
Bilateral inferior altitudinal defects, 84, 85 in infants, 55
Bilateral superior homonymous quadrantanopia, types, 52–53
84, 85 Congenital hemianopia, 60–61
Binasal hemianopias, 61, 87 Congruous homonymous quadrantanopias, 81
Binocular perimetry, 62 Congruous homonymous visual field defects, 68
Binocular vision, 18 Contralateral homonymous hemianopic central scotoma, 152
Bitemporal hemianopias, 9, 46, 49–52, 61, 87–90 Contralateral relative afferent pupillary defect, 49
Contrast sensitivity, 28 F
CO poisoning. See Carbon monoxide (CO) poisoning Finger counting confrontation, 54–55
Cortical blindness, 152 Finger wiggle testing, 54
Cortical magnification, 89, 90 Flash stimulus visual evoked potentials, 148
Cortical processing, 19, 21, 25–26 Fluent reading, 46, 47
Cortical visual areas, 146–147 Functional field maps (FFMaps), 102
Cortical visual perception, hierarchy of, 22–26 Functional magnetic resonance imaging (fMRI)
Covert vision sign, 153 advantages, 101
application, 103
BOLD signal, 101, 102
D
general fMRI designs, 101
Dementias, 28
laminar high field, 103
Diffusion tensor imaging (DTI), 101
neurovascular uncoupling, 103
apparent diffusion coefficient, 97
oxyhemoglobin-sensitive, 101
color-coded directionality images, 98
principle, 101
fiber tracking, 100
visual field mapping, 102
fractional anisotropy index, 97
visually evoked potentials, 102
limitations, 100
Functional visual disorders, 158
multiple sclerosis, 99
Functional visual loss, 61–63
quantitative parameters, 97
Funduscopy, 56–58
tractography, 99
usefulness, 99
Diffusion-weighted imaging, 97 G
Dilated funduscopy, 56 Gerstmann syndrome, 158
Diplopia and hypo-accommodation, 170 Goldmann kinetic perimetry, 52–54, 61
Dominant parietal lobe lesions, 152
Dorsal simultanagnosia, 157
H
Dorsal stream disorders, 147
Half-moon syndrome, 82
Driving
Hemiakinesia, 115
action-perception cycle, 136
Hemianopia
attention, 137
binasal, 61, 87
cognitive process, 137
bitemporal, 9, 46, 49–52, 61, 87–90
complexity, 136
congenital, 60–61
decision-making, 137
early-onset, 60–61
demands, 136–137
fixation shifts, 122–123
fitness assessment
homonymous (see (Homonymous hemianopia))
in on-road studies, 141–142
monocular, 86–87
in simulated environments, 138–141
incomplete homonymous, 68, 152
human-machine-environment interaction, 136
missing half, 61, 62
motor process, 137
organic monocular temporal, 86
planning, 137
scanning patterns, 126–130
sensory process, 137
unilateral nasal, 87
visual functions, 137
Hemianopic dyslexia, 46, 129, 130
working memory, 137
Hemlanopic orientation disorder
Dysconnection syndrome, 154
clinical presentation, 166
quality of life, 166
E rehabilitation
Endovascular treatment, in acute cerebral artery adult visual cortex, plasticity of, 170
occlusion, 33–35 antisaccade training, 169
Epileptic seizures, 27 compensatory approach, 168–169
Evoked saccadic technique, 55 consultation, 169
Exotropic eye, 60 explorative saccade training, 168–169
Exploratory field-of-view (EFOV) testing, 142 optical devices, 169
Extrastriate visual cortex, 13–14 restitutive training, 169–170
Eye movements, 121, 122 spontaneous adaptive mechanisms
and reading, 129, 130 attention shift, 168
saccades exotropia, 168
blind contralateral hemifield targets, 123–124 head turn, 167–168
blindsight, 124–125 saccadic tasks, 167
ipsilateral hemifield targets, 123 scanning eye movements, 166–167
Index 177
P
N Paracentral homonymous hemianopic scotomas
Neuroimaging techniques, 95 Amsler grid testing, 88
Neurological examination axial T1 and T2 MRI, 85–87
nosological diagnosis, 147 cerebrovascular accidents, 88
patient’s history, 147 Parvocellular retinal ganglion cells, 5–6
signs and symptoms, 147 Parvocellular visual pathway, 26
syndromological diagnosis, 147 Perceptual span, 163
topical diagnosis, 147 Photopic vision, 21–22
visual evoked potentials, 148 Postchiasmal lesions, visual acuity in, 45
visual field test, 147–148 Postfixational blindness, 49–52, 62
Neuro-ophthalmological examination, 43 Posttraumatic cortical blindness, 86
Amsler grid, 45–46 Primary visual cortex
color vision testing, 45 columnar organization, 13
funduscopy, 56–57 lesions of, 15
monocular/binocular vision loss, 44 Prosopagnosia, 154, 157
neuropsychological testing, 60 Pupillary hemihypokinesia, 115–118
optical coherence tomography, 59 Pupillary light reflex, neural pathway of, 108
patient history, 44 Pupil light reaction, 108, 113, 115, 116
reading ability, 46–49 chromatic pupillography, 112
visual acuity, 45 pupil perimetry, 109
visual field assessment swinging flashlight test, 109
central 30° threshold automated perimetry, 52 Pupil perimetry
confrontation perimetry (see (Confrontation M-sequence techniques, 109
perimetry)) pituitary adenoma, 109, 111
evoked saccadic technique, 55 problem, 109
finger counting confrontation, 53–55 retinitis pigmentosa, 109, 112
Goldmann kinetic perimetry, 52 sphenoid wing meningioma, 109, 110
macular sparing, 57 visual field defects, 109
Neuro-ophthalmological/orthoptic examination, 170 Pupils, examination of
Neuropsychological examination, in central visual chromatic pupillography, 112, 113
disorders. See Central visual disorders, pupil perimetry, 109–112
neuropsychological examination relative afferent pupillary defect, 109
Neuropsychological testing, 60 swinging flashlight test, 109
Neurovascular uncoupling (NVU) effect, 103 Pure alexia, 158
Nondominant parietal lesions, 152
Nosological diagnosis, 147
Q
Quadrantanopias, 35. See also Homonymous
O quadrantanopias
Object anomia, 158
Octopus semiautomated kinetic perimetry, 52–54
Ocular dominance, 20, 23 R
Ocular motility, 49, 52 RAPD. See Relative afferent pupillary defect (RAPD)
Oculomotor apraxia, acquired, 154 Reading
OFF-neurons, 3 contrast sensitivity, 171
ON-neurons, 3 disorders of, 46
On-road driving assessment, 141–142 eye movements, 163, 164
Optical coherence tomography (OCT), 59–60 macular sparing, 163, 164
Optic ataxia, 154 macular splitting, 163, 164
Optic chiasm, 8 nonword search tasks, 166
Index 179