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an electroencephalogram (EEG) revealed continuous focal slowing in the right hemisphere over the
posterior regions, with no epileptic activity. Administration of valproic acid was begun.
During the next 6 months, the headaches
gradually resolved, but 6 months later, they recurred daily. The patient rated their severity as 8 to
9 on a scale of 1 to 10; they were preceded at times
by a bubbling feeling in his neck and were associated with nausea and vomiting. On several
occasions, his body stiffened and then went limp,
while his eyes remained open and staring; this
was followed by recovery within 30 seconds. An
EEG showed mild, diffuse background slowing
and superimposed intermittent posterior slowing,
with right-sided predominance and no seizure activity. The serum prolactin level was 25.6 ng per
milliliter (reference range, 0.0 to 15.0). MRI of the
brain was again normal. Magnetic resonance angiography (MRA) of the head and neck and computed tomographic angiography (CTA) showed no
abnormalities. Ophthalmologic ultrasonography
was normal.
During the next 3 years, the patient was seen
in the emergency department of this and other
hospitals multiple times; he was repeatedly admitted to the hospital for headaches associated
with visual changes, nausea, and occasionally leg
cramps. Therapy included intravenous fluids, narcotic analgesics, valproic acid, and ondansetron
for the acute episodes; trials of atenolol, nortriptyline, amitriptyline, gabapentin, butalbital, cyproheptadine, topiramate, and riboflavin for prophylaxis; and other medications (ibuprofen, naproxen,
zolmitriptan, frovatriptan, and sumatriptan tablets and nasal spray) for abortive therapy, which
he used with variable adherence. He saw a psychologist and had some improvement with biofeedback techniques.
When the patient was between 19 and 20 years
of age, episodes of syncope, weakness, and difficulty standing occurred with the headaches, and
the pain radiated to the neck and upper back. Repeated physical, neurologic, and ophthalmologic
examinations disclosed no abnormalities. At 20
years of age, he described photopsias (the sensation of perceiving lights or colors) and partial loss
of vision in the right peripheral visual field. On
evaluation by an ophthalmologist, bilateral opticdisk edema and splinter hemorrhages were seen.
In the emergency department of this hospital,
examination revealed bilateral blurred optic-disk
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of
m e dic i n e
Variable
Color
Colorless
Age of Patient
20 Yr 1 Mo
21 Yr 1 Mo,
5th Hospital Day
Colorless
Colorless
Turbidity
Clear
Clear
Clear
Xanthochromia
None
None
None
Tube 1
1667
202
Tube 4
1244
92
Tube 1
05
Tube 4
05
Neutrophils
28
Lymphocytes
68
72
28
555
47
32
Monocytes
Protein (mg/dl)
Glucose (mg/dl)
5075
73
69
Nonreactive
Nonreactive
Nonreactive
Grams stain
No organisms
No organisms
No organisms
No organisms
No organisms
No organisms
No organisms
No organisms
Routine
No growth
No growth
No growth
Fungal
No growth
No growth
No growth
Mycobacterial
No growth
No growth
No growth
Cryptococcal antigen
Negative
Negative
Negative
Negative
Cultures
Test not performed, white- Test not performed, whitecell count and protein
cell count and protein
too low
too low
* Reference values are affected by many variables, including the patient population and the laboratory methods used. The
ranges used at Massachusetts General Hospital are for adults who are not pregnant and do not have medical conditions that could affect the results. They may therefore not be appropriate for all patients.
To convert the values for glucose to millimoles per liter, multiply by 0.05551.
disk margins, and a small flame-shaped hemorrhage at the superior nasal margin of the left
optic disk.
On the fifth day, the patient again reported
numbness in the left groin and leg; neurologic
examination was normal. A lumbar puncture was
performed. The opening pressure was 25 cm of
water; results of CSF analysis are shown in Table 1.
Cytopathological examination revealed no malignant cells, and flow cytometry showed normal
lymphocytes. Two hours after the procedure, the
patient felt constipated; he was able to defecate but
reported a lack of rectal sensation. On examination, there was decreased sensation in the area of
the dermatomes of sacral nerves S1 through S4 on
the left and decreased perianal sensation and rectal tone; strength in the legs was normal, but the
left patellar and ankle reflexes were diminished.
A diagnostic procedure was performed.
Differ en t i a l Di agnosis
Dr. Kalpathy S. Krishnamoorthy: On initial evaluation,
when he was 16 years of age, and on several follow-
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Vascular causes
Meningitis
Recurrent meningitis may cause intermittent meningeal symptoms and CSF abnormalities, followed
by periods of normal clinical and CSF findings.18-20
Patients may have recurrent headache, fever, nausea, change in mental status, nuchal rigidity, seizures, focal neurologic findings, cranial-nerve palsies, and papilledema.21 Examination of the spinal
fluid reveals an elevated opening pressure with
pleocytosis, normal or elevated protein levels, and
a variable glucose level, depending on the cause.
MRI may show meningeal enhancement. Our patient had no fever or meningismus, repeated neuroimaging did not show meningeal enhancement,
and repeated CSF examinations did not support
this diagnosis.18-20
Central Nervous System Neoplasm
ARTIST: mst
FILL
H/T
Combo
H/T
SIZE
16p6
bar spine
(Fig.
2A)redrawn
reveals
hyperintense
Figure
has been
and a
type
has been reset. extraPlease check carefully.
dural mass that nearly
fills the sacral canal, markedly JOB:
compressing
the sacral-nerve
roots. The mass
36124
ISSUE: 12-10-09
is markedly hypointense on T1-weighted MRI and
has no enhancing components (Fig. 2B), suggesting a cyst. Expansion of the sacral canal, with deep
scalloping of the bodies of the sacral segments,
indicates a chronic, gradually expanding cyst.
A bulb-shaped component extends through and
expands the left S2S3 anterior sacral foramen
(Fig. 2C), suggesting a perineural cyst arising from
the sheath of the left S2 nerve root.
A sagittal reformatted image from a CT myelogram (Fig. 2D) shows injected contrast material
in the subdural space and the thecal sac but not
within the cyst, indicating that the wide-open
communication between the thecal sac and the
cyst that would be expected in a meningocele or
pseudomeningocele is missing. However, a left
parasagittal image (Fig. 2E) shows contrast lay-
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Cl inic a l Di agnosis
Spinal arachnoid cyst.
CASE
TITLE
Revised
n
j med 361;24 nejm.org
10, 2009
4-C
Line december
SIZE
Enon
ARTIST: mst
H/T
H/T
The New England
Journal of Medicine
33p9
FILL
Combo
EMail
engl
Downloaded from nejm.org on May 7, 2013. For personal use only. No other uses without permission.
AUTHOR, PLEASE NOTE:
Copyright
2009
Massachusetts
All rights reserved.
Figure
has been
redrawn andMedical
type has Society.
been reset.
Please check carefully.
2375
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n e w e ng l a n d j o u r na l
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A
Epidural
space
Subarachnoid
space
B
Nerve root
Dura
Subarachnoid
space
Arachnoid
S1
Slit valve
opening
Ilium
Cyst
Nerve roots
L5
S1
Subarachnoid
space
Cyst
Cyst
S2
Figure 5. Diagram of the Spinal Arachnoid Cyst and Its Possible Effect on the Flow of Cerebrospinal Fluid (CSF).
COLOR FIGURE
The cyst was located in the subarachnoid space and protruded through a small defect in the dura, compressing the lumbosacral nerve
Rev3
11/18/09
roots (Panel A). There are several possible explanations for the associated increased intracranial pressure. First, the cyst may have
produced
a mass effect on the subarachnoid space. Second, its presence may have led to a reduction in the surface
area of arachnoid
granulations in
Author
Dr. Brass
that region and therefore a reduction in the absorption of CSF. A third theory specifically addresses the presence
of paroxysmal
spikes in
Fig #
5
intracranial pressure associated with changes in position a slitlike opening in the cyst could have acted
as a valve, resulting in intermitTitle
tent release of cyst fluid into the subarachnoid space. (Panel B, arrow), with consequent sudden increases
MEin intracranial pressure. After
partial removal of the cyst wall, the slit valve would have been removed, but the open connection between the subarachnoid space and a
Phimister
DE
developing pseudomeningocele (Panel C, arrows) may have further reduced the capacity of spinal arachnoid granulations to absorb CSF,
Muller
Artist
leading to a more protracted (although milder) state of high intracranial pressure that was necessary for the emergence of chronic papilleAUTHOR PLEASE NOTE:
dema and associated visual-field changes.
Figure has been redrawn and type has been reset
Please check carefully
aches ceased. However, he now described a persistent low-grade (1 on a scale of 1 to 10) headache.
Three months postoperatively, he reported translucencies in his vision.
Dr. Dinkin: Neuro-ophthalmologic examination
revealed worsening papilledema, with hemorrhage (Fig. 1B), and on visual-field testing, there
was a constriction of the inferior nasal visual field,
reflecting damage to the retinal nerve-fiber layer,
which occurs with chronic papilledema and tends
to affect the superior fibers.42
Dr. Copen: A sagittal T2-weighted image of the
lumbar spine approximately 1 month after spinal
surgery (Fig. 4A) shows a large fluid collection
extending from the original location of the cyst
through a new multilevel laminectomy defect. It is
unclear whether this represents cyst reexpansion,
a postoperative seroma, or a pseudomeningocele.
Mass effect on the thecal sac has increased since
the preoperative examination. A repeat examina2376
nejm.org
Issue date
References
1. Rothner AD, Linder SL, Wasiewski
2377
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2378