CARDINAL MANIFESTATIONS

OF NEUROLOGIC DISEASE

Peng Yu
Approaches to neurological diseases
nature of the lesion
 Congenital or developmental
 Vascular
 Neoplastic
 Traumatic
 Systemic – toxic
 Degenerative
 Infectious
 Inflammatory, autoimmune
 Epileptic
 Psychiatric
 Types of Lesions
 Focal:
brain tumor, stroke
 Multifocal:
multiple sclerosis, multiple metastasis
 Diffuse:
infection, metabolic, dementia
 System:
motor neuron disease, multiple sclerosis,
olivopontocerebellar atrophy
 Disorders of
Consciousness
 Consciousness is awareness of the
internal or external world
 The state of consciousness can be
divided into 4 categories

 Alertness

 Drowsiness

 Superficial coma
 Deep coma
 Alertness
 the patients has a good awareness of himself
and the world around him.

 This includes the correct orientation to time,

place and person; the correcte responses when
interviewer asks patients his name, age,
location, time of day
 Drowsiness
 a decrease of alertness
 it is also an early manifestation of disorder of
consiousiness
 The patient has a low spirit and often continues in
a state of sleeping but can be roused with repeated
external stimuli, then the patient is able to answer
simple questions
 Once the external stimuli are removed, the patient
falls asleep again
 Superficial coma
 A manifestation of a more severe disorder of
consciousness.
 the patients cannot be roused by using external
stimulus, but patient will react to light and the
corneal reflex is still present.
 If doctor presses on the eye orbit, patient will
react with voluntary movement
 Deep coma

 the most severe manifestation of a

disorder of consciousness.
 The patient will not have any voluntary
movement.
 There is no reaction to light, no corneal
reflex, or reaction to pressure on eye orbit
 Persistent Vegetative State
 patients who are comatose because of
cerebral hypoxia or ischemia or structural
brain lesions
 regain wakefulness but not awareness.
 After 1 month, this condition is termed
persistent vegetative state
 Persistent Vegetative State
 patients exhibit spontaneous eye opening
and sleep-wake cycles, which distinguish
them from patients in coma, and
demonstrate intact brainstem
 Persistent Vegetative State

 they neither comprehend nor produce

language, and they make no purposeful
motor responses.
 This condition may persist for years
 syncope
Syncope is the transient loss of
consciousness and posture that results from a
global reduction in blood flow to the brain.
 Vasovagal syncope
 There is a subsequent reduction in cardiac
output,which is followed by vagal
stimulation and thus a bradycardia

 Precipitating situations are strong emotion,

sudden intense pain, and prolonged
standing in hot crowed areas.
 Vasovagal syncope
 The patients is usually upright at the onset
syncope

 Prodromal symptoms include feeling light-

headed, gradual dimming of vision, nausea,
and sometimes vomiting
 Vasovagal syncope
 The attack can be aborted if the patient
assumes the supine position

 The patient is pale and clammy,the pulse

almost imperceptible,the systolic blood
pressure drops to about 60 mmHg
 Micturition syncope

Micturition syncope
 Occurs in men who get up during the night
to pass urine
 Results from a combination of
vasodilatation(which occurs with emptying
of the bladder), a degree of postural
hypotension on standing and bradycardia
 Cough syncope

 Sustained coughing can elevate the

intrathoracic pressure sufficiently to impair
the venous return to the heat.
 Increase of the cerebrospinal fluid pressue,
vasoconstriction may also be contributory
 Cardiovascular Syncope
 Underlying cardiac disease:
 cardiac arrhythmias----usually profound
bradycardia
 ventricular outflow obstruction----

aortic stenosis, hypertrophic obstructive

cardiomyopathy
 right ventricular outflow obstruction----
pulmonary stenosis, pulmonary
hypertension, pulmonary embolism
 Cardiovascular Syncope

 Usually abrupt and without prodrome

 The upright position is not a prerequisite
 Loss of consiousness is brief and classically
accompanied by marked pallor with a rapid
return of colour as cardiac output is restored
 Cerebrovascular Syncope
Basilar Artery Insufficiency
 Basilar artery transient ischemic attacks usually
occur after the sixth decade
 The symptom complex of diplopia, vertigo,
dysphagia, dysarthria, various sensory or motor
symptoms, drop attacks, and occipital
headaches suggests diffuse brainstem ischemia.
 Cerebrovascular Syncope
 Attacks are typically sudden in onset and
brief in duration (seconds to minutes), but
when consciousness is lost, recovery is
frequently prolonged (30–60 minutes or
longer)
 Isolated unconsciousness without other
symptoms of brainstem ischemia is rarely
due to basilar artery insufficiency.
 Two-thirds of patients have recurrent
attacks, and strokes eventually occur in
about one-fifth of all cases
 Subclavian Steal Syndrome

 The subclavian steal syndrome results

from subclavian or innominate artery
stenosis that causes retrograde blood
flow in the vertebral artery, with
subsequent brainstem hypoperfusion
 Subclavian Steal Syndrome

 A difference between blood pressures

measured in the two arms is nearly
always found, the average difference
being a 45 mmHg (mercury) decrease in
systolic pressure in the arm supplied
by the stenotic vessel
 Differential diagnosis of transient
loss of consiousness
seizure syncope
Can occur on any Upright position
position and time of
day Usually Prodrome-(light
Sudden onset headache, nausea,ringing
in ears, dimming of vision,
Urinary or faecal pallor, sweating )
incontinence hypotension and thready
Tongue biting pulse
confusion,drowsiness, Relatively rapid recovery
 headache
 Headache is caused by traction,
displacement, inflammation, vascular spasm,
or distention of the pain-sensitive structures in
the head or neck.
Recurrent Chronic daily Subacute-onset Acute-onset
episodic headache headache headache
headache
Migraine Transformed Subdural Subarachnoid

Cluster headache migraine hematoma hemorrhage

Trigeminal Tension-type Intracranial Cerebral

neuralgia headache tumour hemorrhage

hypertension neuralgia Intracranial Meningitis

Post-traumatic abscess Acute

headache meningitis hydrocephalus
Atypical facial Giant-cell Hypertensive
pain arteritis crisis
Benign Acute glaucoma
intracrainal Fist episode of
hypertension migrain\cluster
headache
Visual impairment
 Visual field defect
1. Central scotoma caused by inflammation of
the optic disk or optic nerve

1. Total blindness of the eye from a complete

lesion of the right optic nerve
Lesions of the optic nerve include:
 Optic neuritis
 Optic nerve meningioma or glioma
 Visual field defect
Optic chiasm lesion: cause a bitemporal
hemianopia
 Lesions of the optic chiasm include:
Tumors
Cerebral aneurysm
tuberculosis,sarcoidosis
Optic tract and optic
radiation lesion: cause a
contralateral
homonymous
hemianopia
 Lesions of the optic
tract and optic radiation
include:
 Infarction,such as a
lesion of middle
cerebral artery
 Intracerebral
hemorrhage
 Tumours
 trauma
Occipital cortex lesions
 Unilateral lesions of the
posterior cerebral artery
cause a contralateral
homonymous hemianopia.

 Bilateral occipital cortex

damage(e.g. tumour,
trauma, infarction)give
rise to cortical blindness
characterized by visual
loss throughout the field
in both eyes and
preserved pupillary
responses.
 Visual impairment
Acute visual impairment
 Acute transient visual impairment
 Amaurosis
 Migraine with aura
 Acute persistent visual impairment
 Optic or retrobulbar neuritis
 Retinal or optic nerve ischemia
 Arterial thromboembolism of the middle or posterior
cerebral artery
Subacute visual impairment
 Optic atrophy
Disorders of the pupils
and eye movement
 Normal Pupils

 Normal pupils are 3–4 mm in diameter

and equal bilaterally; they constrict
briskly and symmetrically in response
to light.
 Normal pupils, however, are larger in
children and smaller in the old
 Miotic pupil

 small constricted pupil maybe caused

by:
 Senility
 Drugs: diazepam, pilocarpine
Chlorpromazine
 Miotic pupil
 Horner’s syndrome:
interruption of the sympathetic pathway gives
rise to Horner’s syndrome.
 This characterized by:
1. Pupil constriction
2. Anhidrosis of the ipsilateral side of the face
3. Enophthalmos: a sunken eye
 Miotic pupil
 small constricted pupil maybe caused by:

 Pontine lesion: bilateral unreactive pin-point

pupils in a comatose patient suggest a large
intrapontine hemorrhage causing bilateral
interruption of the sympathetic pathways

 Argyll Robertson pupils: an Argyll Robertson

pupil is small and irregular. There is no
response to light, but there is a response to
accommodation
Argyll Robertson pupil have characteristically
been associated with syphilis
 Mydriatic pupil
Large dilated (mydriatic )pupil
Causes:
 Lesions of the eye- e.g. damage to the iris in acute
glaucoma, trauma to the sphincter muscle
 Drugs-parasympathetic paralysis(atropine), sympathetic
stimulation(adrenaline)
 Third nerve or midbrain lesions
 Fixed Midsized Pupils

 Pupils fixed at about 5 mm in diameter are

the result of brainstem damage at the
midbrain level
 Pinpoint Pupils

 Pinpoint pupils (1–1.5 mm in diameter) in a

comatose patient usually indicate opioid
overdose or focal damage at the pontine
level.
 Under these conditions, the pupils may
appear unreactive to light.
 Pinpoint pupils are also caused by
organophosphate poisoning, miotic eye
drops, and neurosyphilis
 Disorder of eye movement
3rd (oculomotor) cranial nerves and nucleus
lesions give rise to
 Diplopia in all directions of gaze

 Lateral deviation of the eye

 Ptosis

 A dilated pupil unresponsive to light and

accommodation
Nerve IV
 The trochlear nerve innervates the superior
oblique muscle. Lesions of this nerve result in
defective depression of the adducted eye
Nerve VI
 Lesions of the abducens nerve cause lateral
rectus palsy, with impaired abduction of the
affected eye
 Actions of the extraocular muscles

MUSCLE PRIMARY ACTION SECONDARY ACTION OCULOMOTOR

NERVE
Medial rectus Adduction — III
Lateral rectus Abduction — VI
Superior rectus Elevation Intorsion III
Inferior rectus Depression Extorsion III
Superior oblique Intorsion Depression IV
Inferior oblique Extorsion Elevation III
Lesions of the Facial
Nerve
 Peripheral facial paralysis
 The corner of the mouth droops
 the eyelids will not close
 Bell's phenomenon- Upon attempted closure of the lids,
the eye on the paralyzed side rolls upward
 Food collects between the teeth and lips, and saliva may
dribble from the corner of the mouth
 The patient complains of a heaviness or numbness in the
face, but sensory loss is rarely demonstrable and taste is
intact
 If the lesion is in the middle ear portion, taste is lost over
the anterior two-thirds of the tongue on the same side
 If the nerve to the stapedius is interrupted,
there is hyperacusis (sensitivity to loud
sounds)

 Intrapontine lesions that paralyze the face

usually affect the abducens nucleus as well,
and often the corticospinal and sensory
tracts
 Lesions above the facial nerve
nucleus
(= central facial palsy).
 The eye can still be closed on the affected
side
 The forehead can be symmetrically
wrinkled
 Complete right peripheral
facial nerve palsy

a At rest, the right

corner of the mouth
and the right cheek
hang limply downward.

b The patient cannot close the eye on the side

of the weakness (right side).
The eyeball turns upward, and part of it
remains visible (Bell phenomenon
 Left central facial palsy.

a Impaired innervation of b As part of the central hemiparesis,

the lower portion of the face the motor innervation of the left side of
is evident when the patient the tongue is also impaired, and the
tries to show his teeth. tongue therefore deviates to the left
Movement Disorders
 Types of Abnormal Movements
Tremor
A tremor is a rhythmic oscillatory movement best
characterized by its relationship to voluntary motor
activity
 static tremor or rest tremor: occurs when the limb is
at rest, usually has a frequency of 4–6 Hz and is
characteristic of parkinsonism
 postural tremor:present during sustained posture
 intention tremor: present during movement but not at
rest, the arm exhibits tremor during movement, often
more marked as the target is reached
 Causes of Tremor
Postural tremor
 Physiologic tremor
 Enhanced physiologic tremor
1. Anxiety or fear
2. Excessive physical activity or sleep deprivation
3. Sedative drug or alcohol withdrawal
4. Drug toxicity (eg, lithium, antidepressants)
5. Heavy metal poisoning (eg, mercury, arsenic)
6. Carbon monoxide poisoning
7. Thyrotoxicosis
 Causes of Tremor
 Familial (autosomal dominant) or idiopathic
(benign essential) tremor
 Cerebellar disorders
 Wilson disease
 Intention tremor

 Brainstem or cerebellar disease

 Drug toxicity (eg, alcohol, anticonvulsants,
sedatives)
 Wilson disease
 Rest tremor

 Parkinsonism
 Wilson disease
 Heavy metal poisoning (eg, mercury)
 Chorea

The word chorea denotes rapid irregular muscle

jerks that occur involuntarily and unpredictably in
different parts of the body
 Hemiballismus
 Hemiballismus is unilateral chorea that is
especially violent because the proximal muscles
of the limbs are involved
 It is due most often to vascular disease in the
contralateral subthalamic nucleus and commonly
resolves spontaneously in the weeks following
its onset
 It is sometimes due to other types of structural
disease;
 Dystonia & Athetosis

 The term athetosis generally denotes

abnormal movements that are slow, sinuous,
and writhing in character
 When the movements are so sustained that
they are better regarded as abnormal
postures, the term dystonia is used, and
many now use the terms interchangeably
 Dystonia & Athetosis

 The abnormal movements and postures may

be generalized or restricted in distribution.
 one or more of the limbs may be affected
(segmental dystonia) or the disturbance may
be restricted to localized muscle groups
(focal dystonia)
 Tics

 Tics are sudden, recurrent, quick, coordinated

abnormal movements that can usually be
imitated without difficulty
 The same movement occurs again and again and
can be suppressed voluntarily for short periods
 Tics tend to worsen with stress, diminish during
voluntary activity or mental concentration, and
disappear during sleep
Disorders of equilibrium
 Vertigo

 Vertigo is the illusion of movement of the body

or the environment
 It may be associated with other symptoms, such
as impulsion (a sensation that the body is being
hurled or pulled in space), oscillopsia (a visual
illusion of moving back and forth), nausea,
vomiting, or gait ataxia
Characteristics of Peripheral Vertigo
1. Peripheral vertigo
 Tends to be intermittent, lasts for briefer periods,
and produces more distress than vertigo of
central origin
 Nystagmus (rhythmic oscillation of the eyeballs)
is always associated with peripheral vertigo; it is
usually unidirectional and never vertical
 Peripheral lesions commonly produce additional
symptoms of inner ear or acoustic nerve
dysfunction, ie, hearing loss and tinnitus
Characteristics of Central Vertigo
2. Central vertigo
 may occur with or without nystagmus; if
nystagmus is present, it can be vertical,
unidirectional, or multidirectional and may
differ in character in the two eyes
 Central lesions may produce intrinsic brainstem
or cerebellar signs, such as motor or sensory
deficits, hyperreflexia, extensor plantar
responses, dysarthria, or limb ataxia
 Differential Diagnosis

Peripheral Central
Vertigo Often intermittent; severe Often constant; usually less
severe
Nystagmus Always present; unidirectional, May be absent; uni- or
never vertic bidirectional, may be
vertical
Hearing loss or tinnitus Often present Rarely present

brainstem signs Absent Typically present

 Ataxia
 Ataxia is incoordination or clumsiness of
movement that is not the result of muscular
weakness. It is caused by vestibular, cerebellar,
or sensory (proprioceptive) disorders
 Ataxia can affect eye movement, speech
(producing dysarthria), individual limbs, the
trunk, stance, or gait
 Vestibular Ataxia

 Vestibular ataxia can be produced by the same central

and peripheral lesions that cause vertigo.
 Nystagmus is frequently present and is typically
unilateral and most pronounced on gaze away from the
side of vestibular involvement. Dysarthria does not
occur
 Vestibular ataxia is gravity dependent: Incoordination
of limb movements cannot be demonstrated when the
patient is examined lying down but becomes apparent
when the patient attempts to stand or walk
 Cerebellar Ataxia

 Cerebellar ataxia is produced by lesions of the

cerebellum or its afferent or efferent
connections in the cerebellar peduncles, red
nucleus, pons, or spinal cord
 The clinical manifestations of cerebellar ataxia
consist of irregularities in the rate, rhythm,
amplitude, and force of voluntary movements
Clinical Patterns of Cerebellar Ataxia
Pattern of Involvement Signs

Midline Nystagmus, head and trunk titubation, gait ataxia

Superior vermis Gait ataxia

Cerebellar hemisphere Nystagmus, ipsilateral gaze paresis, dysarthria

(especially left hemisphere lesion), ipsilateral
hypotonia, ipsilateral limb ataxia, gait ataxia, falling
to side of lesion
Pancerebellar Nystagmus, bilateral gaze paresis, dysarthria,
bilateral hypotonia, bilateral limb ataxia, gait ataxia
 Sensory Ataxia

 Sensory ataxia results from disorders that affect

the proprioceptive pathways in peripheral
sensory nerves, sensory roots, posterior columns
of the spinal cord, or medial lemnisci
 Examination reveals impaired sensations of
joint position and movement in the affected
limbs, and vibratory sense is also commonly
disturbed
Characteristics of Vestibular, Cerebellar, and Sensory
Ataxia
Vestibular Cerebellar Sensory

Vertigo Present May be present Absent

Nystagmus Present Often present Absent
Dysarthria Absent May be present Absent
Usually present (one Present (typically
Limb ataxia Absent limb, unilateral, legs legs)
only, or all limbs)
May be able to stand Unable to stand with Often able to stand
with feet together; feet together and with feet together
Stance typically worse with eyes either open or and eyes open but
eyes closed closed not with eyes closed
(Romberg sign)
Vibratory and Normal Normal Impaired
position sense
Ankle reflexes Normal Normal Depressed or absent
Motor Deficits
 Monoplegia ：denotes paralysis or severe weakness of
the muscles in one limb
 Monoparesis： denotes less severe weakness in one
limb, although the two words are often used
interchangeably
 Hemiplegia or hemiparesis is weakness in both limbs
(and sometimes the face) on one side of the body
 Paraplegia or paraparesis is weakness of both legs;
 quadriplegia or quadriparesis (also tetraplegia,
tetraparesis) is weakness of all four limbs
Neuromuscular-Transmission Disorders

Signs
 Normal or reduced muscle tone
 Normal or depressed tendon and superficial
reflexes
 No sensory changes
 Weakness, often patchy in distribution, not
conforming to the distribution of any single
anatomic structure; frequently involves the cranial
muscles and may fluctuate in severity over short
periods, particularly in relation to activity
 The Motor Unit

 One lower motor

neuron
(motoneuron)

 The
neuromuscular
junction

 The muscle fibers

it innervates
 What Can Go Wrong With the
Motor Unit
 Lower motor
neuron lesions or
infections;
peripheral nerve
injury

 Neuromuscular
junction disorders

 Muscle atrophy or
dystrophy