Neurology

Dopamine -> substantia nigra in the midbrain

Norepinephrine - locus ceruleus in the pons
Acetylcholine -> nucleus basalis of Meynert in the basal forebrain
(substantia innominata)
Serotonin > raphe' nuclei in the pons and medulla.

Arteries
The internal carotid artery (ICA) and its branches supply the anterior
circulation.
The anterior cerebral artery (ACA) and the middle cerebral artery
(MCA) are branches from the ICA.
The ACA supplies the basal and medial frontal lobes and the MCA
supplies the lateral frontal, farietal and temporal lobes.
The vertebro-basilar system comprises the posterior circulation.
The two vertebral arteries join at the ponto-medullary junction to
form the basilar artery.
The basilar gives off the superior cerebellar artery and the posterior
cerebral arteries (PCA).
The PCA supplies the occipital lobe and the ventral and medial
temporal lobes.

Tremors
Intention tremors are fine movements of the hands that are absent
at rest, occur with activity, and worsen as the target is neared. They
occur with multiple sclerosis and cerebellar disease.
Choreiform movements are brief, rapid, jerky, unpredictable
motions of the hands. They occur both at rest and during normal
actions. They are associated with both Huntington's and Sydenham
choreas.
Asterixis is non-rhythmic flapping movements of the hands,
especially if the wrists are dorsiflexed. It is associated with liver
failure, renal failure, and pulmonary insufficiency.
Static, or resting, tremors are coarse movements of the hands that
are present at rest, disappear with movement, and may involve
alternation of the fingers in a "pill-rolling" manner.They are
associated with Parkinson's syndrome and extrapyramidal
dysfunction.
Postural tremors are fine movements of the hands that occur when
the hands are held in an active position, usually against gravity.
Examples of postural tremors are those resulting from anxiety,
fatigue, and hyperthyroidism, as well as familial postural tremors.
ur patient most probably has Parkinson disease or parkinsonism
(tremor in rest, rigidity, bradykinesia, postural instability).
Parkinsonian gait is characterized by the absence of arm swings,
small shuffling steps, hypokinesia, and reduced walking speed. The
patient has difficulty starting and stopping after starting. Freezing
of gait is most often seen in the advanced stages of the disease. It
is typically transient and lasts less than a minute, during which gait
is halted and the patient feels that his/her feet are glued to the
ground. When the patient overcomes the block, walking can be
performed relatively smoothly. The most common form of freezing
is “start hesitation”, which is when the patient wants to start
walking. It is provoked by placing a patient in a narrow space or in
stressful situations, such as in the doctor’s office. Later in the course
of the disease, it can appear spontaneously.

Titubation is seen when the patient's trunk moves from side to

side as he/ she walks; this is called truncal ataxia. It is a sign of
cerebellar disease (usually the midline of cerebellum in cerebellar
degenerations).
Spastic gait is characteristic of upper motor neuron disease. It is not
transient. Hemiparetic gait involves 1 leg. In a hemiparetic gait,
the spastic stiff leg is circumducted (the leg swings in a semicircle
from a medial to lateral direction) and the toe hits the ground before
the heel does. Often, the arm on the paretic side does not swing and
is flexed at the elbow.Spasticity causes the extremity to be stiff. It is
not transient. In paraparetic spastic gait, both legs are stiff and the
arms are moving much more than the legs. Thighs are adducted,
giving the picture of a "scissors" gait.

A slapping gait occurs in lower motor neuron disease or poor

position sense in the feet. With a foot drop, the patient flexes the
hip so that the foot will clear the floor. Patients with poor position
sense have a wide-based gait; their feet slap the floor, and they
require visual assistance to walk.
Parkinson’s Disease
o 4-6 Hz
◦ ◦ ◦ ◦ Shuffling gate
◦ ◦ ◦ ◦ Cogwheel type gate

Nuclei
Most of the thalamic subnuclei are named for their anatomic
position within the structure itself. The following subnuclei are
named with their corresponding efferent projections.
ventral posterolateral (VPL) - primary sensory cortex
pulvinar -> inferior parietal lobule
lateral geniculate (LG) -> primary visual cortex (occipital lobe)
medial geniculate (MG) - auditory cortex (temporal lobe) (M-
Mesmis)
dorsomedial (DM) - prefrontal cortex
ventrolateral (VL) - primary motor cortex anterior > cingulate gyrus
These same subnuclei have important afferent connections also that
correspond with their output projections:
A medial lemniscus and spinothalamic tracts - VPL
mammillary body & mammillothalamic tract -> anterior nucleus
(limbic)
optic tract -> LG
lateral lemniscus and inferior colliculus - MG
amygdala & temporal neocortex - DM
dentate nucleus, globus pallidus, & substantia nigra-›VL (cerebellar
& basal ganglionic)

Explanation CRANIAL NERVE EXAMINATION

I-olfactory: aromatic odors
1I-optic: vision with Snellen, fundus, visual fields
III-oculomotor,IV-trochlear, VI-abducent: inspect eyelids for
dropping, inspect pupils size for equality, and their direct and
consensual response to light and accommodation, assess cardinal
fields of gaze
V-trigeminal: check face for muscle atrophy and tremors, jaw
muscles for tone and strength, superficial pain and touch sensation,
corneal reflex
VII-facial: symmetry of facial features (smile, frozen puffed cheeks,
etc.), identification of sweet and salty on each side of tongue
VIII-acoustic: hearing-audiometry, bone conduction, sound
lateralization
IX-glossopharyngeal: sour and bitter taste, gag reflex and ability to
swallow
X-vagus: palate and uvula for symmetry with speech sounds and
gag reflex, swallow difficulties, presence of nasal or hoarse quality
to voice
XI-spinal accessory: strength of trapezius and
sternocleidomastoideus muscles
XII-hypoglossal: tongue for symmetry, tremors and atrophy, tongue
movements, strength against cheek, quality of lingual speech
sounds (1, t, d, n).

Central cord
Hemi-cord syndrome (Brown-Sequard Syndrome) usually results
from either a traumatic injury (bullet or knife), or less often, from a
neoplastic process (epidural compression). This causes loss of pain
and temperature sensation (spinothalamic) on the contralateral
side with loss of proprioception, and vibratory sensation (dorsal
columns) on the ipsilateral side. Usually there is an associated
motor deficit on the ipsilateral side due to lateral corticospinal tract
(LCS) involvement.
 Central cord syndrome can be seen in syringomyelia where a
central cystic lesion involves the central gray matter and the
spinothalamic fibers as they cross in the anterior commissure.
The lesion results in a dissociated sensory loss pattern to pain
and temperature modalities bilaterally sparing dorsal column
sensation. Often less common causes include an
intramedullary tumor and trauma (hematomyelia).
 Subacute combined degeneration involves the lateral
corticospinal tract and the dorsal columns causing impairment
of proprioception and vibration.
There is also a spastic paraparesis resulting from an upper
motor neuron lesion in the LCS tract. By far the most common
cause is vitamin B12 deficiency.

• Anterior spinal artery syndrome most often involves infarction in

this artery that supplies the anterior two-thirds of the spinal cord.
Areas affected usually include the spinothalamic tract, corticospinal
tract, and the ventral gray matter. Resulting symptoms include
bilateral loss of pain and temperature sensation below the level of
the lesion. Dorsal column modalities are usually spared since the
dorsal columns are supplied by the posterior spinal artery. Also,
weakness below the lesion is usually due to a combination of
involvement of the corticospinal tract and ventral gray matter (lower
motor neurons).

• Amyotrophic lateral sclerosis (ALS) is a neurodegenerative process

that involves both the upper (corticospinal tract) and lower motor
neurons (ventral gray matter). Corresponding symptoms are due to
concomitant involvement of these two systems with resulting
spasticity with hyper-reflexia along with a Babinski response (upper
motor neuron). Lower motor neuronal involvement results in
progressive muscle atrophy and fasciculations.
Gradually progressive weakness results from both upper and lower
motor involvement.

Subdural hematoma is a brain disorder involving a collection of

blood in the space between the inner and the outer membranes
covering the brain, where symptoms usually develop within a short
time after a brain injury. Subdural hematoma develops when blood
veins that are located between the membranes covering the brain
(the meninges) leak blood after an injury to the head (whether
trauma or a minor bump). Pressure damages the brain tissue and
causes a loss of brain function, which may progressively worsen as
the hematoma enlarges and intracranial pressure (pressure within
the head) increases.
Acute subdural hematoma progresses rapidly, with symptoms
usually appearing within 24 hours of the injury. Rapid deterioration
occurs thereafter. Subacute subdural hematoma usually develops
symptoms within 2 to 10 days after the injury because of a slightly
slower leakage of blood into the subdural area.
Symptoms include recent injury or trauma to the head, loss of
consciousness after original injury, drowsiness, headache (steady or
fluctuating), impaired vision (visual loss), eyes retract into the
socket (enophthalmos), unilateral eyelid drooping, decreased
sensation or numbness, inattention to the environment on the same
side as the sensory loss, unilateral loss of movement (paralysis),
confusion and delirium, changes in personality, decreased memory,
slow thought process, impaired speech (slurring or inability to
understand), and absent sweating on 1 side of the forehead.
Examination may show focal neurologic deficits (localized, specific
brain function changes such as decreased movement or sensation).
Reflexes may be abnormal. The skull may be tender to when gently
tapped on 1 or both sides. Examination may reveal a need for
emergency surgery to relieve pressure within the head without
further testing to pinpoint the location and type of injury. A head CT
scan confirms the diagnosis of subdural hematoma and will pinpoint
the exact location of the hematoma. This disease may also alter the
results of a cranial MRI. Treatment goals include lifesaving
measures, control of symptoms, and minimizing or preventing
permanent brain damage. Lifesaving measures may include support
of breathing and/or circulation. Corticosteroid medications such as
dexamethasone may be used to reduce inflammation. Diuretics may
also be used to reduce swelling. Anticonvulsant medications, such
as phenytoin, may be used to control or prevent seizures.
Epidural abscess is a disorder characterized by inflammation and
a collection of infected material (pus) between the dura (the outer
membrane covering of the brain and spinal cord) and the bones of
the skull or spine. The infection is usually bacterial (staphylococcus
is common) but may be fungal or viral. Enlargement of the abscess
and swelling in conjunction with inflammation cause symptoms
because of compressed tissues in the brain and spinal cord.
Symptoms include deep, localized tenderness in the ear, nose, or
back, headache (progressively worsens), fever, excessive sweating,
general discomfort or uneasiness (malaise), and nausea and
vomiting. Purulent drainage from the ear may occur if the abscess is
caused by an ear infection and the tympanic membrane ruptures.
Other symptoms include variable neurologic losses (that includes
speech difficulty, a decrease in sensation of any area of the body,
weakness or decreased movement of any area of the body, vision
changes, hearing difficulty), seizures (uncommon), and pain
radiating from the midbody into an extremity. Examination of the
spine by touch and percussion may show localized pain in the spine,
which is worsened by examination of the area. Persistent headache,
fever, and/or back pain in a person with a known infection may
indicate epidural abscess. Head and/or spine X-rays, a head CT
scan, or an MRI of the head confirm epidural abscess. Dye may be
necessary for a CT scan or an MRI to show a clear picture of the
abscess. Fluid drained from the abscess during surgery is usually
cultured to definitively show the abscess and the causative
organism. The goal of treatment is to cure the infection and reduce
the risk of permanent neurologic damage. Antimicrobial medications
are given. Antifungal or antiviral medications may be needed if
there is a significant risk for fungal or viral infection. Corticosteroids
may occasionally be prescribed to reduce swelling and pressure on
the spinal cord or brain. Surgery is usually eminently necessary
because the lining (membrane) that encloses the abscess may
prevent the antimicrobial medications from reaching the
microorganism. It may also be indicated to reduce pressure on the
spinal cord or brain.
Cerebral abscess is a space-occupying lesion in the brain caused
by an inflammation and a collection of infected material within the
brain tissue. Cerebral abscess may be a complication of epidural
abscess. Chronic ear infection, chronic sinusitis, and mastoiditis are
other initial sources of the infection that causes cerebral abscess.
The infection may also spread through the blood vessels from
distant locations in the body. Lung infections, such as empyema or a
lung abscess, are common sources. Other sources include infections
of the teeth, skin, bone, or heart.
Symptoms include headache, stiff neck, shoulders, or back,
vomiting, changes in mental status (drowsiness, confusion,
inattention, irritability, slow thought processes, decreasing
responsiveness, eventual coma), seizures, fever, focal neurologic
deficits (vision changes, muscle function/feeling loss, unilateral
decreased sensation, unilateral decreased movement, unilateral
weakness of an area, decreased speech (aphasia), and loss of
coordination. Symptoms may develop gradually over a period of 2
weeks, or they may develop suddenly. Once symptoms occur, they
progressively worsen. A patient history and physical examination
characteristically reveals signs of increased intracranial pressure
and focal neurologic deficits.
A neurologic examination shows losses that correlate to the location
of the abscess. A CBC may indicate infection or inflammation. Tests
should include blood cultures (reveal any bacteria in the
bloodstream), chest X-ray (reveal lung infections, EEG (may be
abnormal if seizures or focal neurologic deficits are present), cranial
CT scan or MRI of head (shows the abscess and its exact location).
Cerebral abscess is a medical emergency. Intracranial pressure may
become high enough to cause fatal brainstem herniation. Broad-
spectrum antibiotics are the most common antimicrobial prescribed.
Antifungal medications (or antiviral medications) may also be
prescribed if fungal or viral infection is likely. Osmotic diuretics are
used to reduce swelling of the brain. Corticosteroids also might be
used to reduce brain swelling, but their use is more controversial.
Surgery is indicated if there is persistent or progressive increase in
intracranial pressure if the mass does not reduce after use of
antimicrobial medications or if the mass contains gas.
Spinal cord abscess is a disorder characterized by inflammation
and a collection of infected material between the spinal cord and
the spine (epidural). This is very rare. Pus forms as a collection of
fluid, destroyed tissue cells, white blood cells, and live and dead
microorganisms. The infection is usually bacterial, often a result of
staphylococcus infection that spreads through the bones and
membranes of the spine. In some rare cases, the infection may be
fungal or viral. The risks include boils (furuncles) on the skin,
particularly on the back or scalp. Abscess may also result from the
spread of any infection through the bloodstream from another body
location. Symptoms include fever, chills, backache in the lower back
(mild, progressive and typically radiating to an extremity), sudden
or rapid progression of neurologic symptoms, loss of movement of
an area of the body, weakness or paralysis, loss of sensation of an
area of the body, numbness in a specific, limited area (localized),
loss of bladder or bowel control, and male impotence. An
examination often shows localized tenderness over the spine. A
neurologic examination may indicate spinal cord compression, with
involvement of the lower body (paraplegia) or the entire trunk,
arms, and legs (quadriplegia). The extent of neurologic losses
corresponds with the location of the lesion. A CSF examination may
be normal or may contain a few white blood cells and slightly
elevated protein levels. A CT scan or MRI confirm epidural abscess.
The goals of treatment are relief of spinal cord compression and
eradication (cure) of the infection. Broad-spectrum antimicrobials or
a combination of antibiotics are the most commonly used
antimicrobials. Corticosteroids may occasionally be prescribed to
reduce swelling and compression of the spinal cord.
Lobar intracerebral hemorrhage occurs when there is bleeding
in the superficial (surface) white matter of the cerebrum (part of the
brain). Lobar intracerebral hemorrhage may be caused by trauma
(brain injury) or abnormalities of the blood vessels, such as
aneurysm, arteriovenous malformation (AVM), or angioma (tumors
involving blood vessels). The disorder is likely to be associated with
amyloid deposits in the blood vessels (amyloid angiopathy). Blood
irritates the tissues of the brain and may cause swelling (cerebral
edema) while the blood collects into a mass (hematoma). Both
cerebral edema and the presence of a hematoma within the brain
will put increasing pressure on the tissues of the brain and destroy
those tissues. Risks for lobar intracerebral hemorrhage (in addition
to the causative disorders) include various blood/bleeding disorders
(disseminated intravascular coagulation, hemophilia, sickle cell
anemia, leukemia, decreased levels of blood platelets) use of aspirin
or anticoagulant medications ('blood thinners'), liver disease (which
is associated with increased bleeding risk), cerebral amyloid,
infection, and autoimmune disorders.
Symptoms usually develop suddenly without warning, often during
activity. Symptoms include change in alertness (level of
consciousness), vomiting, headache, vision changes, decreased
sensation (numbness and tingling), difficulty swallowing, difficulty
speaking or understanding speech, difficulty writing or reading, loss
of coordination and loss of balance. Treatment goals may include
lifesaving interventions such as intubation and hyperventilation (a
breathing tube is inserted and the person is forced to breath rapidly;
this reduces pressure in the brain). Treatment goals may also
include supportive measures or control of symptoms.

Hernia in Foramen Magnum - High BP, Bradycardia, Dilated pupils

Sodium chloride (High) in CSF
150cc normal in adults.
25cc/hour normal or 500cc/day

Explanation Apneustic respirations, a pattern of irregular and

disorganized breathing, is typically seen with lesions of the lower
pons.
Central neurogenic hyperventilation can be seen with lesions of the
upper pons and lower midbrain, but has also been seen with lesions
of the hemispheres.
Cheyne-Stokes respirations signify dysfunction of the diencephalon
or hemispheres bilaterally.
Loss of automatic respirations (Ondine's Curse) results from lesions
of the medulla.
Rapid, shallow respirations can be seen with intoxications.

Seizure

Explanation Seizures are classified accordig to their effect on

consciousness and whether the abnormal movements affect the
entire body or only a part.
A simple seizure does not affect consciousness complex seizure
impairs consciousness.
Partial seizures produce shaking only in part of the body;
Generalized seizures affect the entire body. Thus, a partial simple
seizure will produce shaking only in a limited part of the body and
will not impair consciousness.

Arteritis - high ESR

The most characteristic signs and symptoms of cervical myelopathy

are a painful, stiff neck, arm pain, and spastic leg weakness with
ataxia.

Guillain-Barré syndrome causes absent muscle stretch reflexes;

meningitis produces headache and encephalopathy. high proteins in
CSF

'Whiplash' (or cervical) strain causes musculoskeletal neck pain, and

cerebellar atrophy would not produce any of the above findings,
except ataxia.
An internuclear ophthalmoplegia consists of paralysis of adduction
of one eye with nystagmus on abduction of the other eye.
The neurological lesion is located in the medial longitudinal
fasciculus on the side ipsilateral to the eye with adduction
paralysis.
When this occurs bilaterally, the most common cause is MULTIPLE
SCLEROSIS. Tumor is a much less common cause of bilateral INO.
INOs caused by strokes are generally unilateral, since the vascular
lesion does not tend to cross the midline.
Myasthenia gravis can cause disorders of ocular motility that can
appear to mimic an INO. Trauma does not generally cause an INO.

A fracture of the temporal bone can cause a laceration of the middle

meningeal artery, leading to an epidural hemorrhage.
Laceration of a bridging vein is often the cause of subdural
hematoma, especially in the elderly.
Aneurysm rupture will produce subarachnoid hemorrhage.

Bulbar palsy is a lower motor neuron palsy that affects the nuclei of
the IXth, Xth, XIth, and XIIth cranial nerves.
Pseudobulbar palsy is an upper motor neuron palsy that affects
the corticobulbar tracts of the Vth, VIIth, IXth, Xth, XIth,
and XIIth cranial nerves.

Charcot-Marie-Tooth disease is a hereditary, usually autosomal

dominant, sensorimotor neuropathy, usually with onset in
adolescence or early adulthood. The disease causes chronic
degeneration of the peripheral nerves. Distal muscle atrophy initially
appears in the feet and lower legs and later in the hands. Classic
findings include high-arched feet, bilateral foot drop, 'stork' or
'inverted champagne bottle' legs, and clawed hands.
Myotonic dystrophy produces bitemporal wasting, bilateral ptosis,
and prolonged involuntary muscle contraction after activation, (i.e.,
myotonia).
Spinal muscular atrophy causes a progressive deterioration of
the motor neurons of the spinal cord and/or the brainstem; sensory
nerves are not involved.
Syringomyelia causes weakness, atrophy, and areflexia of the
hands and arms; decreased pain and temperature sensation (but
preserved light touch) in the neck, shoulders, and arms; as well as
spasticity, weakness, and ataxia of the lower extremities.
Myasthenia gravis produces muscle weakness, which is worsened
with use, and it typically affects extraocular or proximal muscles
first.
Pituitary apoplexy, a medical emergency caused by
hemorrhage in the pituitary gland. Immediate treatment with
high-dose corticosteroids and surgical decompression is necessary.

Botulism is the clinical syndrome caused by the toxin

of Clostridium botulinum, which prevents neuromuscular
transmission by blocking release of acetylcholine at the
neuromuscular junction.
Lambert-Eaton syndrome also causes a presynaptic defect of
neuromuscular transmission, but repeated activation of the muscle
will cause a progressive increase of muscle strength. Bulbar and
extraocular muscles are usually spared.
Myasthenia gravis has a post-synaptic defect of neuromuscular
transmission and usually also spares the pupils.
Guillain-Barré syndrome will have elevated protein in the CSF.
Muscle fasciculations, which are not present with botulism, are
prominent in poliomyelitis.

Mononeuropathy (or mononeuropathy multiplex) affects both

motor and sensory nerves, generally 1 nerve at time. The onset is
rapid, with wasting and tenderness of the involved muscles. There
may or may not be sensory loss. On testing, there is perfect
anatomic localization noted. In the lower extremities, the femoral,
obturator, and sciatic nerves are most often involved. In the upper
extremities, the median and ulnar are most often involved. This is
one of a group of diabetic neuropathies.
Carpal tunnel syndrome is an overuse syndrome that presents
with paresthesias and tingling in the 1st, 2nd, 3rd, and 1/2 of the
4th digit. This is due to median nerve entrapment. Muscle wasting is
a late finding in the thenar area.
Peripheral symmetric neuropathy is a slowly progressive
primary sensory disorder manifesting bilaterally with anesthesia,
hyperesthesia, or pain. It affects both upper and lower extremities,
although lower and more distal sections of the nerve are more
intensely involved.
Volkmann's ischemic paralysis is due to injury to the nutrient
vessels of a peripheral nerve secondary to a compartment
syndrome in the upper or lower extremities. A tight-fitting cast can
iatrogenically induce the same injury.
Diabetic amyotrophy has an abrupt onset of proximal muscle pain
with subsequent wasting. Pelvic girdle muscles are most frequently
involved, but upper extremity involvement is also reported.

Juvenile myoclonic epilepsy commonly begins between the ages

of 12 and 18 years. It is characterized by myoclonic attacks, most
often involving the upper extremities. They usually occur in the
morning upon awakening, with about 1/3 of these associated with
absence seizures. A majority of patients also experience generalized
tonic-clonic seizures at some point. While most patients can achieve
freedom from seizures on antiepileptics, the condition is life-long,
requiring continuous therapy. Valproic acid is the initial treatment of
choice. Intellectual prognosis is favorable.
West syndrome, which is also called infantile spasms, is a disorder
that develops during the 1st year of life, most often between the
ages of 4 and 8 months. Seizures often occur in clusters, and they
are characterized by flexion/extension myoclonus and head drops.
This is also known as Salaam attacks. EEG's demonstrate
hypsarrhythmia. Intellectual disability is common, often due to the
underlying disorder (structural abnormality, metabolic disorder,
infection, etc.). Seizure control can be attained if detected early
enough, although more than half will have seizures later in life.
ACTH is generally considered the preferred treatment, although
newer antiepileptics such as zonisamide appear to be promising.
Lennox-Gastaut syndrome is a severe form of epilepsy with an
onset between the ages of 1 and 7 years. About 25% of these
children have a history of infantile spasms, and many of the same
underlying diseases are associated with both syndromes. Slow
spike-wave discharges are seen on EEG. Seizures are usually tonic-
clonic or atypical absence, and status epilepticus is common.
Intellectual disability is the norm. Treatment is extremely difficult,
often requiring poly-therapy. The newer drugs lamotrigine and
topiramate have shown good results in early studies. Felbamate is
also effective, but it has been relegated to refractory cases due to
the risk of aplastic anemia and liver failure.
Progressive myoclonic epilepsy is caused by a number of
disorders including subacute sclerosing panencephalitis (most
common), Lafora disease, Unverricht-Lundborg disease, sialidosis,
and mitochondrial encephalopathy. These are degenerative
conditions, which usually begin between the ages of 6 and 15 years.
Unlike JME, they result in progressive intellectual decline often
accompanied by ataxia. Aside from myoclonic attacks, seizures are
generally tonic-clonic in nature. Treatment with valproic acid is
usually only partially effective, although newer antiepileptics show
some promise.
Benign rolandic epilepsy has an age of onset between 4 to 12
years and has a male predominance. Seizures are brief, usually
nocturnal, and involve facial muscles; they often progress to
generalization. They are well controlled with medication (when
needed), and they are usually outgrown by 16 years of age.
Childhood absence epilepsy generally begins between 3 and 8
years of age. Seizures begin abruptly and last less than 30 seconds.
Clinical manifestations of these generalized seizures include staring,
usually with the cessation of all motor activity (and often involving
automatisms), followed by resumption of normal activity. The child
is unaware that the seizure has occurred, and the events are not
followed by postictal confusion. Ethosuximide or valproic acid is the
treatment of choice, and the disorder often resolves spontaneously.

A nerve root lesion at S1 includes paresis of Mm. peronei, triceps

surae, and gluteus maximus, with diminished Achilles tendon reflex.
There is also pain radiating to the back of the thigh and the back of
the calf and lateral foot, behind malleolus, exacerbated with the
Lasegue test (straight leg raise).
A nerve root lesion at L2 does not have any reflex loss. It has a
deficit in hip flexion and adduction of thigh. The pain radiates across
the thigh.
A nerve root lesion at L3 has a loss in the adductor reflex. Knee
extension and adduction of the thigh are deficient. The pain radiates
across the thigh.
A nerve root lesion at L4 has a loss in the knee jerk. Inversion and
dorsiflexion of the foot are deficient, with inability to stand on the
heel. The pain radiates to medial leg.
A nerve root lesion at L5 does not have any reflex loss.j It has a
deficit in dorsiflexion of toes and foot, with inability to stand on the
heel. The pain radiates to the dorsum of the foot.

A headache that lasts for 4 months, combined with visual

disturbances, vertigo, nausea, and vomiting, is highly suggestive of
a brain tumor. The finding of the gaze nystagmus points to a
central vestibularis lesion on the right side. Considering the vertigo,
ataxia, and hypoacusis, it is very likely an acoustic neuroma, a
benign tumor of the vestibulocochlear nerve.

Carpal tunnel syndrome is a chronic repetitive strain injury

(CRSI). The median nerve is impinged at the wrist, resulting in
tingling and numbness, which often awakens the person from sleep.
Lateral epicondylitis is known as tennis elbow and is also a CRSI
(chronic repetitive strain injury). Tendonitis occurs at insertions of
the forearm extensor muscles at the lateral epicondyle of the elbow.
Medial epicondylitis is also a CRSI and known as golfer's elbow. It
is due to tendonitis at insertions of the wrist flexors and pronators at
medial epicondyle of the elbow giving varying degrees of
paresthesias in hands and fingers.
Thoracic outlet syndrome is due to compression of the
neurovascular bundle of the upper limb in the upper thoracic region.
There is tingling, aching, and burning in the distribution of the ulnar
nerve. This also may be due to CRSI.
Phantom pain (phantom limb) is an entity in which patients feel
pain at the site of an amputated limb.

Typical features of Reyes syndrome include mental status change,

lethargy, vomiting, often in the context of a recent illness. An
abnormal liver profile with elevated aminotransferase levels,
prolonged prothrombin time, and high serum ammonia level will be
found early in the process. The cause of Reye syndrome is
unknown, but it often occurs following a viral illness such as
influenza, herpes, or varicella. It has classically been associated
with the use of aspirin, but aspirin use is not involved in all cases.
The disease most commonly affects children between the ages of 2
and 16 years.
The mortality is high – between 10 and 40%. Early supportive care,
including administration of vitamin K, monitoring of intracranial
pressure (and treatment if elevated), and avoidance of
hypoglycemia, can be life-saving.
After an apparent viral illness, pernicious vomiting develops.
Subsequently, mental status changes occur- confusion and
agitation, then stupor and coma. Infants may have seizures or
apneic episodes. Prognosis is most related to the duration of
disordered cerebral function during the acute stage. The overall
fatality rate is approximately 20%. An ammonia level greater than
300 microgram/dl implies a poor prognosis. The basic defect lies in
energy metabolism on a cellular level and electron micrographs
demonstrate a derangement of mitochondria.

Neurofibromatosis is caused by autosomal dominant mutations. The

condition has no cure. There are 2 major varieties: NF-1 (incidence
1/3000) and NF-2 (1/25,000). This disease is characterized by
tumors forming on nerve fibers. In addition, some patients have
hamartomas of the iris (Lisch nodules).The irregularly shaped, 2 X 3
cm, hyperpigmented lesions on his skin are café au lait spots. He
has freckles in his axilla. The cutaneous and subcutaneous masses
are neurofibromas. They frequently do not develop until late in
childhood or adolescence. This patient has also developed a glioma
on the optic nerve, resulting in visual disturbances.
Tuberous sclerosis is manifest by adenoma sebaceum, small bright
red or brownish nodules in a butterfly distribution over the nose and
cheeks. These children have CNS lesions leading to seizures and
intellectual disability. Large, often unilateral cafe au lait spots
characterize McCune-Albright syndrome. There are associated bony
and endocrine disorders.
Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is
characterized by a congenital capillary hemangioma on the skin,
often in the trigeminal distribution on the face, and meningeal
hemangioma that may cause seizures.
Spider angiomas consist of a central artery with enlarged vessels
radiating outwards and a surrounding erythematous flush.

The patient suffers from hydrocephalus as a result of congenital

aqueductal stenosis. The cerebral aqueduct of Sylvius is a narrow
conduit that permits the flow of cerebrospinal fluid from the third to
the fourth ventricle. Congenital narrowing of this passage often
results in asymptomatic compensation of CSF flow. Occasionally, an
unrecognized trauma or infectious process overwhelms the fragile
balance of CSF flow, and non-communicating hydrocephalus occurs.
Progressive headache and loss of peripheral vision raise the concern
for hydrocephalus. Treatment options commonly include CSF
diversion procedures and endoscopic third ventriculostomy.

Ruptured saccular aneurysm onset is typically described as

"thunder-clap headache", which is the sudden onset of the worst
headache of ones life. This does not fit with the patient's history of
ongoing and escalating headache. Ruptured aneurysm can also
result in hydrocephalus as a result of impaired absorption of CSF
through the arachnoid villi. However, this patient does not have any
subarachnoid or intraventricular blood products to indicate a
ruptured aneurysm.

Carotid artery dissection can lead to headache and further visual

changes secondary to ischemic stroke. In the absence of trauma,
dissection occurs most frequently with connective tissue disorders.
Given his longstanding history of headache and CT scan
demonstrating hydrocephalus, it is unlikely that he suffers from a
carotid artery dissection.
Lead poisoning can affect the nervous system, resulting in
encephalopathy and neuropathy. However, there is no association
between hydrocephalus and lead poisoning.

Retinal artery occlusion results in painless monocular vision loss.

Visual loss is usually central and dense. In this case, the patient has
less distinct peripheral vision loss.

Ischemic stroke of the occipital lobe should certainly be

considered with the symptoms of headache and visual loss.
However, CT findings of stroke include hypodensity in the affected
vascular territory, which is not seen in this imaging scan.

Tension headaches, the most common type of headache, present as

rather diffuse/generalized head pain, without the classic
characteristics of the other types of headaches. A component of
psychological stress is often present.
Cluster headaches are a severe type of headache, most often
occurring in middle-aged men, in which the severe headache is
accompanied by several other symptoms, such as lacrimation, nasal
rhinorrhea, and congestion. The pain is usually localized in the
periorbital region.
About 1/3 of patients with a brain tumor will present with a
headache, but tension-type headaches are far more common in the
general population. Headaches secondary to brain tumor can vary in
character and intensity. New onset headaches later in life, especially
those without a coinciding significant psychosocial stressor, are of
greater concern; they have a larger possibility of being caused by
an intracranial mass. Nausea and vomiting or neurologic symptoms
may accompany headaches caused by a brain tumor.
Migraine headaches are also quite common. They are classically
described as throbbing in nature and are accompanied by nausea
(and possibly vomiting), photophobia (sensitivity to light), and
phonophobia (sensitivity to sounds); visual changes and aura are
also possible. Migraines are more common in women than men, but
they can occur in men. They are usually a chronic condition,
beginning in adolescence or the early adult years. Stress can be
among many triggers for migraines. This patient's pain pattern and
age of onset are inconsistent with migraine, and he lacks the
associated symptoms.
Subarachnoid hemorrhage (SAH) is classically described by
patients as an acute headache that is the "worst headache of my
life." There may be preceding trauma and associated nausea and
vomiting; nuchal rigidity (due to meningeal irritation) and mental
status changes are also seen. This patient's headache history is
inconsistent with SAH.

This patient most likely has complex regional pain

syndrome (CRPS). CRPS is indeed a complex syndrome often
occurring in a limb; patients exhibit vasomotor and neurologic
symptoms out of proportion with objective findings. It often occurs
following some type of event, such as a surgery (note the patient’s
recent ipsilateral knee surgery), fracture, stroke, or myocardial
infarction. Most of the time, there is no nerve lesion. CRPS is
classified into 3 stages, and symptoms can vary depending on the
stage. It is characterized by persistent burning and/or throbbing
pain in an extremity. Swelling, redness, and increased hair/nail
growth occur in stage I; it may progress to cool, pale skin and even
osteoporosis in the latter 2 stages. Allodynia, the phenomenon in
which a normally painless stimulus produces significant pain
response, is a common finding. No single exam finding or test is
diagnostic of CRPS, which is a clinical diagnosis.