Educational Objective:

Damage to the lateral spinothalamic tracts causes contralateral loss of pain and temperature sensation
beginning two levels below the level of the lesion.
(remember thatthe spinothalamic tracts cross very early on in the spinal cord).
Educational Objective:

Craniopharyngiomas are benign suprasellar tumors which usually present with signs of
hypopituitarism.
headaches and bitemporal blindness.
Craniopharyngiomas are benign tumors arising from Rathke's pouch. and are more common
in children.
Although they are infrequently seen in adults. craniopharyngiomas actually have a bimodal
age distribution children and 55-65 years age group.
whereas sexual dysfunction is more prominent in adults. Women can present with
amenorrhea.
The diagnosis is made with an MRI or CT scan. and treatment is
with surgery and/or radiotherapy.
Educational objective:

Hypertension is a common cause of spontaneous intracranial hemorrhage with the most

common sites being
the basal ganglia. cerebellum. thalamus. and pons.
Educational objective:
Hypothyroidism is an important cause of reversible changes in memory and mentation. It will be
accompanied by systemic changes such as weight gain. fatigue. and constipation.
Educational Objective:

Oligoclonal bands are present in 85-90% of cases of multiple sclerosis. CSF pressure. protein
and cell count
are grossly normal.
Educational Objective:
Paroxysmal, lightning-like pain on the face is usually due to trigeminal neuralgia.
Carbamazepine is the drug
of choice.
severe. intense. burning or
electric shock-like. This symptom occurs in paroxysms that last a few seconds to minutes
each. but occurs
many times a day. The trigger factors may vary, and the cause remains unknown.
Carbamazepine is the
drug of choice.
Educational Objective:
Subdural hematomas are serious intracranial hemorrhages that occur due to tearing of bridging veins.
Blunt
trauma is the most common cause. and surgical evacuation is usually required.
On non-contrast CT of the head. SOH appears as a white crescent. A midline shift may also be seen.
The
CT image above shows a large right-sided subdural hematoma producing a significant mass effect and
midline shift. The ventricles on the right side are completely obliterated. Emergent neurosurgical
consultation
for hematoma evacuation is necessary.
SOHs are more common in older patients and alcoholics due to brain atrophy and
vessel fragility.

Educational objective:
Hemi-sensory loss with severe dysesthesia of the affected area is typical for a thalamic stroke
r thalamic stroke (Dejerine-Roussy syndrome). This condition is
caused by a stroke involving ventral postero-lateral (VPL) nucleus of the thalamus. which transmits
sensory
information from the contralateral side of the body. The classic presentation involves contralateral
hemianesthesia that can be accompanied by transient hemiparesis. athetosis. or ballistic movements.
Dysesthesia of the area affected by the sensory loss is characteristic. and is called thalamic pain
phenomenon.
Educational objective:
Cerebral emboli are one of the most dreaded consequences of endocarditis. They occur when pieces of
infected valvular vegetations break off and enter the CNS circulation. Treatment is with antibiotics.
Although IV drug abusers can get right-sided endocarditis, left-sided endocarditis is also very
common! The next steps in the management of this patient are initiation of broad-spectrum antibiotics
and
echocardiogram to evaluate for valvular vegetations.
Educational objective:
Essential tremor is characterized by a tremor that is suppressed at rest and exacerbated toward the end
of a
goal-directed movement. Affected patients typically have difficulty holding a newspaper, writing
legibly,
drinking a cup of coffee, or feeding themselves.
ducational objective:
First line treatment for essential tremor is propranolol, especially if the patient is also hypertensive.
Alternative
medications include primidone ortopiramate.
Educational Objective:
Vertigo is a sensation of excessive motion compared to physical reality. It is most commonly due to
dysfunction within the vestibular system.
Vertigo resulting from vestibular dysfunction tends to be of sudden onset,
interfere with walking, and cause nausea and vomiting. Typical causes of vestibulopathy include
Meniere's
disease, perilymphatic fistulas, benign positional vertigo, labyrinthitis, and acoustic neuromas.
Educational objective:
Guillain-Barre syndrome is characterized by ascending paralysis. antecedent history of infection. and
CSF
finding of albumino-cytologic dissociation. The treatment includes IV immunoglobulin and
plasmapheresis.
Cranial nerve paralysis (most likely facial nerve palsy) often develops.
The classic presentation includes ascending
paralysis. areflexia and sensory changes 3-4 weeks after an upper respiratory tract infection or
gastroenteritis.
Educational Objective:

Primidone is an anticonvulsant agent which can be used to treat benign essential tremors. Its
administration
can precipitate acute intermittent porphyria. which can be diagnosed by checking for urine
porphobilinogen.
The administration of primidone may precipitate acute intermittent
porphyria. which manifests as abdominal pain. neurologic and psychiatric abnormalities (as
seen in this
patient).
Educational objective:

Restless leg syndrome is a potentially debilitating condition characterized by the

uncomfortable desire to
move ones legs at rest or at night. It can be treated with dopamine agonists.
RLS is more common in
middle aged and older patients. as well as in those with chronic kidney disease and/or iron
deficiency
anemia.
Educational Objective:

It is important to know the function of each cranial nerve. The ophthalmic branch (V1) of the
trigeminal nerve
(V) controls corneal sensation. When it is damaged. patients can suffer from corneal injury
without
awareness.
he ophthalmic nerve (V1). carries sensory fibers to the scalp.
forehead. upper eyelid. conjunctiva. cornea. nose. and frontal sinuses. Damage to V1 results
in corneal
anesthesia; thus. a lesion on the eye may not be sensed if a V1 lesion is present.
Educational Objective:

Rupture of a saccular aneurysm is the most frequent cause of non-traumatic subarachnoid

hemorrhage
(SAH). Know the appearance of SAH on CT.
Aneurysms are more likely to rupture when they are larger than 7 mm, and are most
frequent in the anterior circulation on the circle of Willis.
Educational Objective:

Patients with Huntington's chorea usually present in their forties or fifties with chorea and/or
behavioral
disturbance. Atrophy of the caudate nucleus is a characteristic feature.
Chorea
is characterized by sudden. jerky and irregular movements of the extremities.
Memory impairment is a late findin
Educational objective:

Tick-borne paralysis is characterized by rapidly progressive ascending paralysis. absence of

fever. absence
of sensory abnormalities and normal CSF examination.
Meticulous search and removal of the tick usually result in improvement within an hour and
complete recovery
after several days.
Educational objective:
Fronto-temporal dementia is characterized by personality changes, compulsive behaviors, and
impaired
memory.
Dementia with Lewy bodies (DLB) is characterized by fluctuating cognitive impairment. Bizarre,
visual hallucinations may occur. Parkinsonism may also occur, but there is a poor response to
dopaminergic
agonist therapy.
Alzheimer's disease is a progressive dementia. Its associated risk factors are: age, female
gender, positive family history, head trauma, and Down's syndrome. The typical first symptoms are
subtle
memory loss, language difficulties and apraxia, followed by impaired judgment and personality
changes. The

treatment is specifically targeted towards the particular symptoms, but generally includes psychosocial
intervention and pharmacotherapy with donepezil or tacrine.
Educational objective:
NPH is thought to result from decreased CSF absorption or transient increases in intracranial pressure
that
cause permanent ventricular enlargement without chronically increasing intracranial pressure. It is
characterized clinically by dementia. gait disturbances. and incontinence.
The initial increase in ventricular size may be due to either diminished CSF absorption at the
arachnoid villi or obstructive hydrocephalus.
Educational objective:

It is important to be able to differentiate between the different types of dementia. Dementia

with Lewy bodies
causes alterations in alertness. visual hallucinations. and extrapyramidal symptoms.
Lewy bodies are a pathologic finding also present in Parkinsons disease. The key distinction
between these
two conditions is the early appearance of dementia in Lewy body disease and of motor
symptoms in
Parkinsons disease.
Educational objective:
Pseudotumor cerebri or idiopathic intracranial hypertension (IIH) can present as an acute.
subacute. or
chronic headache with associated blurry vision or visual loss. papilledema. pulsatile tinnitus.
abducens nerve
palsy. nausea. and vomiting. Patients with IIH are typically young obese women. Certain
medications.
including vitamin A and oral contraceptive pills. can also lead to IIH. Brain MRI can show an
empty sella and
slit-like ventricles. Because IIH is a diagnosis of exclusion. head magnetic resonance
venography is required
to exclude cerebral venous sinus thrombosis as the cause of elevated opening pressure.
Papilledema is not a
contraindication to an LP unless the patient has evidence of obstructive or noncommunicating
hydrocephalus
and/or a space-occupying lesion with/without mass effect or midline shift. Pseudotumor
cerebri causes a
communicating hydrocephalus (i.e.. pressures in the ventricular and subarachnoid spaces are
equilibrated
with the lumbar cistern); therefore. an LP is considered safe.
The following criteria are used for the diagnosis of IIH:
1 . Presence of features of increased intracranial pressure in an alert patient
2. Absence of focal neurologic signs except for sixth nerve palsy
3. Normal CSF examination except for increased CSF opening pressure
4. Absence of any ventricular abnormality. other than reduction in ventricular size (slit-like
ventricles). and
other causes of increased intracranial pressure
Educational objective:

HIV and hepatitis C infection should be suspected in patients with weight loss and history of

intravenous drug
abuse. Patients with HIV are at risk of developing depression and dementia.
It would be reasonable to perform a CT or MRI of the head if this patient's serologic work-up
is
negative or if he develops focal neurologic deficits.
Educational objective:
Lacunar strokes are due to microatheroma and lipohyalinosis in small penetrating arteries of the brain.
They
often affect the internal capsule and result in pure motor dysfunction. Lacunes comprise 25% of
ischemic
strokes. Hypertension and diabetes are two major risk factors.
Educational Objective:

Several well-designed clinical trials have proven that interieron-beta decreases the frequency
of relapse. and
reduces disability in patients with the relapsing-remitting form of MS.
Educational Objective:

Cauda equina syndrome is a serious neurologic disorder characterized by saddle anesthesia.

bowel and
bladder dysfunction. low back pain. and a variety of lower extremity findings. The cauda
equina consists of
spinal nerves before they exit the spinal canal. The etiology of CES must be identified rapidly
in order to
preserve neurologic function.
The cauda equina is particularly sensitive to damage because the
nerves have a poorly developed epineurium.
An emergent MRI of the spine must be obtained when entertaining a diagnosis of
CES. In this patient. he likely has CES due to prostate cancer or a reaction to radiation
therapy.
Educational objective:

Normal pressure hydrocephalus is characterized by the triad of gait disturbance. dementia

and urinary
incontinence. Lumbar puncture reveals the normal CSF pressures. and MRI shows the
enlarged ventricles.
Educational objective:
Steroid-induced myopathy is a well-described result of chronic corticosteroid use
characterized by painless
proximal muscle weakness. It will slowly improve once the offending medication is
discontinued.
The
mechanism of steroid myopathy is thought to be due to decreased protein synthesis.
mitochondrial
alterations. increased protein degradation. and electrolyte and carbohydrate metabolism
disturbances.
For both acute and chronic forms. the diagnosis is clinical. with no definitive diagnostic
test. Muscle power improves after discontinuation of the steroids. butthe improvement can
take weeks to
months.
Educational objective:
In heat stroke the temperature is usually above 40.5 C (105 F). Heat stroke results from an insufficient

evaporative cooling mechanism. Therefore. treatment involves induction of evaporative cooling to

reverse
hyperthermia.
Other signs and symptoms of heat stroke may
include altered mental status. headache. dizziness. dry skin. hypotension. tachycardia and tachypnea.
Hyperthermia may also induce systemic damage including rhabdomyolysis. acute renal failure. and
DIC.
Educational objective:
If a patient presents within 4.5 hours after the onset of an ischemic stroke. thrombolytic therapy with
tPA (after
CT scan) should be started. Many trials have demonstrated improved neurological outcomes with this
approach.
The use of streptokinase in stroke patients has not shown any benefits. The drug actually
increases the risk of bleeding. The FDA currently recommends only tPA in the management of stroke
patients.

tRIGENMIANL Aplastic anemia can occur with prolonged use; therefore. routine CBC is
included in the follow-up
management of such patients. When medication fails to control the pain. surgical gangliolysis
or suboccipital
craniectomy for decompression of the trigeminal nerve are options.
Educational Objective:

Subdural hematoma results from the rupture of bridging veins. Epidural hematoma results
from the rupture of
the middle meningeal artery. Recognize the characteristic appearances of these hematomas on
CT.
The condition can also occur secondarily to minor trauma if the patient is on
anticoagulation therapy. especially a combination of aspirin and Coumadin (warfarin).
Bleeding occurs due to
the tearing of the bridging veins between the cortex and venous sinuses
SOH is more common in the elderly and alcoholics.
Educational Objective:
MRI is the test of choice to support the clinical diagnosis of multiple sclerosis.
The typical locations of the plaques are the
periventricular regions. corpus callosum. deep white matter and basal ganglia.
fundoscopy is normal.
central visual field defect
Educational Objective:
Suspect pseudodementia in elderly patients with dementia in the setting of severe depression.
Antidepressants are the treatment of choice.
Pseudodementia is reversible with the treatment of depression.
using antidepressant medications such as SSRis
A
good distinguishing factor to remember is that patients with pseudodementia seem significantly
concerned
abouttheir impaired memory; whereas. patients with Alzheimer's dementia seem relatively
unconcerned.

ducational Objective:
Expressive aphasia results from the lesions in dominant frontal lobe. Frontallobe also contains motor
cortex
whose damage results in contralateral paresis.
Sensory cortex is present in the parietal lobe and damage to sensory cortex results in contralateral
hemianesthesia. Damage to dominant parietal lobe also results in dysgraphia. dyscalculia and right/left
confusion. Damage to nondominant parietal lobe causes construction apraxia. contralateral sensory
neglect
and anosognosia.
Posterior part of dominant frontal lobe contains motor speech area of Broca
and damage to this area results in expressive aphasia in which patient can't articulate speech or write
normally but can comprehend written and spoken language.

Educational Objective:
Suspect Huntington's disease in a patient with mood disturbances, dementia, chorea and a family
history of
similar symptoms.
ducational objective:

The classic symptom triad of normal pressure hydrocephalus (NPH) is abnormal gait.
incontinence. and
dementia. NPH is treated with large volume lumbar punctures and. if successful.
ventriculoperitoneal
shunting.
Educational Objective:
Hypokalemia is a common electrolyte abnormality that causes weakness. fatigue. and muscle cramps.
When severe. it can lead to paralysis and arrhythmia. The ECG may show U waves. flat and broad T
waves.
and premature ventricular beats.
Stroke does not cause muscle pain and usually has focal findings. The presentation for an
ischemic stroke will depend on the location of the lesion.

CN Ill (oculomotor nerve) has the following functions:

1. Its somatic component innervates the inferior. superior. and medial rectus. inferior oblique and
levator
palpebrae muscles. Ptosis occurs due to paralysis of the levator palpebrae; whereas. the unopposed
action of the lateral rectus (CN VI) and superior oblique (CN IV) muscles lead to a "down and out"
gaze.
2. Parasympathetic fibers of CN Ill innervate the sphincter of the iris and the ciliary muscle. Paralysis
of
parasympathetic fibers causes a fixed. dilated pupil and causes an inability of the lens to change

shape (loss of accommodation).

Educational Objective:
Diabetic mononeuropathy often involves CN Ill. Nerve damage is ischemic. and only somatic nerve
fibers are
affected. Parasympathetic fibers of CN Ill retain function. Ptosis and a "down and out" gaze in
conjunction
with normal light and accommodation reflexes indicate diabetic CN Ill neuropathy.
Educational Objective:

The absence of forehead furrows indicates peripheral facial nerve palsy and excludes central
causes.
Educational objective:
Normal age-related cognitive changes include tiredness. occasional forgetfulness. occasional word
finding
difficulty. and trouble falling asleep. Dementia cannot be diagnosed unless there are functional
impairments.
Educational Objective:
Pregnancy testing should be performed in women of child-bearing age (high risk of being pregnant)
before starting treatment with sumatriptan.
The contraindications of triptans are as follows:
1 . familial hemiplegic migraine
2. uncontrolled hypertension
3. coronary artery disease
4. Prinzmetal angina
5. pregnancy
6. ischemic stroke
7. basilar migraine
Educational Objective:
Hemi-neglect syndrome is characterized by ignoring the left side of a space. and involves the right
(non-dominant) parietal lobe.
Educational Objective:
The typical CT/MRI findings in high-grade astrocytoma are heterogenous and serpiginous contrast
enhancement. Recognize the classic butterfly appearance of GBM.
Patients with brain metastasis usually have a duration of symptoms of less than 2 months. The
site of metastasis is the gray-white junction or watershed zones.
Educational objective:
Alzheimer's dementia is the most common type in the United States and is characterized by early
memory
and visuospatial impairments. Gait impairment is the most prominent clinical features of NPH and
appears
early in its disease course.
Subcortical and frontal features dominate the cognitive disturbance of NPH. Cortical features (e.g.
aphasia. agnosia) are less common.
Educational Objective:
Acute vertigo (sensation of spinning of head) is often due to dysfunction of the labyrinth. Patients with
BPPV
have vertigo related to head positioning. Global cerebral ischemia results in presyncope
(lightheadedness
with visual blurring. not spinning) and syncope.
Educational Objective:

Riluzole is a glutamate inhibitor that is currently approved for use in patients with
amyotrophic lateral sclerosis.

Its side effects are dizziness, nausea, weight loss, elevated liver enzymes and skeletal
weakness.
Educational Objective:
GBS can lead to respiratory muscle weakness and may occasionally result in respiratory failure. The
best
way to monitor respiratory function in such cases is through serial measurements of bedside vital
capacity.
The risk for ventilatory failure
increases significantly when the VC falls below 15 mUkg. especially if there has been a clear
downward
trend.
Educational Objective:
Guillain-Barre syndrome is an acute or subacute ascending paralysis. CSF analysis reveals an elevated
protein level with normal glucose levels, WBC, and RBC counts.
Elevated WBC and RBC counts with normal protein and glucose levels can be seen with herpes
encephalitis.
Educational objective:

Because the facial/ophthalmic venous system is valveless. uncontrolled infection of the skin
can result in
cavernous sinus thrombosis. Red-flag symptoms include severe headache; bilateral
periorbital edema; and
cranial nerve Ill, IV, V, and VI deficits.
Treatment includes
broad-spectrum intravenous antibiotics and prevention or reversal of cerebral herniation.
As a result, unilateral symptoms (eg, headache, binocular palsies, periorbital edema,
hypoesthesia, or
hyperesthesia in V1N2 distribution) can rapidly become bilateral.
Educational objective:

Pronator drift is a relatively sensitive and specific physical exam finding for upper motor
nerve damage
affecting the upper extremities. In particular, many patients with strokes will demonstrate
pronator drift.
Educational objective:

Amaurosis fugax is the painless loss of vision from emboli, and is a warning sign for an
impending stroke.
Most emboli occur from the carotid bifurcation; hence, a duplex ultrasound of the neck
should be performed.
Cholesterol particles (Hollenhorst bodies)
may be seen in the eye.
Educational Objective:
Suspect syringomyelia in a patient with areflexic weakness in the upper extremities and dissociated
anesthesia in a "cape" distribution. The presence of a cord cavity is the most characteristic feature.
Caudal
displacement of the fourth ventricle or cerebellar tonsils, and focal cord enlargement may occur.
dissociated anesthesia (loss of pain and temperature with preserved position and vibration)
Educational objective:

A transient ischemic attack (TIA) is defined by focal neurologic symptoms lasting less than
24 hours due to
cerebral ischemia. A head CT should always be obtained in patients with stroke symptoms to
distinguish

ischemic from hemorrhagic etiologies. In patients with strokes or TIAs due to ischemia, if a
thrombotic cause
is suspected, an antiplatelet agent such as aspirin is used to prevent future events.
Medications such as
warfarin are used to prevent future ischemic events due to cardiogenic emboli.
CONTROLLING HYPERTENTION IS THE SINGLE MOST IMPORTANT
FACTOR IN PREVENTING STROKE.

Educational objective:

The three cardinal signs of Parkinson disease are resttremor, rigidity, and bradykinesia. The
presence of at
least two of these signs on physical examination is grounds for a clinical diagnosis of this
disease.
Educational objective:
Always suspect lacunar stroke if a patient presents with a limited neurologic deficit. The typical
lacunar stroke
scenarios are pure motor stroke, pure sensory stroke (thalamus), ataxic-hemiparesis, and dysarthriaclumsy
hand syndrome. The principal cause of lacunar stroke is hypertension.
Educational objective:
An isolated, round, smooth-bordered, ring-enhancing intracranial lesion on contrast CT scan of the
brain in an
immunocompetent patient with a known extracranial bacterial infection is most likely a brain abscess.
Aerobic
and anaerobic streptococci and Bacteroides species (anaerobes) are the most common causative
organisms.
Educational objective:
The most common site of hypertensive hemorrhages is the putamen (35%). The internal capsule that
lies
adjacent to the putamen is almost always involved, thereby leading to hemiparesis.
The onset of symptoms is not abrupt as in subarachnoid hemorrhage or embolic stroke.
Educational Objective:
Amyotrophic lateral sclerosis (ALS) is characterized by the presence of both upper (spasticity, bulbar
symptoms, hyperreflexia) and lower (fasciculations) motor neuron lesions.
Educational Objective:
Herpes mainly affects the temporal lobe of the brain and may present acutely ( <1week

duration) with focal

neurological findings. The characteristic CSF findings are lymphocytic pleocytosis, increased
number of
erythrocytes, and elevated protein. HSV polymerase chain reaction analysis is the gold
standard.
Intravenous acyclovir is the treatment of choice.
Educational Objective:
Metoclopramide is a prokinetic agent used to treat nausea, vomiting, and gastroparesis. Patients taking
this
medication should be monitored closely for the development of drug-induced extrapyramidal
symptoms.

Treatment for a dystonic reaction involves discontinuation of the medication and

administration of benztropine or diphenhydramine.
Educational Objective:

Propranolol is the drug of choice for benign essential tremor.

An autosomal dominant pattern of transmission may be
present. The head and upper distal extremities (i.e., hands) are usually affected, while the legs
are usually
spared. Tremors become especially apparent in the upper extremities when the arms are
outstretched.
The
amplitude of the tremors typically increases at the very end of goal-directed activities (e.g.,
trying to reach an
object, drinking from a glass, finger-to-nose testing. Propranolol is the drug of choice.
Educational Objective:
Vasospasm is the major cause of morbidity and mortality in patients with subarachnoid hemorrhage
(SAH).
Calcium channel blockers (e.g., nimodipine) are used to prevent vasospasm in patients with SAH.
Vasospasm following SAH occur in approximately 30% of patients, and is the major cause
of morbidity and mortality in such patients.
Educational objective:

Migraine should be suspected in patients with a unilateral headache with a pulsatile quality,
particularly if it is
accompanied by vomiting and photophobia. Intravenous antiemetics (chlorpromazine,
prochlorperazine or
metoclopramide) can be used as monotherapy for acute migraine attacks, particularly in
patients with
accompanying nausea and vomiting.
Educational Objective:

Suspect progressive multifocalleukoencephalopathy in an HIV-infected patient with focal

neurological signs
and multiple non-enhancing lesions with no mass effect on the CT scan.
It is caused
by the JC virus, a human polyomavirus.
The most common
presenting symptoms are hemiparesis and disturbances in speech, vision and gait. Cranial
nerve deficits
may occasionally develop.
Educational Objective:

The most common site of ulnar nerve entrapment is the elbow where the ulnar nerve lies at
the medial
epicondylar groove.
Prolonged, inadvertent compression of the nerve by leaning on the elbows while working at a
desk or
table is the typical scenario
Educational Objective:
Acute angle closure glaucoma presents as unilateral eye pain, redness, and a dilated pupil
with poor light
response. It is important to distinguish acute angle closure glaucoma from migraine, cluster
headache,

temporal arteritis, and keratoconjunctivitis because failure to correctly diagnose this disease
can lead to
blindness.
The affected eye will
appear injected and the pupil will be dilated and poorly responsive to light.
It is characterized by the rapid onset of
severe pain and vision loss. Patients may complain of seeing halos around lights.
Educational objective:
Oral anticholinesterase is usually the initial treatment of choice for myasthenia gravis.
Plasmapheresis removes acetylcholine receptor antibodies from the circulation. It is used in
seriously ill patients when other treatments are not effective or contraindicated. Its effect is transient
and
cannot be used on a long-term basis. Other indications of plasmapheresis include (1) stabilization of
the
patient before thymectomy, and (2) myasthenia crisis.
Educational Objective:
Steppage gait is associated with foot drop. The most common causes of foot drop are neuropathy, L5
radiculopathy and traumatic damage to the common peroneal nerve.
Educational objective:
Muscular weakness in paraneoplastic syndromes can be due to a variety of processes at different
levels. A
good history and physical examination can help localize the process in most cases.
The
muscle involvement may be due to inflammation, necrosis, fiber atrophy, and antibody-mediated
damage.
Proximal muscles are typically affected, and muscle strength is diminished symmetrically. Reflexes
are
normal, and no sensation abnormality is present. Elevated CK levels and myopathic electromyography
help
to confirm the diagnosis.
Educational Objective:
Multiple observational studies have demonstrated that patients with hypertension have approximately
four
times the risk of stroke when compared to non-hypertensive subjects.
Educational Objective:

It is important to distinguish between the different patterns of muscle weakness. The

hallmark of myasthenia
gravis is decreasing muscular strength with continued contraction. While this can affect any
skeletal muscle, ptosis is for Myasthenia
P'xtr:::.nr111:::.r ~vmntnm~ (P n rlinlnni:::.) :::.rP mn~t rnmmnn

Educational Objective:
Nonsense and frameshift mutations are typically more severe than missense mutations. Silent (same
sense)
mutations do not affect the structure of the protein.
neurofibromatosis, type 2: subcutaneous neurofibromas,
hyperpigmented cafe-au-lait spots, deafness due to acoustic neuromas (typically bilateral), and a
family
history of the disease.
It is an autosomal dominant disorder
Educational Objective:
A lesion in the upper thoracic spinal cord results in paraplegia, bladder and rectal incontinence, and
absent

sensation from the nipple downwards.

Choice A) A lesion in the cerebellum causes posterior fossa symptoms (e.g., nausea, vomiting, ataxia).
(Choice B) A lesion in the posterior columns causes ataxia.
(Choice D) A lesion in the lower thoracic spinal cord causes absent sensation from the umbilicus
downwards.
(Choice E) A lesion located supratentorially produces partial or complete hemiparesis.
Educational objective:

Delirium is an acute confusional state due to organic illness which is commonly

superimposed upon dementia
in the elderly. Urinary tract sepsis and metabolic disturbances are common precipitants in
older patients.
The initial work-up of delirium should include urinalysis and measurement of serum
electrolytes.
. The most common cause in
hospitalized patients overall is substance abuse or withdrawal. Other potential causes include
medication
adverse effects, infection, metabolic/electrolyte derangements, vascular disease affecting
cerebral perfusion,
and psychiatric illness.
In the elderly, urinary tract infection, pneumonia and metabolic disturbances are all
common precipitating factors
Educational Objective:
1DO % oxygen (treatment of choice) is an effective and rapid method used to abort an acute attack of
cluster
headache.
The headache is always unilateral, sudden in onset, severe,
deep, excruciating, and continuous or explosive in quality. It generally starts as retro-orbital pain and
later
spreads to the hemicranium. It is not associated with nausea or vomiting, but is frequently associated
with
ipsilateral lacrimation, rhinorrhea, red eye, stuffy nose, pallor or even Horner's syndrome.
Educational Objective:

Elderly patients with depression often present with memory loss (pseudodementia). This can
mimic other
causes of dementia, including Alzheimer's dementia. CT findings are usually normal and noncontributory for
the differential diagnosis, whereas DST Dexamethose supprestion test may be of value.
Educational objective:
Creutzfeldt-Jakob disease is characterized by rapidly progressive dementia, myoclonus and sharp,
triphasic,
synchronous discharges on EEG. This spongiform encephalopathy is caused by a prion. Most patients
die
within one year of symptom onset.
Educational Objective:

Patients who develop serious bleeding (e.g., intracerebral hemorrhage) due to excess
anticoagulation with
warfarin should be given fresh frozen plasma (FFP) for the rapid reversal of anticoagulation.
Patients with serious intracranial bleeding (such as in this case) should have rapid correction
of excess

anticoagulation, with a target INR of less than 1.5. Fresh-frozen plasma (FFP) reverses the
action of warfarin,
works immediately, and lasts for a few hours; therefore, FFP should be administered
emergently. In addition,
emergency neurosurgical evaluation should be done for decompression of the increased
intracranial pressure.
Educational Objective:
The resolution of muscular weakness with rest is a hallmark feature of myasthenia gravis.
Patients typically present with
intermittent dysarthria, dysphagia, drooping eyelids (ptosis), and diplopia.
Educational objective:
Fibrinolytic therapy improves neurologic outcomes in patients with ischemic strokes when given
within 3 to
4.5 hours of symptom onset. Before administering tPA, a non-contrast head CT should be performed
to rule
out hemorrhagic stroke and the patient should be screened for other contraindications to therapy.
Blood pressure control is critical in patients undergoing fibrinolytic therapy, and should be kept
below 185/11 0 during in the 24 hours after it is administered. Labetalol, an alpha-1 and beta
adrenergic
blocker, may be used for this purpose. This particular patient's blood pressure is already below 185/11
D.

Educational objective:

Cerebellar dysfunction is common among chronic alcohol abusers. Symptoms include gait
instability.
difficulty with rapid movements. and intention tremor.
Common
signs of cerebellar dysfunction are ataxia. broad-based gait. dysmetria. intention tremor.
difficulty with rapid
alternating movements. and nystagmus. Muscle hypotonia can also be observed leading to a
pendular knee
reflex as described in the question.
Educational Objective:
Alzheimer's disease is the most common cause of dementia in the western world. It is initially
characterized
by memory loss. language difficulties and apraxia. followed by impaired judgment and personality

changes.
CT scan shows generalized cortical atrophy.
The associated risk
factors include age. female gender. positive family history. head trauma and Down's syndrome. The
typical
first symptoms are subtle memory loss. language difficulties and apraxia. followed by impaired
judgment and
personality changes. Psychotic features may appear later in the course of the disease. CT scan usually
depicts cortical atrophy.
Educational objective:

The most effective symptomatic therapy for parkinsonism is levodopa/carbidopa. The most
common early
side effects are hallucinations. dizziness. headache. and agitation. After several years of
therapy. involuntary
movements are more likely to occur.
Educational Objective:

Cluster headache usually presents with acute. severe retroorbital pain that wakes the patient
from sleep. It
may be accompanied by redness of the ipsilateral eye. tearing. stuffed or runny nose. and
ipsilateral Horner's
syndrome.
The
recommended prophylactic medications include verapamil. lithium and ergotamine. The
treatment for an
acute attack is inhalation of 100% oxygen and subcutaneous sumatriptan.

Educational objective:

A patient with aphasia usually has a problem involving the cerebral cortex, usually on the
left. Broca's aphasia
with right-sided weakness is a common presentation of a left MCA infarct. A cardiac embolic
source should
be considered in any patient with a cerebral infarct who has atrial fibrillation.
Educational objective:
Computed tomography (CT) scan of the brain without contrast is the initial imaging study of
choice in patients
with unprovoked first seizure to exclude acute neurologic problems (eg. intracranial or

subarachnoid bleed)
that might require urgent intervention. Magnetic resonance imaging is more sensitive than CT
in identifying
most structural causes of epilepsy and is the neuroimaging modality of choice in elective
situations.
Educational objective:
Typical and atypical antipsychotics are good for treating acute agitation in elderly and demented
patients. Benzodiazepines are typically not recommended in this setting.
Lewy body dementia is one
exception where typical antipsychotics should not be used. as these patients tend to be very sensitive
to these
medicines.
Educational objective:
The sudden onset of vertigo. vomiting and occipital headache in a hypertensive patient is strongly
suggestive
of cerebellar hemorrhage. Other manifestations are 6th nerve paralysis. conjugate deviation.
blepharospasm
and coma.
Educational objective:
Patients with acute. severe pain should receive the same standard of pain management regardless of
drug
history. IV morphine is appropriate treatment for acute, severe pain. Physicians should never
undertreat pain,
even if there is a risk of abuse. In the case of concern for abuse. frequent reassessment, outpatient
follow-up.
and referral to a pain specialist is appropriate.
Educational Objective:
Suspect neurofibromatosis type II in a young patient with acoustic neuroma and multiple cafe-au-lait
spots.
MRI with gadolinium enhancement is the best method to diagnose acoustic neuromas.
Educational objective:

Pseudotumor cerebri is a condition that presents with headache. blurry vision. papilledema.
and/or cranial
nerve palsies. CSF examination reveals increased opening pressure. Young. obese women are
most
commonly affected. Vitamin A and isotretinoin can also predispose.
Educational objective:
Impairment of daily functioning is essential in distinguishing between dementia and normal
changes of aging.
Patients with dementia have functional impairments.
Educational objective:
Continued pressure on a bony prominence for a prolonged period of time can result in ischemic
necrosis of
overlying muscle, subcutaneous tissue, and skin leading to formation of a pressure (decubitus) ulcer.
Caregivers should reposition vulnerable patients regularly to reduce the incidence of decubitus ulcers.
Educational Objective:

Suspect pseudotumor cerebri in a young obese female with a headache that is suggestive of a
brain
tumor, but with normal neuroimaging and elevated CSF pressure. The treatment includes
weight reduction
and acetazolamide. Shunting or optic nerve sheath fenestration may be performed to prevent
blindness.
Educational Objective:
Lewy body dementia is characterized by fluctuating cognitive impairment, recurrent visual
hallucinations and
motor features of Parkinsonism.
Educational objective:
Immediate anticoagulation with heparin and surgical intervention (i.e., embolectomy) are crucial to
prevent
tissue death in a patient with ongoing ischemia of the limb.
Educational objective:
Always consider multiple system atrophy when a patient with Parkinsonism experiences orthostatic
hypotension, impotence, incontinence, or other autonomic symptoms.
Educational objective:
Exertional heat stroke occurs in otherwise healthy individuals undergoing conditioning in extreme
heat and
humidity due to thermoregulation failure. Heat exhaustion is due to inadequate fluid and salt
replacement. CNS dysfunction (eg, altered mental status) is not present in heat exhaustion.
Educational Objective:

Acetazolamide is the first line medical treatment for idiopathic benign intracranial
hypertension.
Educational objective:

Brain death refers to a total loss of brain function and is a legally acceptable definition of
death. Criteria
include: absent cranial nerve reflexes, fixed and dilated pupils, no spontaneous breaths, and
agreement of two
physicians.
Educational objective:
Thiamine deficiency can cause Wernicke's encephalopathy, the triad of encephalopathy, oculomotor
dysfunction, and gait ataxia. This condition may be induced iatrogenically in susceptible patients by
administration of glucose without thiamine. Chronic thiamine deficiency can also cause Korsakoff's
syndrome, characterized by irreversible amnesia, confabulation, and apathy.
Educational Objective:
Cerebellar tumors usually produce ipsilateral ataxia, nystagmus, intention tremors, and loss of
coordination.

Other characteristic features of cerebellar tumors include

nystagmus, intention tremor, ipsilateral muscular hypotonia, and marked difficulty in
coordination and
performing rapid, alternating movements
Educational objective:

It is important to be able to identify the signs and symptoms of anticholinergic toxicity and to
know common
possible causes. The classic findings are dry skin, dry mouth, constipation, urinary retention,
flushing, vision
changes, and confusion.
Educational objective:

It is important to be able to identify the signs and symptoms of anticholinergic toxicity and to

know common
possible causes. The classic findings are dry skin, dry mouth, constipation, urinary retention,
flushing, vision
changes, and confusion.
Educational Objective:
Campy/obacferjejuni is the most frequent precipitant of Guillain-Barre Syndrome (GBS).
GBS occurs more frequently in
patients with lymphoma, sarcoidosis and systemic lupus erythematosus. Recent HIV infection and
recent
immunization have also been associated with GBS.
Educational Objective:
It is important to be able to distinguish between different types of tremors. Resting tremor is often the
presenting symptom Parkinson's disease. It usually starts in one hand before involving the other
extremities.
Educational objective:
Acute confusion, extreme hyperthermia >40.5c ( 105.F), tachycardia, and coagulopathic bleeding
after heavy
work under direct sunlight are most likely due to exertional heat stroke.
Educational objective:
Recent studies have suggested that a brain that has seized for >5 minutes (status epilepticus) is at
increased
risk of developing permanent injury due to excitatory cytotoxicity. Cortical laminar necrosis is the
hallmark of
prolonged seizures and can lead to persistent neurologic deficits and recurrent seizures.
Educational objective:
A good history and neurologic examination of a patient with a stroke can often localize the region of
brain
dysfunction and the involved vasculature. An anterior cerebral artery stroke is characterized by
contralateral
motor and/or sensory deficits which are more pronounced in the lower limb than the upper limb.
Educational Objective:
Hyponatremia is one of the important complications of subarachnoid hemorrhage.
Educational objective:
Fatigable muscle weakness that primarily involves the extraocular and bulbar musculature is most
consistent
with myasthenia gravis. Approximately 15% of patients with myasthenia gravis have a coexisting
thymoma,
thus, screening CT of the chest is generally recommended.
Educational Objective:
CT scan without contrast should be done in all patients with a stroke to distinguish between
hemorrhagic or
ischemic stroke. If there is evidence of ischemic stroke, carotid Doppler and TEE are performed to
evaluate
the possible source of embolism.
Educational objective:
Suspect acute glaucoma in a patient with a sudden onset of eye pain, photophobia, and a mid-dilated
pupil.
Ocular tonometry is the best diagnostic test.
Symptoms of acute glaucoma
include a sudden onset of photophobia, eye pain, headache, and nausea. On palpation, the affected eye
may
appear very hard and fleshy. A non-reactive, mid-dilated pupil suggests acute glaucoma. The best

diagnostic
test is tonometry. Drugs that dilate the pupil can precipitate glaucoma.
ducational Objective:
Parkinsonism is caused by overactivity of cholinergic neurons and underactivity of dopaminergic
neurons in
the substantia nigra. A shuffling gait (i.e., the patient appears as if he was chasing his center of gravity)
is
characteristic of the disease.
Educational objective:

Internuclear ophthalmoplegia is a characteristic finding of multiple sclerosis, and is due to

demyelination of the
medial longitudinal fasciculus.
Educational objective:
While effective in the treatment of many bacterial infections, aminoglycosides can also cause
nephrotoxicity
and ototoxicity. Gentamicin sometimes causes severe vestibulotoxicity, resulting in potentially
permanent
vertigo and ataxia.
Educational Objective:
Multiple sclerosis is best diagnosed with MRI. The MRI shows white matter disease, which is very
characteristic for multiple sclerosis.
Educational objective:
Intracranial hypertension is diagnosed when the intracranial pressure equals or exceeds 20 mm Hg. It
may be
due to a number of different causes and typically presents with headaches, vision changes, nausea and
vomiting, changes in awareness, and/or focal neurologic deficits.
Educational Objective:
Treatment of myasthenia crisis consists of endotracheal intubation and withdrawal of
anticholinesterases for
several days.
Educational objective:
Patients with cerebellar hemorrhage present with ataxia. vomiting. occipital headache. gaze palsy. and
facial
weakness. There is no hemiparesis. It is crucial to make an early and correct diagnosis. because
emergent
surgical decompression may be life-saving in such cases.

Educational Objective:
Cauda equina is an acute compression syndrome presenting with acute motor and sensory loss. loss of
rectal tone and urinary retention. This is a surgical emergency.
Educational Objective:

Suspect Creutzfeldt-Jakob disease in an old patient (between 50- to 70-years-old) with

rapidly progressive
dementia. myoclonus and periodic synchronous bi or triphasic sharp wave complexes on
EEG.
Educational objective:
A MMSE score of less than 24 points is suggestive of dementia (total maximum is 30).
Alzheimers disease
presents initially with memory symptoms. with symptoms that progress over a period of
years. Social
disinhibition and personality changes are the early features in frontotemporal dementia.
Neuroimaging may

demonstrate atrophy which is more prominent in the temporal and parietal lobes in patients
with Alzheimers
disease. although imaging should primarily be used to exclude alternative causes for
dementia as opposed to
making a diagnosis of Alzheimers.