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Neurology: UWorld Flashcards

1
Q

What part of the brain is predominantly affected by Progressive multifocal leukoencephalopathy (PML)?

A

Cortical white matter

The brainstem and cerebellum can be involved too.

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1
Q

The following findings are consistent with what diagnosis?

  1. Spontaneous speech: Fluent & voluminous but lacks meaning
  2. Comprehension: Greatly diminished
  3. Repetition: Impaired
  4. Associated features: Right superior visual field defect
A

Wernicke’s (Receptive) aphasia

Patient’s with Wernickes’s aphasia have difficulty comprehending and following commands but are able to speak fluently. However, their speech tends to be rambling without concrete meaning. Receptive aphasia results from a dominant temporal lobe lesion.

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2
Q

A person’s ability to comprehend emotional gestures can be affected by a brain lesion located where?

A

Nondominant temporal lobe

These patients can also develop a contralateral homonymous quadrantanopsia due to the inferior optic radiations involvement.

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3
Q

Any patient admitted for Guillain-Barre syndrome should be evaluated frequently with serial measurements of what?

A

Vital capacity

Vital capacity is the best way to monitor respiratory function and assess for respiratory failure.

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3
Q

The following findings are consistent with what diagnosis?

  1. Spontaneous speech: Fluent with phonemic errors
  2. Comprehension: Relatively preserved
  3. Repetition: Very poor
  4. Associated features: None
A

Conduction aphasia

Conductive aphasia results from a dominant temporal lobe lesion (like receptive aphasia). It occurs when the lesion involves the arcuate fasciculus.

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3
Q

Herpes mainly affects which lobe of the brain?

A

Temporal lobe

Herpes encephalitis accounts for 10-20% of all viral encephalitis, and is the most common cause of fatal sporadic encephalitis in the United States. HSV-1 is the etiological agent in almost all cases beyond the neonatal period. Intravenous acyclovir is the treatment of choice.

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3
Q

What is the management for both cauda equina syndrome and conus medularis syndrome?

A
  1. Emergency magnetic resonance imaging
  2. Intravenous glucocorticoids
  3. Neursurgical evaluation
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4
Q

Diffuse atrophy of the cerebral cortex is a feature of what disease?

A

Alzheimer’s disease

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5
Q

What test/study is useful in differentiating conditions like infectious meningitis and multiple sclerosis?

A

Lumbar puncture

Oligoclonal bands are characteristic of Multiple Sclerosis.

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5
Q

The following characteristics are indicative of which subtype of stroke?

  1. Risk factors: Uncontrolled hypertension, diabetes
  2. Fluctuating symptoms - stuttering progression with periods of improvement
A

Thrombotic (ischemic) stroke

Local in-situ obstruction of an artery occurs.

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5
Q

The following signs/symptoms are characteristic of which diagnosis?

  1. Severe lower back pain with bilateral radiculopathy
  2. Saddle anesthesia
  3. Hyporeflexia
  4. Profound asymmetric motor weakness
  5. Bowel and bladder dysfunction.
A

Cauda equina syndrome

This is contrasted to conus medullaris syndrome which presents with

  1. Severe back pain with lesser degree of radiculopathy
  2. Perianal anesthesia
  3. Hyperreflexia
  4. Mild bilateral motor weakness
  5. Bowel and bladder disturbances
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6
Q

What is the study of choice in diagnosing multiple sclerosis?

A

Magnetic resonance imaging (MRI)

Cerebral and spinal plaques composed of clusters of demyelinated axons appear as hypo/hyperintense lesions on MRI. The lesions involve the white matter of the central nervous system, particularly the periventricular and subpial white matter of the cerebrum (corpus callosum), optic nerves, brain stem, and spinal cord. Lumbar puncture is also recommended to document oligoclonal IgG bands (found in >95% of clinically definite multiple sclerosis patients) in the cerebrospinal fluid to establish the diagnosis.

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6
Q

Tabes dorsalis is a manifestation of what condition?

A

Neurosyphilis

It is characterized by destruction of the posterior columns leading to loss of proprioception. The patient walks with his/her legs wide apart. The feet are lifted higher than usual, and make a slapping sound when they come in contact with the floor. Romberg’s sign may be positive.

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6
Q

Unilateral eye pain, vision loss, and an associated afferent pupillary defect are characteristic features of what condition?

A

Optic neuritis

This is commonly associated with multiple sclerosis, but can be seen with other disorders as well.

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7
Q

The following findings are consistent with a lesion to what region?

  1. Contralateral ataxia
  2. Hemiparesis of the face, trunk, and limbs (ataxic hemiparesis)
  3. Variable loss of contralateral tactile and position sense
A

Medial mid-pontine infarction

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8
Q

Cognitive dysfunction with motor and sensory neurological dysfuntion is characteristic of what condition?

A

Multi-infarct dementia

This accounts for 15-20% of dementia cases. The associated risk factors are: older age, male sex, black race, cigarette smoking, hypertension, diabetes, and vasculitis. The treatment is generally directed towards the risk factors.

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9
Q

The following findings are consistent with what diagnosis?

  1. Deep coma and paraplegia that develops within minutes
  2. Pupils pinpoint and reactive to light
  3. Decerebrate rigidity
A

Pontine hemorrhage

This accounts for 5-12% of cases of hypertensive intraparenchymal hemorrhages.

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10
Q

Hemi-sensory loss with severe dysesthesia (unpleasant abnormal sense of touch) of the affected area is typical for what?

A

A 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.

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11
Q

Corticospinal tract lesions cause what?

A

Spastic paresis

Unilateral corticospinal lesions may cause hemiplegic gait, where the spastic leg is held extended and internally rotated and walking is accomplished by abducting and advancing the spastic leg.

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12
Q

Fatigable muscle weakness that primarily involves the extraocular and bulbar musculature is most consistent with what condition?

A

Myasthenia gravis

Myasthenia gravis affects only motor nerves, with preservation of the sensory nerves. The creatine phosphokinase (CPK) is generally normal, which helps to distinguish it from a primary muscle problem. Approximately 15% of patients with myasthenia gravis have a coexisting thymoma, thus screening computed tomography of the chest is generally recommended.

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13
Q

What condition presents as recurrent, acute-onset vertigo that is precipitated by head movement, and has associated symptoms of nystagmus, nausea, and vomiting?

A

Benign paroxysmal positional vertigo

This occurs when calcium crystals within the inner ear shift position.

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14
Q

What two medications can be given to treat essential tremor in a patient who cannot tolerate propranolol?

A
  1. Primidone
  2. Topiramate
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15
Q

What is the most effective symptomatic therapy for parkinsonism?

A

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.

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16
Q

Acute exacerbations of multiple sclerosis (MS) are treated with what?

A

Corticosteroids

Plasmapheresis may enhance the beneficial effect of immunosuppression. Although they have no proven role in the treatment of acut MS exacerbations, beta-interferon or glatiramer acetate can be used used to decrease the frequency of exacerbations in patients with relapsing-remitting or secondary progressive form of MS.

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17
Q

If there is evidence of ischemic stroke (suggested by computed tomography without contrast of the head), what two tests should be pursued next?

A

Carotid doppler and transesophageal echocardiogram

These are performed to locate a possible source of embolism.

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17
Q

What diagnosis should be suspected in elderly patients with dementia in the setting of severe depression?

A

Pseudodementia

Antidepressants are the treatment of choice.

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18
Q

Lacunar stroke at the basis pontis will have what presentation?

A

Dysarthria-clumsy hand syndrome

It presents as hand weakness and mild motor aphasia, but no sensory abnormalities.

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19
Q

Lower extremity weakness that may ascend over hours to days to involve the arms, and cranial nerves is the typical presentation of what condition?

A

Guillain-Barre syndrome (GBS)

GBS is an acute idiopathic inflammatory demyelinating polyneruopathy.

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19
Q

What condition is characterized by gross dysfunction of the autonomic nervous system with severe orthostatic hypotension?

A

Familial dysautonomia (Riley-Day syndrome)

This is an autosomal-recessive disease seen predominately in children of Ashkenazi jewish ancestry.

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20
Q

What should you suspect in an HIV-infected patient with focal neurological signs and multiple non-enhancing lesions with no mass effect on the CT scan?

A

Progressive multifocal leukoencephalopathy (PML)

PML is an opportunistic infection caused by the JC virus (Human polyomavirus) and is seen in immunocompromised patients.

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20
Q

Diffuse cortical and subcortical atrophy which is disproportionately greater in the temporal and parietal lobes is characteristic of what condition?

A

Alzheimer’s disease

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21
Q

The following findings are consistent with a brain lesion occurring where?

  1. Alternate syndromes with contralateral hemiplegia & ipsilateral cranial nerve involvement
  2. Possible ataxia
A

Vertebrobasilar system (supplying the brain stem)

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22
Q

What condition is characterized by the presence of both upper (spasticity, bulbar symptoms, hyperreflexia) and lower (fasciculations) motor neuron lesions?

A

Amyotrophic lateral sclerosis (ALS)

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23
Q

In all patients with symptoms of stroke, what should be obtained as soon as possible?

A

Computed tomography (CT) without contrast

This is done to rule out hemorrhage, as hemorrhagic and ischemic strokes are treated differently. Once hemorrhage is ruled out, fibrinolytic therapy should be considered. Intravenous alteplase has been shown to improve outcomes in victims of ischemic stroke when given within 3-4.5 hours of symptom onset.

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24
Q

What often presents with signs and symptoms of elevated intracranial pressure like headache, vision changes, nausea, vomiting, and papilledema, and shows a lesion, edema, or a midline shift on non-contrast CT of the brain?

A

Brain tumor

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24
Q

Unilateral motor weakness of the face, arm, and leg without any higher cortical dysfunction or visual field abnormalities is characteristic of a lesion occuring where?

A

Posterior limb of the internal capsule

Lesions in the vertebrobasilar system that supply the brainstem are characterized by “alternate” syndromes, with contralateral hemiplegia and ipsilateral cranial nerve involvement.

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25
Q

Sensory ataxia is associated with which gait abnormality?

A

Wide-based, high stepping gait

This is the result of the loss of proprioception, and is seen with lesions involving the peripheral nerves, dorsal roots or posterior columns. Romberg’s sign may be positive (I.E. the patient sways on standing with his feet together and eyes closed).

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26
Q

What condition is characterized by fluctuating cognitive impairment, bizarre visual hallucinations, and parkinsonism?

A

Dementia with Lewy Bodies

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27
Q

The following signs/symptoms are characteristic of what diagnosis?

  1. Dizziness
  2. Hearing loss
  3. Tinnitus
A

Meniere’s disease

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28
Q

A wide-based, erratic, staggering gait is caused by a lesion in what location?

A

Cerebellum

Patients with cerebellar lesions classically walk as if they are drunk.

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29
Q

Strokes involving the internal capsule are typically accompanied by what category of symptoms?

A

Motor symptoms

It occurs in 7% of patients with hypothyroidism.

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30
Q

Ptosis and a “down and out” gaze in conjunction with normal light and accommodation reflexes indicate what diagnosis?

A

Diabetic cranial nerve III neuropathy

Nerve damage is ischemic, and only somatic nerve fibers are affected. Parasympathetic fibers of CN III retain function.

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31
Q

What condition typically presents with optic neuritis (painful loss of vision), diplopia, sensory deficits, motor weakness, bowel and bladder dysfunction, and neuropsychiatric disturbances?

A

Multiple Sclerosis

Other features include Uhthoff phenomenon (worsening of symptoms with heat) and Lhermitte’s sign (electric shock-like sensation down the spine or limbs on flexion of the neck.

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32
Q

What condition is characterized by ataxia, ophthalmoplegia, confusion, and amnesia with confabulation?

A

Thiamine deficiency (Wernicke-Korsakoff syndrome)

It is particularly common in alcoholics.

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33
Q

What is the treatment of choice for pseudodementia?

A

Antidepressants

Suspect pseudodementia in elderly patients with dementia in the setting of severe depression.

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34
Q

Where does syringomyelia most frequently occur?

A

Lower cervical or upper thoracic spine

When the syringes occur in the upper cervical cord and extend proximally to involve the medulla oblongata, the condition is called syringobulbia.

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35
Q

What is characterized by progressive muscle weakness and areflexia, and is usually associated with spontaneous remission?

A

Guillain-Barre syndrome (GBS)

Sensory symptoms are less pronounced than motor ones, but distal paresthesias can occur. Autonomic disturbances and respiratory failure can also occur and may be life-threatening.

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36
Q

Blindness is a symptoms characteristic of a lesion occurring where?

A

Optic nerve

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37
Q

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

A

Medial longitudinal fasciculus

This is located in the dorsal pontine tegument. It is characterized by the following: On attempted left gaze, the left eye abducts and exhibits horizontal jerk nystagmus, but the right eye remains stationary. On attempted right gaze, the right eye abducts and exhibits horizontal jerk nystagmus, but the left eye remains stationary. The patient will be able to converge both eyes together, without any associated nystagmus.

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38
Q

Which electrolyte abnormality is an important complication of subarachnoid hemorrhage?

A

Hyponatremia

“Cerebral salt-wasting syndrome” may occur in patients with subarachnoid hemorrhage. The pathology involves an inappropriate secretion of vasopressin (causing water retention), and an increased secretion of atrial/brain natriuretic peptide (causing cerebral salt-wasting).

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39
Q

Atrophy of the frontal and/or temporal lobes is a feature of what disease?

A

Pick’s disease

This presents with dementia and personality changes.

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40
Q

Occlusion of which vessel causes contralateral somatosensory and motor weakness (face, arm, leg), conjugate eye deviation toward the side of the infarct, Broca’s expressive aphasia (when dominant side affected), homonymous hemianopia, and hemineglect (when nondominant side affected)?

A

Middle cerebral artery occlusion

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41
Q

Which anticonvulsant used to treat benign essential tremors can precipitate acute intermittent prophyria?

A

Primidone

Most patients who are only mildly affected by essential tremor do not require treatment. Patients who require medication are given either beta-blockers or primidone. Primidone is an anticonvulsant that converts into phenylethylmalonamide and phenobarbital. The administration of primidone may precipitate acute intermittent porphyria, which manifests as abdominal pain, neurologic, and psychiatric abnormalities. Acute intermittent porphyria can be diagnosed by checking for urine porphobilinogen.

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42
Q

Broca’s aphasia is caused by a brain lesion located where?

A

Dominant frontal lobe

Broca’s aphasia is an expressive aphasia.

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43
Q

What condition is characterized as a labyrinthe dysfunction caused by increased pressure of the endolymph, and resulting in recurrent vertigo, tinnitus, and hearing problems?

A

Meniere disease

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44
Q

Imaging studies revealing cortical and subcortical atrophy as well as secondary ventricular enlargement are suggestive of what?

A

AIDS dementia complex

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45
Q

What medication used to treat moderate to severe Alzheimer’s disease works by blocking glutamate’s actions on the NMDA receptor?

A

Memantine

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46
Q

Destruction of the posterior columns leading to loss of proprioception is characteristic of what?

A

Tabes dorsalis

This is a manifestation of neurosyphilis.

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47
Q

What diagnosis should be suspected in a patient with a sudden onset of eye pain, photophobia, and a mid-dilated pupil?

A

Acute glaucoma

Drugs that dilate the pupil can precipitate glaucoma. The best diagnostic test is tonometry.

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48
Q

What diagnosis should be suspected in an old patient (age 50-70 years old) with rapidly progressive dementia, myoclonus, and periodic synchronous bi or triphasic sharp wave complexes on Electroencephalogram (EEG)?

A

Creutzfeldt-Jakob disease

This is a fatal neurodegenerative disease caused by prions. EEG pattern of periodic synchronous bi or triphasic sharp wave complexes is very typical. Brain biopsy shows cortical spongiform changes. The cerebrospinal fluid is usually normal. Most cases are sporadic (80-85%), others are familial or iatrogenic. Death usually occurs within 12 months. There is no specific treatment.

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49
Q

What occurs when calcium crystals within the inner ear shift position?

A

Benign positional vertigo

Patients present with recurrent, acute-onset vertigo that is precipitated by head movement. Nystagmus, nausea and vomiting are common associated findings.

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50
Q

A stepwise cognitive deterioration is characteristic of what diagnosis?

A

Multi-infarct dementia

Psychiatric disturbances such as depression and agitation are common.

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51
Q

What is the most frequent pathogen responsible for causing Guillain-Barre syndrome (GBS)?

A

Campylobacter jejuni

More than two-thirds of patients with GBS have an antecedent respiratory or gastrointestinal infection, of which campylobacter jejuni is the most frequent precipitant. Other precipitants include human herpes viruses, mycoplasma, and hemophilus influenzae. GBS also occurs more frequently in patients with lymphoma, sarcoidosis, and systemic lupus erythematosus. Recent HIV infection and recent immunization have also been associated with GBS.

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52
Q

What is the initial treatment of choice for myasthenia gravis?

A

Oral anticholinesterase (I.E. pyridostigmine)

There are 3 treatment options available for myasthenia gravis. These include acetylcholinesterase inhibitors, immunosuppressive agents, and thymectomy. Anticholinesterases provide symptomatic benefit, but do not induce remission. Immunosuppressive agents and thymectomy may induce remission. Oral anticholinesterase is usually the initial treatment for myasthenia gravis. It produces its useful effect by increasing the availability of acetylcholine at the neuromuscular junction, where the number of acetylcholine receptors is reduced due to acetylcholine receptor antibodies. Pyridostigmine or neostigmine is used for treatment purposes. Edrophonium is a short-acting anti-cholinesterase, and is used for diagnostic purposes only.

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53
Q

What causes paraplegia, bladder and bowel incontinence, and absent sensation from the nipple downwards?

A

A lesion in the upper thoracic spinal cord

A lesion in the lower thoracic spinal cord causes absent sensation from the unbilicus downwards.

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54
Q

In what condition does a T2-weighted magnetic resonance imaging show multifocal ovoid subcortical white matter lesions located in periventricular, juxtacortical, infratentorial, or spinal cord areas?

A

Multiple sclerosis

Cerebrospinal fluid analysis shows normal pressure and the presence of olioclonal IgG bands in >95% of patients.

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55
Q

What condition is characterized by ignoring the left side of a space, and involves the right (non-dominant) parietal lobe?

A

Hemi-neglect syndrome

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56
Q

What is the treatment for heat stroke?

A

Induction of evaporative cooling to reverse hyperthermia

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57
Q

What is the drug of choice for trigeminal neuralgia?

A

Carbamazepine

It is the most effective treatment for trigeminal neuralgia, being effective in approximately 80% of such individuals. 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.

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58
Q

Atrophy of the caudate nucleus is a characteristic feature of what condition?

A

Huntington’s chorea

This usually presents at 40-50 years old.

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59
Q

What test/study is useful in excluding multi-infarcts or normal pressure hydrocephalus from a differential diagnosis?

A

MRI

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60
Q

A CT scan that shows scarring and atrophy in the setting of an antecedant measles infection many years previous is suggestive of what diagnosis?

A

Subacute sclerosing panencephalitis

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60
Q

Approximately 2/3 of Guillain-Barre patients give a history of what?

A

An antecedent respiratory tract or gastrointestinal (especially Campylobacter) infection

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60
Q

Parkinson Disease is a neurodegenerative disorder caused by the accumulation of what within the neurons of the substantia nigra pars compacta?

A

Alpha-synuclein

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60
Q

What test/study is typically used to assess for peripheral neuropathy?

A

Peripheral nerve conduction study

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60
Q

Intracranial hypertension is defined as intracranial pressure of what?

A

_>_20 mmHg

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60
Q

Dizziness, ataxia, weakness, and a tendency to sway to one side is characteristic of what?

A

Lateral cerebellar infarction

The tendency is to sway towards the side of the lesion.

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60
Q

What is due to microatheroma and liphyalinosis in small penetrating arteries of the brain?

A

Lacunar stroke

They often affect the internal capsule and result in pure motor dysfuntion. Lacunes comprise 25% of ischemic strokes. Hypertension and diabetes are two major risk factors.

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60
Q

What disease is similar to Alzheimer’s disease, except that it presents at an earlier age?

A

Pick’s disease

It is seen more frequently in females, and frequently causes personality changes due to involvement of the frontal lobes.

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60
Q

Wernicke’s aphasia is caused by a brain lesion located where?

A

Dominant temporal lobe

Wernicke’s aphasia is a receptive aphasia.

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60
Q

What condition is characterized by a tremor that is suppressed at rest and exacerbated toward the end of a goal-directed movement?

A

Essential tremor

Affected patients typically have difficulty holding a newspaper, writing legibly, drinking a cup of coffee, or feeding themselves.

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60
Q

What is the only antiplatelet agent that is effective in reducing the risk of early recurrence of ischemic stroke?

A

Aspirin

It should be given within 24 hours to all patients presenting with ischemic stroke. Aspirin plus dipyridamole or clopidogrel is recommended for patients who have recurrent stroke on aspirin therapy.

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60
Q

Hemi-neglect syndrome is cause by what?

A

A lesion of the right (non-dominant) parietal lobe

The non-dominant parietal lobe is responsible for spatial organization.

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60
Q

What is the test of choice in diagnosing cavernous sinus thrombosis?

A

Magnetic resonance imaging with magnetic resonance venography

Treatment includes broad-spectrum intravenous antibiotics and prevention or reversal of cerebral herniation.

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60
Q

What medication is indicated when urgen reversal of the action of heparin is needed?

A

Protamine sulfate

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61
Q

Tarsal tunnel syndrome results from entrapment of what nerve?

A

Posterior tibial nerve

This occurs as it passes underneath the flexor retinaculum on the medial aspect of the ankle. Symptoms include dysesthesia and numbness in the plantar foot.

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62
Q

What 3 medications are recommended for prophylaxis of cluster headaches?

A
  1. Verapamil
  2. Lithium
  3. Ergotamine

The treatment for an acute attack is inhalation of 100% oxygen and subcutaneous sumatriptan.

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63
Q

Cavitary expansion of the spinal cord is the pathophysiological mechanism of what condition?

A

Syringomyelia

The most characteristic feature is the presence of a cord cavity, which usually communicates with the central canal of the spinal cord. The most frequent site of involvement is the lower cervical or upper thoracic region. When the syringes occur in the upper cervical cord and extend proximally to involve the medulla oblongata, the condition is called syringobulbia.

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64
Q

What is the most common site of ulnar nerve entrapment?

A

The elbow

This is where the ulnar nerve lies at the medial epicondylar groove.

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65
Q

The following signs/symptoms are characteristic of which diagnosis?

  1. Severe back pain with lesser degree of radiculopathy
  2. Perianal anesthesia
  3. Hyperreflexia
  4. Mild bilateral motor weakness
  5. Bowel and bladder disturbances
A

Conus medullaris syndrome

This is contrasted to cauda equina syndrome which presents with

  1. Severe lower back pain with bilateral radiculopathy
  2. Saddle anesthesia
  3. Hyporeflexia
  4. Profound asymmetric motor weakness
  5. Bowel and bladder dysfunction.
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67
Q

The following findings are consistent with what diagnosis?

  1. Severe headache
  2. Bilateral periorbital edema
  3. Cranial nerve III, IV, V, and VI deficits
A

Cavernous sinus thrombosis

Cranial nerves III, IV, V, and VI pass through the cavernous sinus, which has anastomoses crossing midline. As a result, unilateral symptoms (I.E. headache, binocular palsies, periorbital edema, hypoesthesia, or hyperesthesthia in V1/V2 distribution) can rapidly become bilateral. Magnetic resonance imaging with magnetic resonance venography is the imaging modality of choice for diagnosis of cavernous sinus thrombosis.

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68
Q

The following symptoms are suggestive of what?

  1. Diffuse headaches that tend to be worse in the morning
  2. Nausea and vomiting early in the day
  3. Vision changes
  4. Papilledema
  5. Cranial nerve deficits
  6. Somnolence
  7. Confusion
  8. Unsteadiness
  9. Cushing’s reflex (Hypertension and bradycardia)
A

Intracranial hypertension

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69
Q

The following findings are consistent with a lesion to which vessel in the brain?

  1. Contralateral somatosensory & motor weakness (predominantly in lower extremity)
  2. Abulia (lack of will or initiation)
  3. Dyspraxia (inability to perform coordinated movements)
  4. Emotional disturbances
  5. Urinary incontinence
A

Anterior cerebral artery

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70
Q

What diagnosis should be suspected in a patient with mood disturbance, dementia, chorea, and a family history of similar symptoms?

A

Huntington’s chorea

This is an autosomal dominant condition affecting both sexes equally. The age at presentation is typically between 30-50 years. The typical presenting features are mood disturbances (depression, apathy), dementia, and choreiform movements (facial grimacing, ataxia, dystonia, tongue protrusion, writhing movements of extremities). The symptoms are progressive, eventually disabling the patient severely.

71
Q

What medication has been shown to decrease the frequency of relapse, and reduce disability in patients with the relapsing-remiting form of multiple sclerosis?

A

Interferon-beta

Interferon-beta may slow the long-term progression of relapsing-remitting multiple sclerosis.

72
Q

The “clasp-knife” phenomenon refers to a velocity-dependent resistance to passive movement of the limb, and is seen in patients with hypertonia due to what?

A

Pyramidal tract disease (upper motor neuron lesion)

The clasp knife reflex refers to a stretch reflex with a rapid decrease in resistance when attempting to flex a joint, usually during a neurological examination. It is one of the characteristic responses of an upper motor neuron lesion.

73
Q

Damage to the lateral spinothalamic tracts causes what symptoms?

A

Contralateral loss of pain and temperature sensation beginning two levels below the level of the lesion

74
Q

What will the cerebrospinal fluid of a patient with Guillain-Barre syndrome show regarding the following?

Protein

White blood cell count (WBC)

Red blood cell count (RBC)

Glucose

A
  1. Protein: High
  2. WBC: Normal
  3. RBC: Normal
  4. Glucose: Normal
75
Q

When can prophylactic anticoagulation with warfarin be started for a patient following an acute cardioembolic stroke?

A

2 weeks after stroke

This is done to prevent recurrence. Urgent anticoagulation with heparin is not recommended for acute ischemic stroke, even when the etiology is cardioembolic, because of the increased risk for intracerebral hemorrhage.

76
Q

What is the mechanism by which normal pressure hydrocephalus (NPH) is thought to occur?

A

Decreased cerebrospinal fluid absorption or transient increases in intracranial pressure that cause permanent ventricular enlargement without chronically increasing intracranial pressure

NPH is characterized clinically by dementia, gait disturbances, and incontinence.

77
Q

What condition is associated with small cell carcinoma of the lung, and results from autoantibodies directed against voltage-gated calcium channels in the presynaptic motor nerve terminal?

A

Eaton-Lambert syndrome

79
Q

An MRI showing a ring-enhancing lesion that is usually solitary (or a few in number), weakly enhancing, and periventricular is suggestive of what?

A

Primary CNS lymphoma

This is also known as microglioma. It is a primary intracranial tumor appearing mostly in patients with severe immunosuppression (typically patients with AIDS). Primary CNS lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients, but not in immunocompetent patients. MRI or contrast enhanced CT classically shows multiple ring-enhancing lesions in the deep white matter. The major differential diagnosis based on imaging is cerebral toxoplasmosis, which is also prevalent in AIDS patients and also presents with a ring-enhanced lesion, although toxoplasmosis generally presents with more lesions and the contrast enhancement is typically more pronounced. Imaging techniques cannot distinguish the two conditions with certainty, and cannot exclude other diagnoses. Thus, patients undergo a brain biopsy.

80
Q

What is Amaurosis fugax a warning sign for?

A

Impending stroke

Amaurosis fugax is defined as painless loss of vision from emboli. Cholesterol particles (Hollenhorst bodies) may be seen in the eye. This event is a warning sign of an impending stroke. An underlying embolic disease is almost always present. Most emboli occur from the carotid bifurcation; hence, a duplex ultrasound of the neck is often done to identify any plaques which may be present.

81
Q

Where is the most common site for a lacunar infarct?

A

The posterior internal capsule

This produces a pure motor stroke (the posterior internal capsule carries corticospinal and corticobulbar motor fibers).

82
Q

What are the first two steps in the treatment of myasthenia crisis?

A

Endotracheal intubation an withdrawal of anticholinesterases for several days

Myasthenia crisis is a life-threatening condition that is characterized by weakness of the respiratory and pharyngeal muscles. The treatment of all patients includes endotracheal intubation and withdrawal of anticholinesterases for several days. The most common cause of myasthenic crisis is an intercurrent infection, and in such cases antibiotics are an important part of management. All patients with suspected myasthenic crisis should have bedside pulmonary function tests monitored, such as vital capacity and tidal volume. Plasmapheresis or intravenous immunoglobulin may hasten the recovery of patients with myasthenia crisis, but these are not the first steps in management.

83
Q

What typically occurs in elderly patients and presents with eye pain, visual loss, and headache?

A

Acute angle-closure glaucoma

84
Q

What participates in the initiation and control of movement?

A

Basal ganglia

85
Q

Lacunar infarction in the anterior limb of the internal capsule will have what presentation?

A

Ataxic-hemiparesis

  • It will present with weakness that is more prominent in the lower extremity, along with ipsilateral arm and leg incoordination. The internal capsule is a white matter structure in the brain that carries information past the basal ganglia, separating the caudate nucleus and the thalamus from the putamen and the globus pallidus. The internal capsule contains both ascending and descending axons. The corticospinal tract constitutes a large part of the internal capsule.* The anterior limb (crus anterius) is the part in front of the genu, between the head of the caudate nucleus and the lenticular nucleus**. The posterior limb (crus posterius) is the part behind the genu, between the thalamus and lenticular nucleus
  • .*
86
Q

Intravenous thrombolytics are recommended for eligible patients presenting with acute ischemic stroke within what time frame?

A

Within 3-4.5 hours of symptom onset

Thrombolytics are used in nonhemorrhagic ischemic strokes.

87
Q

What is the treatment of choice for agitation in elderly and demented patients?

A

Typical and atypical antipsychotics

The treatment of choice is haloperidol, but risperidone and quetiapine can also be used. Lewy body demential is one exception where typical antipsychotics should not be used, as these patients tend to be very sensitive to these medicines. Benzodiazepines may be used to treat agitation in young patients, however, they are typically contraindicated in older patients because they metabolize the drugs slowly (making the drug effect last very long), because benzodiazepines often worsen confusion, and because the elderly are at increased risk for all adverse events associated with benzodiazepines (I.E. withdrawal, dependence, and motor impairment).

88
Q

What percent of patients with Guillain-Barre syndrome develop neuromuscular respiratory failure requiring mechanical ventilation?

A

20-30%

The risk for ventilatory failure increases significantly when the vital capacity falls below 15mL/kg, especially if there has been a clear downward trend.

89
Q

What condition is characterized by dementia, abnormal broad-based shuffling gait, and urinary incontinence?

A

Normal pressure hydrocephalus

Patients tend to be bradykinetic.

90
Q

What are the 3 most common causes of foot drop?

A
  1. Neuropathy
  2. Radiculopathy
  3. Traumatic damage to the common peroneal nerve

Foot drop is most commonly caused by peripheral neuropathy. Foot drop also classically results from trauma to the common peroneal nerve or radiculopathy to any of the spinal roots that contribute to the common peroneal nerve (L4-S2). Finally, foot drop may also be congenital, such as in Charcot-Marie-Tooth disease.

91
Q

A lacunar infarct in the posterior limb of the internal capsule will have what presentation?

A

Pure motor hemiparesis

It will present with unilateral motor deficit (face, arm, and to a lesser extent the leg) and mild dysarthria will be present but no sensory, visual, or higher cortical dysfunction.

92
Q

What is the prognosis for progressive multifocal leukoencephalopathy?

A

The mean duration of survival from the time of diagnosis is 6 months

There is no effective treatment for PML

93
Q

Lateral cerebellar infarction results in what symptoms?

A

Dizziness, ataxia, and weakness

95
Q

Lateral medullary infarct (Wallenberg syndrome) occurs due to occlusion of which artery?

A

Posterior inferior cerebellar artery

or

Vertebral artery

96
Q

What is the principle cause of a lacunar stroke?

A

Hypertension

Hypertension induces lipohyalinotic thickening of the small vessels. Lacunae are formed as a result of thrombotic occlusion of penetrating vessels, which are usually 0.5-0.8 mm in diameter. The area of ischemic damage in the brain tissue is relatively small, thus explaining why the neurologic deficit is typically not prominent. There are several classical lacunar syndromes, including dysarthria-clumsy hand syndrome, which can occur as a result of stroke in the basal pons.

97
Q

The following signs/symptoms are characteristic of what diagnosis?

  1. Fluctuating cognitive impairment
  2. Bizarre visual hallucinations
  3. Parkinsonism that is poorly responsive to dopaminergic agonist therapy
A

Dementia with Lewy bodies

Symptoms worsen with neuroleptic drugs.

98
Q

The following bulbar symptoms are consistent with what diagnosis?

  1. Dysphagia
  2. Hoarseness
  3. Diminished gag reflex
A

Lateral medullary infarct (Wallenberg syndrome)

The nucleus ambiguus is located in the ventrolateral medulla and contains the motor neurons that contribute to the 9th and 10th cranial nerves. The motor neurons of the nucleus ambiguus innervate the striated muscles of the larynx and pharynx as well as provide the preganglionic parasympathetic supply to thoracic organs, including the esophagus, heart, and lungs. Injury to this nucleus and its pathways causes hoarseness and dysphagia. Patients also develop loss of pain and temperature over the ipsilateral face and contralateral body, ipsilateral bulbar muscle weakness, vestibulocerebellar impairment (I.E. vertigo, nystagmus), and Horner’s syndrome. Motor function of the face and body is typically spared.

99
Q

Most cases of syringomyelia occur in association with what?

A

Arnold-chiari malformation

This is caudal displacement of cerebellar tonsils through the foramen magnum. In such patients, neuroimaging may show caudal displacement of the fourth ventrical.

100
Q

What diagnosis should be suspected in a young female with bilateral trigeminal neuralgia?

A

Multiple sclerosis (MS)

MS is one of the few conditions that may present with bilateral trigeminal neuralgia.

102
Q

Medial medullary syndrome is typically due to branch occlusion of which vessel?

A

Vertebral artery

or

Anterior spinal artery

103
Q

Livedo reticularis, a mottled vascular pattern on the lower extremities, is a side effect of what medication?

A

Amantadine

Ankle edema is another side effect of amantadine.

105
Q

The following signs/symptoms are characteristic of what diagnosis?

Personality change (euphoria, disinhibition, apathy)

Compulsive behaviors (Peculiar eating habits)

Hyperorality

Impaired memory

A

Pick’s disease (fronto-temporal dementia)

Visual and spatial functions are usually intact.

107
Q

Which nerve controls corneal sensation?

A

The ophthalmic branch (V1) of the trigeminal nerve (CN V)

V1 carries sensory fibers to the scalp, forehead, upper eyelid, conjunctiva, cornea, nose, and frontal sinuses. When it is damaged, patients can suffer from corneal injury without awareness.

109
Q

What is the test of choice to support the clinical diagnosis of multiple sclerosis?

A

Magnetic resonance imaging (MRI)

The characteristic MRI lesions are cerebral or cerebellar plaques. The typical locations of the plaques are the periventricular regions, corpus callosum, deep white matter and basal ganglia.

110
Q

The following findings are consistent with what diagnosis?

  1. Spontaneous speech: Sparse and nonfluent
  2. Comprehension: Relatively preserved
  3. Repetition: Impaired
  4. Associated features: Right hemiparesis
A

Broca’s aphasia

An easy way to remember is that Broca’s aphasia often represents a broken speech system. Expressive aphasia results from a dominant frontal lobe lesion. Contralateral hemiparesis occurs due to involvement of the supplementary motor cortex.

111
Q

A lesion in the posterior column would cause what?

A

Ataxia

113
Q

Atrophy of the lenticular nucleus is most marked in patients with what disease?

A

Wilson’s disease

114
Q

Intense unilateral retroorbital pain of sudden onset (often at night) that peaks rapidly and lasts approximately 2 hours is characteristic of which diagnosis?

A

Cluster headache

This occurs more commonly in men. It may be accompanied by redness of the ipsilateral eye, tearing, stuffed or runny nose, and ipsilateral Horner’s syndrome. The attacks occur in clusters, daily, for 6-8 weeks, followed by remission lasting for up to a year. Since attacks are short but severe, prophylaxis is the key to management. The recommended prophylactic medications include verapamil, lithium, and ergotamine. The treatment for an acute attack is inhalation of 100% oxygen and subcutaneous sumatriptan.

116
Q

Lesions of the lateral cerebellar hemisphere present with what?

A

Dizziness and ipsilateral ataxia

117
Q

What is indicated in all victims of ischemic stroke who are not candidates for fibrinolytic therapy?

A

Aspirin

Aspirin has proven benefits for both primary and secondary stroke prevention, however, it should not be used as a substitute for fibrinolytic therapy as it does not confer the same benefits for neurologic recovery. In patients who do recieve fibrinolytic therapy, aspirin should be held for 24 hours.

119
Q

Hyperactive reflexes and an upgoing plantar reflex are suggestive of what pathology?

A

An upper motor neuron lesion

120
Q

What complication of prolonged seizures can lead to persistent neurological deficits and recurrent seizures?

A

Cortical laminar necrosis

This is the death of cells in the cerebral cortex of the brain in a band-like pattern, with a relative preservation of cells immediately adjacent to the meninges. 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.

121
Q

Convergent strabismus and horizontal diplopia are symptoms characteristic of a lesion occurring where?

A

Abducens nerve

123
Q

What condition presents with early problems in visuospatial abilities and anterograde memory formation, while maintaining the preservation of old memories?

A

Alzheimers disease

Personality and behavioral changes (I.E. hypersexuality, agitation) may occur as the disease progresses. Hallucinations and changes in alertness are late findings.

124
Q

What diagnosis should be suspected in a patient with parkinsonism who experiences orthostatic hypotension, impotence, or other autonomic symptoms?

A

Multiple system atrophy (Shy-Drager syndrome)

This is a degenerative disease characterized by parkinsonism, autonomic dysfunction, and widespread neurological signs (cerebellar, pyramidal or lower motor neuron signs). The accompanying bulbar dysfunction and laryngeal stridor may be fatal. Anti-Parkinsonism drugs are generally ineffective, and treatment is aimed at intravascular volume expansion with fludrocortisone, salt supplementation, alpha-adrenergic agonsists, and application of constrictive garments to the lower body.

125
Q

The following findings are characteristic of a lesion to which vessel in the brain?

  1. Homonymous hemianopia
  2. Alexia without agraphia (if dominant hemisphere)
  3. Visual hallucinations
  4. Sensory symptoms
  5. Third nerve palsy with pareses of vertical eye movement
  6. Motor deficits
A

Posterior cerebral artery

126
Q

The following findings are consistent with what diagnosis?

  1. Flu-like illness
  2. Malaise
  3. Vomiting
  4. Photophobia
  5. Stupor
  6. Fever
  7. Cerebrospinal fluid with elevated WBC count
A

Aseptic (viral) meningitis

This usually occurs in the summer months, and is caused by an echovirus. Treatment is supportive care.

127
Q

Sumatriptan and high-flow oxygen are the treatment regimen for what condition?

A

Cluster headaches

128
Q

What is the most rapid-acting and effective in aborting the pain during an acute attack of a cluster headache?

A

100% oxygen

Subcutaneous or nasal sumatriptan may be used to abort an acute attack of cluster headache; however, nasal oxygen is more effective and rapid in action.

129
Q

Malignant hyperthermia affects genetically susceptible individuals during anesthesia involving which agents?

A

Halothane and succinylcholine

131
Q

What diagnostic test would be the best method to diagnose patient with multiple cafe-au-lait spots and suspected acoustic neuromas?

A

Magnetic resonance imaging (MRI) with gadolinium enhancement.

Such a patient likely has neurofibromatosis type II. MRI with gadolinium is enhancement is preferred over computed tomography (CT).

132
Q

What are the most common presenting symptoms of progressive multifocal leukoencephalopathy (PML)?

A

Hemiparesis and disturbances in speech, vision and gait. Cranial nerve deficits may occasionally develop.

PML lesions typically do not produce a mass effect, and the onset of symptoms is usually gradual.

134
Q

What is the best diagnostic test for suspected acute glaucoma?

A

Tonometry

Acute glaucoma should be suspected in a patient with a sudden onset of eye pain, photophobia, and a mid-dilated pupil.

135
Q

What is used to prevent stroke in patients who have failed or are intolerant of aspirin?

A

Clopidogrel

It is not recommended for aspirin-naive patients presenting with stroke.

136
Q

The following findings are consistent with a brain lesion occurring where?

  1. Contralateral somatosensory & motor weakness
  2. Conjugate eye deviation toward side of lesion
  3. Homonymous hemianopia
  4. Aphasia or hemineglect
A

Middle cerebral artery

Aphasia occurs when lesion is in dominant hemisphere. Hemineglect occurs when lesion is in nondominant hemisphere.

138
Q

What are 5 risk factors for Alzheimer’s disease?

A
  1. Older age
  2. Female gender
  3. Positive family history
  4. Dead trauma
  5. Down’s dyndrome

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.

139
Q

What condition is characterized by dementia, posterior spinal column deficits, and anemia?

A

Vitamin B12 deficiency

The most common cause is pernicious anemia.

140
Q

The following characteristics are indicative of which subtype of stroke?

  1. Severe headache at onset of neurological symptoms
  2. Neck stiffness
A

Spontaneous subarachnoid hemorrhage

Rupture of an arterial saccular “berry” aneurysm or from an arteriovenous malformation. Focal deficits are uncommon.

141
Q

Polymyalgia rheumatica can be seen in up to 50% of patients with what diagnosis?

A

Temporal arteritis

Patients typically present with aching and morning stiffness, with pain in the shoulders, hip girdle, and neck. The Erythrocyte sedimentation rate (ESR) is usually elevated, and symptoms usually improve with steroids.

142
Q

What is this initial imaging study of choice in patients with an unprovoked first seizure?

A

Computed tomography (CT) scan

This is done to exclude acute neurologic problems (I.E. 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. EEG is a useful diagnostic study in patients with unprovoked first seizure. It helps to stratify patient risk in the need for maintenance antiepileptic agents, however, it is important to rule out any structural brain lesions (I.E. mass, intracranial bleeding) with neuroimaging prior to EEG.

143
Q

Fever, severe focal back pain, and neurological deficits compose the classic triad of which condition?

A

Spinal epidural abscess

The symptoms are progressive and include radiculopathy, motor/sensory deficits, bowel/bladder dysfunction, and eventual paralysis. Risk factors include intravenous drug use, immunocompromised state, and spinal trauma or surgery.

144
Q

Damage to which nerve tract causes contralateral loss of pain and temperature sensation beginning two levels below the level of the lesion?

A

Lateral spinothalamic tract

145
Q

What is the most common cause of drug-induced myopathy?

A

Steroids

It can occur weeks to months after starting steroid therapy. 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.

146
Q

Patients taking metoclopramide should be monitored closely for the development of what drug-induced side effect?

A

Extrapyramidal symptoms

Metoclopramide is a prokinetic agent used to treat nausea, vomiting, and gastroparesis. It is a dopamine receptor antagonist used to treat nausea, vomiting, and gastroparesis. It has significant prokinetic properties, which include promoting increased peristalsis, increased strength of gastric contractions, and relaxation of the pyloric sphincter. Common side effects include agitation and loose stools. Extrapyramidal symptoms (tardive dyskinesia, dystonic reactions, and parkinsonism) occur less frequently. Rarely, neuroleptic malignant syndrome may occur. Treatment for a dystonic reaction involves discontinuation of the medication and administration of benztropine or diphenhydramine.

147
Q

What disorder results in a waddling gait owing to weakness of the gluteal muscles?

A

Muscular dystrophy

148
Q

Upper and lower motor neuron degeneration suggests what diagnosis?

A

Amyotrophic lateral sclerosis (ALS)

149
Q

What condition is characterized by personality change, compulsive behavior, and impaired memory?

A

Fronto-temporal dementia (Pick’s disease)

The characteristic features of this condition are personality changes (I.E. euphoria, disinhibition, apathy), compulsive behaviors (I.E. Peculiar eating habits, hyperorality), and impaired memory. Visuospatial functions usually remain intact. A positive family history may be present (20-40% of patients have a family history of frontotemporal dementia).

150
Q

What is present in the cerebrospinal fluid (CSF) in 85-90% of patients with multiple sclerosis (MS)?

A

Oligoclonal bands

CSF pressure, protein, and cell count are grossly normal. The predominate immunoglobulin type is IgG, although IgM and IgA are also increased.

151
Q

What is the treatment for a dystonic reaction to a medication?

A

Discontinuation of the medication and administration of benztropine or diphenhydramine

152
Q

The following findings are consistent with what diagnosis?

  1. Contralateral paralysis of the arm and leg
  2. Tongue deviation toward the lesion
  3. Contralateral loss of tactile and position sense can also occur
A

Medial medullary syndrome (alternating hypoglossal hemiplegia)

This typically occurs due to branch occlusion of the vertebral or anterior spinal artery.

153
Q

The sudden onset of vertigo, vomiting an occipital headache in a hypertensive patient is strongly suggestive of what?

A

Cerebellar hemorrhage

Other manifestations are 6th nerve paralysis, conjugate deviation, blepharospasm, and coma.

155
Q

The following characteristics are indicative of which subtype of stroke?

  1. Risk factors: Uncontrolled hypertension, coagulopathy, illicit drug use
  2. Symptoms progress over minutes to hours
  3. Focal neurological symptoms appear early, followed by features of increased intracranial pressures
A

Intracerebral hemorrhagic stroke

157
Q

What test/study is typically used to assess patients with seizure, sleep, and metabolic disorders?

A

Electroencephalography (EEG)

158
Q

Idiopathic intracranial hypertension is caused by what mechanism?

A

Impaired cerebral spinal fluid absorption or excess production

It can present with acute thunderclap headache (worsened with lying flat) associated with vision changes and nausea. Noncontrast head CT scan usually shows slit-like lateral ventricles without evidence of acute blood products.

159
Q

The following findings are consistent with what diagnosis?

  1. Weakness of the muscles of mastication
  2. Diminished jaw jerk reflex
  3. Impaired tactile and position sensation over the face
A

Lateral mid-pontine lesion

This is where the motor and principal sensory nuclei of the ipsilateral trigeminal nerve is. The trigeminal nerve is responsible for mastication, jaw jerk reflex, and tactile sensation and proprioception of the face.

160
Q

The following findings are consistent with what diagnosis?

  1. Gait disturbance
  2. Dementia
  3. Urinary incontinence
A

Normal pressure hydrocephalus

Lumbar puncture reveals the normal CSF pressures, and MRI shows the enlarged ventricles.

161
Q

A stroke in the ventoposterolateral nucleus of the thalamus will have what presentation?

A

Pure sensory deficits

It will present with unilateral numbness, paresthesias, and hemisensory deficit involving the face, arm, trunk, and leg.

162
Q

How would Cerebral toxoplasmosis appear on magnetic resonance imaging (MRI)?

A

The MRI would show lesions that are usually multiple, spherical, and located in the basal ganglia

This diagnosis is less likely if the patient is receiving Trimethoprim-Sulfamethoxazole (TMP-SMX)

163
Q

What condition is characterized by choreathetoid movements, behavioral disturbances, and dementa?

A

Huntington’s disease

This is an autosomal dominant neurodegenerative disease affecting the caudate and putamen. The mean age of onset is between 35-44 years of age.

164
Q

The following findings are consistent with which diagnosis?

  1. Insidious progression of headache that worsens when lying down
  2. Pulsatile tinnitus
  3. Blurry vision or vision loss
  4. Nausea and vomiting
  5. papilledema
  6. Cranial nerve palsies
  7. Increased opening pressure on lumbar puncture with otherwise normal cerebrospinal fluid
A

Idiopathic intracranial hypertension (pseudotumor cerebri)

It is most common in young, obese women. Growth hormone, tetracyclines (I.E. minocycline, doxycycline), and excessive vitamin A and its derivatives (I.E. isotretinoin, all-trans-retinoic acid) can cause pseudotumor cerebri. Withdrawal of these medications leads to symptom resolution.

166
Q

What is the treatment for an acute episode of cluster headache?

A

Inhalation of 100% oxygen and subcutaneous sumatriptan

The recommended prophylactic medications include verapamil, lithium and ergotamine. The treatment for an acute attack is inhalation of 100% oxygen and subcutaneous sumatriptan.

167
Q

The presence of EBV DNA in CSF is quite specific for what diagnosis?

A

Primary CNS lymphoma

169
Q

What is the most common presenting sign of Parkinson Disease (PD)?

A

Asymmetric resting tremor in the distal part of an upper extremity.

170
Q

Ptosis and “down and out” deviation of the eye is characteristic of a lesion occurring where?

A

Occulomotor nerve

171
Q

The following signs/symptoms are characteristic of what diagnosis?

  1. Dementia
  2. Gait ataxia
  3. Urinary incontinence
A

Normal Pressure Hydrocephalus

172
Q

Pronator drift is a relatively sensitive and specific physical exam finding for what?

A

Upper motor neuron damage affecting the upper extremities

Some stroke patients will demonstrate pronator drift in the absence of other significant findings. Upper motor neuron lesions cause a weakness in supination that results in the pronator muscles becoming dominant. As a result, when these patients close their eyes and extend their arms with the palms up, the affected arm will tend to pronate. When people feign upper-extremity weakness, however, they typically will drop the “affected” arm without pronating.

173
Q

Bitemporal hemianopia is a symptom characteristic of a lesion occurring where?

A

Optic chiasm

174
Q

What medication can cause severe vestibulotoxicity, resulting in potentially permanent vertigo and ataxia?

A

Gentamicin

Aminoglycosides can cause nephrotoxicity and ototoxicity.

176
Q

The following signs symptoms are characteristic of what diagnosis?

  1. Ataxia
  2. Vomiting
  3. Occipital headache
  4. Gaze palsy
  5. Facial weakness
A

Cerebellar hemorrhage

It is crucial to make an early and correct diagnosis, because emergent surgical decompression may be life-saving in such cases.

177
Q

Contralateral hemianopia is a symptom characteristic of a lesion occurring where?

A

Optic radiation

178
Q

A lateral frontal cortex lesion results in what symptoms?

A
  1. Hemiparesis
  2. Motor aphasia (if the dominant lobe is involved)
179
Q

What is the most common site of hypertensive hemorrhage?

A

The putamen

This accounts for 35% of hypertensive hemorrhages. The internal capsule lies adjacent to the putamen and is almost always involved, thereby leading to hemiparesis. Other neurological signs include hemi-sensory loss, homonymous hemianopsia, stupor, and coma. The eyes are deviated away from the paralytic side.

180
Q

Painless, transient monocular blindness that lasts a few seconds is characteristic of what condition?

A

Amaurosis fugax

It is usually vascular (I.E. embolus to ophthalmic artery) in origin. It would be unlikely in a young patient with low risk of atherosclerosis.

181
Q

What condition is characterized by acute motor and sensory loss, as well as loss of rectal tone and urinary incontinence?

A

Cauda equina syndrome

This is a surgical emergency, and a neurosurgeon should be notified as soon as possible. An MRI should be performed to identify the site of compression/fracture, followed by surgery. Spinal cord compression may occur repeatedly in males with prostate cancer that has metastasized to the vertebral column.

182
Q

Infarction of the medial vermis of the cerebellum can cause what symptoms?

A

Severe vertigo and nystagmus

183
Q

What is the treatment for restless leg syndrome?

A
  1. Iron supplementation and conservative measures (I.E. leg massage, heating pads, regular exercise, and avoiding aggravating factors)
  2. Pharmacotherapy with dopamine agonists (I.E. pramipexole) or alpha-2-delta calcium channel ligands (I.E. gabapentin).
184
Q

What is characterized by rapidly progressive (over a matter of hours) ascending paralysis, absence of fever and sensory abnormalities, and normal CSF examination?

A

Tick-borne paralysis

Ticks must feed for 4-7 days and are typically found on patients after meticulous searching. Removal of the tick results in spontaneous improvement in most patients. Guillain-Barre syndrome presents with an ascending symmetrical paralysis over days to weeks, but not usually hours.

186
Q

Diabetic mononeuropathy often involves which cranial nerve?

A

Cranial nerve III

Nerve damage is ischemic, and only somatic nerve fibers are affected. Parasympathetic fibers of CN III retain function. Ptosis and a “down and out” gaze in conjunction with normal light and accommodation reflexes indicate diabetic CN IIII neuropathy.

187
Q

Muscular dystrophy is associated with which gait abnormality?

A

Waddling gait

This occurs because of weakness of the gluteal muscles.

188
Q

The following characteristics are indicative of which subtype of stroke?

  1. Risk factors: Atrial fibrillation, endocarditis, carotid athersclerosis
  2. Abrupt symptoms onset with highest severity at onset of symptoms
A

Embolic (ischemic) stroke

Multiple infarcts within different vascular territories occur.

189
Q

What is the first-line treatment for essential tremor?

A

Propranolol

This is especially true if the patient is also hypertensive. Alternative medications include primidone or topiramate.

190
Q

Rapid onset of unilateral upper and lower facial weakness is consistent with Bell’s palsy, an acute peripheral neuropathy of cranial nerve VII with the lesion occurring where?

A

Below the pons

Lesions in the central nervous system occurring above the facial nucleus (above the pons) will typically cause a contralateral lower facial weakness that spares the forehead.

191
Q

Upper motor neuron lesions are associated with which gait abnormality?

A

Spastic gait

This occurs with spinal cord injury or cerebral palsy. The movements of the affected extremities are slow, stiff, and effortful.

192
Q

What risk factor has the highest association with a stroke?

A

Hypertension

Multiple observational studies have demonstrated that patients with hypertension have approximately four times the risk of stroke when compared to non-hypertensive subjects. Smoking and diabetes mellitus are established risk factors for strokes, but their association with strokes is not as strong as hypertension.

193
Q

The following findings are consistent with what diagnosis?

  1. Loss of pain and temperature over the ipsilateral face
  2. Loss of pain and temperature over the contralateral body
  3. Ipsilateral bulbar muscle weakness (dysphagia, horseness)
  4. Vestibulocerebellar impairment (I.E. vertigo, nystagmus)
  5. Horner’s syndrome
  6. Motor function of the face and body is typically spared
A

Lateral medullary infarct (Wallenberg syndrome)

This occurs due to occlusion of the posterior inferior cerebellar or vertebral artery.

194
Q

Elevated intraocular pressure is the hallmark of what?

A

Glaucoma

Acute glaucoma presents over days with unilateral vision changes, red eye, and headache

195
Q

Parkinsonism is associated with which gait abnormality?

A

Hypokinetic (shuffling) gait

This is typically narrow-based gait.

196
Q

What are the 3 cardinal signs of Parkinson Disease (PD)?

A
  1. Resting tremor
  2. Rigidity
  3. Bradykinesia

The presence of at least 2 of these signs suggests a clinical diagnosis of PD which can be further confirmed by physical examination. There are no imaging or laboratory tests that can be used to confirm this diagnosis with greater accuracy than physical exam.

197
Q

Gait instability, truncal ataxia, difficulty with rapid alternating movements, hypotonia, and intention tremor are all signs of what?

A

Cerebellar dysfunction

198
Q

The following findings are consistent with what diagnosis?

  1. Encephalopathy (altered mental state)
  2. Oculomotor dysfunction
  3. Gait ataxia
A

Wernickes’s encephalopathy

This is caused by thiamine deficiency. 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.

199
Q

Uncontrolled efflux of calcium from the sarcoplasmic reticulum is the underlying pathophysiology of what condition?

A

Malignant hyperthermia

This is a rare autosomal dominant disorder. It occurs in genetically susceptible patients after administration of the anesthetic drugs halothane and succinylcholine and may be associated with severe hyperthermia.

200
Q

What is the most significant complication of benign intracranial hypertension (pseudotumor cerebri)?

A

Blindness

When medical measures fail or visual field defects are progressive, shunting or optic nerve sheath fenestration may be performed to prevent this.

202
Q

The way a person conveys emotion through speech is affected by a brain lesion located where?

A

Nondominant frontal lobe

This is called motor aprosodia. Contralateral weakness and apraxia can also occur with a lesion in this location.

203
Q

Vertical diplopia and extorsion of the eye are symptoms characteristic of a lesion to which nerve?

A

Trochlear nerve

The actions of the superior oblique muscle are depression of the eyeball, especially when the eye is adducted; and intorsion of the eyeball, especially when the eye is abducted. In trochlear palsy, the affected eye drifts upward relative to the normal eye, due to the unopposed actions of the remaining extraocular muscles. The patient sees two visual fields (one from each eye), separated vertically. To compensate for this, patients learn to tilt the head forward (tuck the chin in) in order to bring the fields back together – to fuse the two images into a single visual field. Weakness of intorsion results in torsional diplopia, in which two different visual fields, tilted with respect to each other, are seen at the same time. To compensate for this, patients with trochlear nerve palsies tilt their heads to the opposite side, in order to fuse the two images into a single visual field. The characteristic appearance of patients with fourth nerve palsies (head tilted to one side, chin tucked in) suggests the diagnosis, but other causes must be ruled out. For example, torticollis can produce a similar appearance.

204
Q

What is the treatment for normal pressure hydrocephalus?

A

Large volume lumbar punctures and if successful, ventriculoperitoneal shunting

205
Q

What will the cerebrospinal fluid of a patient with bacterial meningitis show regarding the following?

  1. Protein
  2. White blood cell count (WBC)
  3. Red blood cell count (RBC)
  4. Glucose
A
  1. Protein: High
  2. WBC: High
  3. RBC: Variable
  4. Glucose: Low
206
Q

What are 4 most serious complications of heat stroke?

A
  1. Rhabdomyolysis
  2. Renal failure
  3. Acute respiratory distress syndrome (ARDS)
  4. Coagulopathic bleeding (Epistaxis)

Heat stroke presents with acute confusion, extreme hyperthermia >105 F, Tachycardia, and coagulopathic bleeding

207
Q

Paresthesias of the first three-and-a-half digits and occasionally thenar eminence atrophy are typical of what diagnosis?

A

Carpal tunnel syndrome

This is the most common mononeuropathy of the upper extremity.

209
Q

What condition presents as chronic neck pain and unilateral radiculopathy?

A

Cervical nerve root compression

210
Q

What diagnosis should be suspected in a patient with areflexic weakness in the upper extremities and dissociated anesthesia in a “cape” distribution?

A

Syringomyelia

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.

211
Q

What is the most common ring-enhancing mass lesion in HIV-infected patients?

A

Cerebral toxoplasmosis

212
Q

What is pseudodementia?

A

Memory loss in an elderly patient with depression

Pseudodementia can be easily confused with dementia; however, the diagnosis of dementia requires the presence of more symptoms. The CT scan is non-contributary, even in Alzheimer’s disease. There is no single test that readily allows the clinician to distinguish pseudodementia from dementia; however, the dexamethasone suppression test (DST) may be used clinically to detect endogenous depression. In up to 50% of patients with depression, DST will be abnormal.

214
Q

Cerebellar infarction of the medial vermis results in what symptoms?

A

Severe vertigo and nystagmus

215
Q

What diagnosis should be suspected in an HIV-infected patient with an altered mental status, EBV DNA in the cerebrospinal fluid (CSF), and a solitary, weakly ring-enhancing periventricular mass on MRI?

A

Central Nervous System lymphoma

The presence of Epstein Barr Virus DNA in the CSF is specific for this condition. MRI reveals a weakly ring-enhancing mass that is usually solitary and periventricular. Toxoplamosis is a good differential diagnosis; however, it is unlikely if the patient is receiving trimethoprim-sulfamethoxazole, and would more likely present with multiple, ring-enhancing, spherical lesions in the basal ganglia. Furthermore, a positive toxoplama serology is quite common in normal subjects in the United States, and is therefore not specific for toxoplasmosis.

216
Q

What usually produces unilateral ataxia, nystagmus, intention tremor, and loss of coordination?

A

Cerebellar tumors

The ataxia is usually ipsilateral.

217
Q

Destruction of the frontal lobe will result in eye deviation in which direction?

A

Ipsilateral deviation of the eyes to the side of the lesion

218
Q

Disruption of normal neural thermoregulatory mechanisms, resulting in neurogenic fever could follow a stroke in what region?

A

Hypothalamus

219
Q

Vestibular ataxia is associated with which gait abnormality?

A

En-bloc gait

This is characterized by minimal movements of the head during walking. It is typically a staggering gait, and is accompanied by vertigo and nystagmus.

220
Q

The following findings are consistent with a lesion occurring in what region of the brain?

  1. Unilateral motor impairment
  2. No sensory or cortical deficits
  3. No visual field abnormalities
A

Posterior limb of internal capsule

221
Q

What is the second most common cause of mass lesions in HIV-infected patients?

A

Primary CNS lymphoma

222
Q

What will the cerebrospinal fluid of a patient with viral meningitis show regarding the following?

  1. Protein
  2. White blood cell count (WBC)
  3. Red blood cell count (RBC)
  4. Glucose
A
  1. Protein: High
  2. WBC: High
  3. RBC: Normal
  4. Glucose: Normal
223
Q

The following findings are consistent with a lesion to what region of the brain/brainstem?

  1. Hemiparesis
  2. Eyes deviated away from paralytic side
  3. Hemi-sensory loss
  4. Homonymous hemianopsia
  5. Stupor
  6. Coma
A

Hemorrhage at the location of the putamen

This is the most common site of hypertensive hemorrhage.

224
Q

The resolution of muscular weakness with rest is a hallmark feature of what condition?

A

Myasthenia gravis

This is most common in women between the ages of 18 and 25. Patients typically present with intermittent dysarthria, dysphagia, ptosis, and diplopia. Generalized weakness often develops (trunk, arms, and legs) within a year of onset. Weakness tends to worsen as the day progresses.

225
Q

Insidious onset of decreased consciousness, headaches, cognitive and memory deficits, balance problems, aphasia, and motor deficits in an elderly or alcoholic patient is characteristic of what diagnosis?

A

Chronic subdural hematomas

226
Q

The cranial neuropathy most commonly found with Lyme disease is that associated with damage to which cranial nerve?

A

Cranial nerve VII

Facial weakness may be the only neurological sign of Lyme disease.

227
Q

What is the most appropriate treatment for Progressive multifocal leukoencephalopathy?

A

Highly active antiretroviral therapy (HAART)

228
Q

What is the appropriate treatment for Toxoplasma gondii infection?

A

Sulfadiazine and pyrimethamine

229
Q

What is the most common source of metastatic tumors to the brain in patients without a known primary tumor?

A

The lung

Lung, breast, kidney, skin, and uterus are all common sources of metastasis to the brain, but the lung accounts for 2/3 of cases of brain metastasis presenting without a known primary.

230
Q

Metastatic lesions to the brain most often appear in which location in the brain?

A

At the gray-white junction

231
Q

Loss of vertical gaze, loss of pupillary light reflex, lid retraction, and convergence-retraction nystagmus (in which the eyes appear to jerk back into the orbit on attempted upgaze) is characteristic of what diagnosis?

A

Parinaud syndrome

This syndrome occurs in lesions owing to involvement of the dorsal midbrain in the region of the superior colliculus. Other tumors appearing in the pineal region that can produce a similar clinical picture include germ cell tumors, teratomas, and gliomas.

232
Q

Cerebellar damage may be associated with severe vertigo if the tissue damaged is in the distribution of which artery?

A

Posterior inferior cerebellar artery (PICA)

The medial branches of PICA supply the brainstem. With occlusion of these branches, vestibular nuclei in the brainstem are infarcted, and vertigo is common. Even with an occlusion limited to the lateral branches of PICA, vertigo is likely.

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