Neurology: UWorld

Question 1

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

Answer 1

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.

Question 2

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

Answer 2

Nondominant temporal lobe

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

Question 3

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

Answer 3

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

Question 4

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

Answer 4

Alzheimer’s disease

Question 5

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.

Answer 5

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

Question 6

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

Answer 6

Optic neuritis

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

Question 7

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

Answer 7

Medial mid-pontine infarction

Question 8

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

Answer 8

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.

Question 9

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

Answer 9

Pontine hemorrhage

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

Question 10

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

Answer 10

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.

Question 11

Corticospinal tract lesions cause what?

Answer 11

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.

Question 12

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

Answer 12

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.

Question 13

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

Answer 13

Benign paroxysmal positional vertigo

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

Question 14

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

Answer 14

1. Primidone
2. Topiramate

Question 15

What is the most effective symptomatic therapy for parkinsonism?

Answer 15

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.

Question 16

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

Answer 16

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.

Question 17

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

Answer 17

Pseudodementia

Antidepressants are the treatment of choice.

Question 18

Lacunar stroke at the basis pontis will have what presentation?

Answer 18

Dysarthria-clumsy hand syndrome

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

Question 19

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

Answer 19

Familial dysautonomia (Riley-Day syndrome)

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

Question 20

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

Answer 20

Alzheimer’s disease

Question 21

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

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

Answer 21

Vertebrobasilar system (supplying the brain stem)

Question 22

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

Answer 22

Amyotrophic lateral sclerosis (ALS)

Question 23

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

Answer 23

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.

Question 24

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?

Answer 24

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.

Question 25

Sensory ataxia is associated with which gait abnormality?

Answer 25

Wide-based, high stepping gait ## Footnote *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).*

Question 26

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

Answer 26

Dementia with Lewy Bodies

Question 27

The following signs/symptoms are characteristic of what diagnosis? 1. Dizziness 2. Hearing loss 3. Tinnitus

Answer 27

Meniere's disease

Question 28

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

Answer 28

Cerebellum ## Footnote *Patients with cerebellar lesions classically walk as if they are drunk.*

Question 29

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

Answer 29

Motor symptoms ## Footnote *It occurs in 7% of patients with hypothyroidism.*

Question 30

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

Answer 30

Diabetic cranial nerve III neuropathy ## Footnote *Nerve damage is ischemic, and only somatic nerve fibers are affected. Parasympathetic fibers of CN III retain function.*

Question 31

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

Answer 31

Multiple Sclerosis ## Footnote *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.*

Question 32

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

Answer 32

Thiamine deficiency (Wernicke-Korsakoff syndrome) It is particularly common in alcoholics.

Question 33

What is the treatment of choice for pseudodementia?

Answer 33

Antidepressants ## Footnote *Suspect pseudodementia in elderly patients with dementia in the setting of severe depression.*

Question 34

Where does syringomyelia most frequently occur?

Answer 34

Lower cervical or upper thoracic spine ## Footnote *When the syringes occur in the upper cervical cord and extend proximally to involve the medulla oblongata, the condition is called syringobulbia.*

Question 35

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

Answer 35

Guillain-Barre syndrome (GBS) ## Footnote *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.*

Question 36

Blindness is a symptoms characteristic of a lesion occurring where?

Answer 36

Optic nerve

Question 37

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

Answer 37

Medial longitudinal fasciculus ## Footnote *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.*

Question 38

Which electrolyte abnormality is an important complication of subarachnoid hemorrhage?

Answer 38

Hyponatremia ## Footnote *"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).*

Question 39

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

Answer 39

Pick's disease ## Footnote *This presents with dementia and personality changes.*

Question 40

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)?

Answer 40

Middle cerebral artery occlusion

Question 41

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

Answer 41

Primidone ## Footnote *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.*

Question 42

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

Answer 42

Dominant frontal lobe ## Footnote *Broca's aphasia is an expressive aphasia.*

Question 43

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

Answer 43

Meniere disease

Question 44

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

Answer 44

AIDS dementia complex

Question 45

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

Answer 45

Memantine

Question 46

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

Answer 46

Tabes dorsalis ## Footnote *This is a manifestation of neurosyphilis.*

Question 47

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

Answer 47

Acute glaucoma ## Footnote *Drugs that dilate the pupil can precipitate glaucoma. The best diagnostic test is tonometry.*

Question 48

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)?

Answer 48

Creutzfeldt-Jakob disease ## Footnote *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.*

Question 49

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

Answer 49

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

Question 50

A stepwise cognitive deterioration is characteristic of what diagnosis?

Answer 50

Multi-infarct dementia ## Footnote *Psychiatric disturbances such as depression and agitation are common.*

Question 51

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

Answer 51

Campylobacter jejuni ## Footnote *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.*

Question 52

What is the initial treatment of choice for myasthenia gravis?

Answer 52

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. **Edrophon******ium** is a short-acting anti-cholinesterase, and is used for diagnostic purposes only.*

Question 53

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

Answer 53

A lesion in the upper thoracic spinal cord ## Footnote *A lesion in the lower thoracic spinal cord causes absent sensation from the unbilicus downwards.*

Question 54

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?

Answer 54

Multiple sclerosis ## Footnote *Cerebrospinal fluid analysis shows normal pressure and the presence of olioclonal IgG bands in \>95% of patients.*

Question 55

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

Answer 55

Hemi-neglect syndrome

Question 56

What is the treatment for heat stroke?

Answer 56

Induction of evaporative cooling to reverse hyperthermia

Question 57

What is the drug of choice for trigeminal neuralgia?

Answer 57

Carbamazepine ## Footnote *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.*

Question 58

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

Answer 58

Huntington's chorea ## Footnote *This usually presents at 40-50 years old.*

Question 59

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

Answer 59

MRI

Question 60

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

Answer 60

Protamine sulfate

Question 61

Tarsal tunnel syndrome results from entrapment of what nerve?

Answer 61

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.

Question 62

What 3 medications are recommended for prophylaxis of cluster headaches?

Answer 62

1. Verapamil 2. Lithium 3. Ergotamine ## Footnote *The treatment for an acute attack is inhalation of 100% oxygen and subcutaneous sumatriptan.*

Question 63

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

Answer 63

Syringomyelia ## Footnote *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.*

Question 64

What is the most common site of ulnar nerve entrapment?

Answer 64

The elbow ## Footnote *This is where the ulnar nerve lies at the medial epicondylar groove.*

Question 65

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

Answer 65

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.*

Question 66

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

Answer 66

Magnetic resonance imaging with magnetic resonance venography ## Footnote *Treatment includes broad-spectrum intravenous antibiotics and prevention or reversal of cerebral herniation.*

Question 67

The following findings are consistent with what diagnosis? 1. Severe headache 2. Bilateral periorbital edema 3. Cranial nerve III, IV, V, and VI deficits

Answer 67

Cavernous sinus thrombosis ## Footnote *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.*

Question 68

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)

Answer 68

Intracranial hypertension

Question 69

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

Answer 69

Anterior cerebral artery

Question 70

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

Answer 70

Huntington's chorea ## Footnote *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.*

Question 71

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

Answer 71

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

Question 72

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?

Answer 72

Pyramidal tract disease (upper motor neuron lesion) ## Footnote *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.*

Question 73

Damage to the lateral spinothalamic tracts causes what symptoms?

Answer 73

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

Question 74

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

Answer 74

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

Question 75

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

Answer 75

2 weeks after stroke ## Footnote *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.*

Question 76

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

Answer 76

Decreased cerebrospinal fluid absorption or transient increases in intracranial pressure that cause permanent ventricular enlargement without chronically increasing intracranial pressure ## Footnote *NPH is characterized clinically by dementia, gait disturbances, and incontinence.*

Question 77

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?

Answer 77

Eaton-Lambert syndrome

Question 78

Hemi-neglect syndrome is cause by what?

Answer 78

A lesion of the right (non-dominant) parietal lobe ## Footnote *The non-dominant parietal lobe is responsible for spatial organization.*

Question 79

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

Answer 79

Primary CNS lymphoma ## Footnote *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.*

Question 80

What is Amaurosis fugax a warning sign for?

Answer 80

Impending stroke ## Footnote *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.*

Question 81

Where is the most common site for a lacunar infarct?

Answer 81

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

Question 82

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

Answer 82

Endotracheal intubation an withdrawal of anticholinesterases for several days ## Footnote *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.*

Question 83

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

Answer 83

Acute angle-closure glaucoma

Question 84

What participates in the initiation and control of movement?

Answer 84

Basal ganglia

Question 85

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

Answer 85

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* * .*

Question 86

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

Answer 86

Within 3-4.5 hours of symptom onset ## Footnote *Thrombolytics are used in nonhemorrhagic ischemic strokes.*

Question 87

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

Answer 87

Typical and atypical antipsychotics ## Footnote *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).*

Question 88

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

Answer 88

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.*

Question 89

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

Answer 89

Normal pressure hydrocephalus ## Footnote *Patients tend to be bradykinetic.*

Question 90

What are the 3 most common causes of foot drop?

Answer 90

1. Neuropathy 2. Radiculopathy 3. Traumatic damage to the common peroneal nerve ## Footnote *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.*

Question 91

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

Answer 91

Pure motor hemiparesis ## Footnote *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.*

Question 92

What is the prognosis for progressive multifocal leukoencephalopathy?

Answer 92

The mean duration of survival from the time of diagnosis is 6 months *There is no effective treatment for PML*

Question 93

Lateral cerebellar infarction results in what symptoms?

Answer 93

Dizziness, ataxia, and weakness

Question 94

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

Answer 94

Aspirin ## Footnote *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.*

Question 95

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

Answer 95

Posterior inferior cerebellar artery or Vertebral artery

Question 96

What is the principle cause of a lacunar stroke?

Answer 96

Hypertension ## Footnote *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.*

Question 97

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

Answer 97

Dementia with Lewy bodies ## Footnote *Symptoms worsen with neuroleptic drugs.*

Question 98

The following bulbar symptoms are consistent with what diagnosis? 1. Dysphagia 2. Hoarseness 3. Diminished gag reflex

Answer 98

Lateral medullary infarct (Wallenberg syndrome) ## Footnote *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.*

Question 99

Most cases of syringomyelia occur in association with what?

Answer 99

Arnold-chiari malformation ## Footnote *This is caudal displacement of cerebellar tonsils through the foramen magnum. In such patients, neuroimaging may show caudal displacement of the fourth ventrical.*

Question 100

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

Answer 100

Multiple sclerosis (MS) *MS is one of the few conditions that may present with bilateral trigeminal neuralgia.*

Question 101

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

Answer 101

Essential tremor ## Footnote *Affected patients typically have difficulty holding a newspaper, writing legibly, drinking a cup of coffee, or feeding themselves.*

Question 102

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

Answer 102

Vertebral artery or Anterior spinal artery

Question 103

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

Answer 103

Amantadine ## Footnote *Ankle edema is another side effect of amantadine.*

Question 104

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

Answer 104

Dominant temporal lobe ## Footnote *Wernicke's aphasia is a receptive aphasia.*

Question 105

The following signs/symptoms are characteristic of what diagnosis? Personality change (euphoria, disinhibition, apathy) Compulsive behaviors (Peculiar eating habits) Hyperorality Impaired memory

Answer 105

Pick's disease (fronto-temporal dementia) ## Footnote *Visual and spatial functions are usually intact.*

Question 106

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

Answer 106

Pick's disease ## Footnote *It is seen more frequently in females, and frequently causes personality changes due to involvement of the frontal lobes.*

Question 107

Which nerve controls corneal sensation?

Answer 107

The ophthalmic branch (V1) of the trigeminal nerve (CN V) ## Footnote *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.*

Question 108

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

Answer 108

Lacunar stroke ## Footnote *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.*

Question 109

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

Answer 109

Magnetic resonance imaging (MRI) ## Footnote *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.*

Question 110

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

Answer 110

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.*

Question 111

A lesion in the posterior column would cause what?

Answer 111

Ataxia

Question 112

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

Answer 112

Lateral cerebellar infarction ## Footnote *The tendency is to sway towards the side of the lesion.*

Question 113

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

Answer 113

Wilson's disease

Question 114

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

Answer 114

Cluster headache ## Footnote *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.*

Question 115

Intracranial hypertension is defined as intracranial pressure of what?

Answer 115

_\>_20 mmHg

Question 116

Lesions of the lateral cerebellar hemisphere present with what?

Answer 116

Dizziness and ipsilateral ataxia

Question 117

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

Answer 117

Aspirin ## Footnote *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.*

Question 118

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

Answer 118

Peripheral nerve conduction study

Question 119

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

Answer 119

An upper motor neuron lesion

Question 120

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

Answer 120

Cortical laminar necrosis ## Footnote *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.*

Question 121

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

Answer 121

Abducens nerve

Question 122

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

Answer 122

Alpha-synuclein

Question 123

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

Answer 123

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

Question 124

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

Answer 124

Multiple system atrophy (Shy-Drager syndrome) ## Footnote *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.*

Question 125

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

Answer 125

Posterior cerebral artery

Question 126

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

Answer 126

Aseptic (viral) meningitis ## Footnote *This usually occurs in the summer months, and is caused by an echovirus. Treatment is supportive care.*

Question 127

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

Answer 127

Cluster headaches

Question 128

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

Answer 128

100% oxygen ## Footnote *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.*

Question 129

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

Answer 129

Halothane and succinylcholine

Question 130

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

Answer 130

An antecedent respiratory tract or gastrointestinal (especially Campylobacter) infection

Question 131

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

Answer 131

Magnetic resonance imaging (MRI) with gadolinium enhancement. ## Footnote *Such a patient likely has neurofibromatosis type II. MRI with gadolinium is enhancement is preferred over computed tomography (CT).*

Question 132

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

Answer 132

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.*

Question 133

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

Answer 133

Subacute sclerosing panencephalitis

Question 134

What is the best diagnostic test for suspected acute glaucoma?

Answer 134

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

Question 135

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

Answer 135

Clopidogrel ## Footnote *It is not recommended for aspirin-naive patients presenting with stroke.*

Question 136

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

Answer 136

Middle cerebral artery ## Footnote *Aphasia occurs when lesion is in dominant hemisphere. Hemineglect occurs when lesion is in nondominant hemisphere.*

Question 137

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?

Answer 137

Brain tumor

Question 138

What are 5 risk factors for Alzheimer's disease?

Answer 138

1. Older age 2. Female gender 3. Positive family history 4. Dead trauma 5. Down's dyndrome ## Footnote *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.*

Question 139

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

Answer 139

Vitamin B₁₂ deficiency ## Footnote *The most common cause is pernicious anemia.*

Question 140

The following characteristics are indicative of which subtype of stroke? 1. Severe headache at onset of neurological symptoms 2. Neck stiffness

Answer 140

Spontaneous subarachnoid hemorrhage ## Footnote *Rupture of an arterial saccular "berry" aneurysm or from an arteriovenous malformation. Focal deficits are uncommon.*

Question 141

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

Answer 141

Temporal arteritis ## Footnote *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.*

Question 142

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

Answer 142

Computed tomography (CT) scan ## Footnote *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.*

Question 143

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

Answer 143

Spinal epidural abscess ## Footnote *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.*

Question 144

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

Answer 144

Lateral spinothalamic tract

Question 145

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

Answer 145

Steroids ## Footnote *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.*

Question 146

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

Answer 146

Extrapyramidal symptoms ## Footnote *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.*

Question 147

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

Answer 147

Muscular dystrophy

Question 148

Upper and lower motor neuron degeneration suggests what diagnosis?

Answer 148

Amyotrophic lateral sclerosis (ALS)

Question 149

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

Answer 149

Fronto-temporal dementia (Pick's disease) ## Footnote *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).*

Question 150

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

Answer 150

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

Question 151

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

Answer 151

Discontinuation of the medication and administration of benztropine or diphenhydramine

Question 152

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

Answer 152

Medial medullary syndrome (alternating hypoglossal hemiplegia) ## Footnote *This typically occurs due to branch occlusion of the vertebral or anterior spinal artery.*

Question 153

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

Answer 153

Cerebellar hemorrhage ## Footnote *Other manifestations are 6th nerve paralysis, conjugate deviation, blepharospasm, and coma.*

Question 154

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?

Answer 154

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

Question 155

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

Answer 155

Intracerebral hemorrhagic stroke

Question 156

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

Answer 156

Guillain-Barre syndrome (GBS) *GBS is an acute idiopathic inflammatory demyelinating polyneruopathy.*

Question 157

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

Answer 157

Electroencephalography (EEG)

Question 158

Idiopathic intracranial hypertension is caused by what mechanism?

Answer 158

Impaired cerebral spinal fluid absorption or excess production ## Footnote *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.*

Question 159

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

Answer 159

Lateral mid-pontine lesion ## Footnote *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.*

Question 160

The following findings are consistent with what diagnosis? 1. Gait disturbance 2. Dementia 3. Urinary incontinence

Answer 160

Normal pressure hydrocephalus ## Footnote *Lumbar puncture reveals the normal CSF pressures, and MRI shows the enlarged ventricles.*

Question 161

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

Answer 161

Pure sensory deficits ## Footnote *It will present with unilateral numbness, paresthesias, and hemisensory deficit involving the face, arm, trunk, and leg.*

Question 162

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

Answer 162

The MRI would show lesions that are usually multiple, spherical, and located in the basal ganglia ## Footnote *This diagnosis is less likely if the patient is receiving Trimethoprim-Sulfamethoxazole (TMP-SMX)*

Question 163

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

Answer 163

Huntington's disease ## Footnote *This is an autosomal dominant neurodegenerative disease affecting the caudate and putamen. The mean age of onset is between 35-44 years of age.*

Question 164

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

Answer 164

Idiopathic intracranial hypertension (pseudotumor cerebri) ## Footnote *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.*

Question 165

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

Answer 165

Carotid doppler and transesophageal echocardiogram ## Footnote *These are performed to locate a possible source of embolism.*

Question 166

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

Answer 166

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

Question 167

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

Answer 167

Primary CNS lymphoma

Question 168

Tabes dorsalis is a manifestation of what condition?

Answer 168

Neurosyphilis ## Footnote *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.*

Question 169

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

Answer 169

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

Question 170

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

Answer 170

Occulomotor nerve

Question 171

The following signs/symptoms are characteristic of what diagnosis? 1. Dementia 2. Gait ataxia 3. Urinary incontinence

Answer 171

Normal Pressure Hydrocephalus

Question 172

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

Answer 172

Upper motor neuron damage affecting the upper extremities ## Footnote *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.*

Question 173

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

Answer 173

Optic chiasm

Question 174

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

Answer 174

Gentamicin ## Footnote *Aminoglycosides can cause nephrotoxicity and ototoxicity.*

Question 175

What is the study of choice in diagnosing multiple sclerosis?

Answer 175

Magnetic resonance imaging (MRI) ## Footnote *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.*

Question 176

The following signs symptoms are characteristic of what diagnosis? 1. Ataxia 2. Vomiting 3. Occipital headache 4. Gaze palsy 5. Facial weakness

Answer 176

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

Question 177

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

Answer 177

Optic radiation

Question 178

A lateral frontal cortex lesion results in what symptoms?

Answer 178

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

Question 179

What is the most common site of hypertensive hemorrhage?

Answer 179

The putamen ## Footnote *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.*

Question 180

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

Answer 180

Amaurosis fugax ## Footnote *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.*

Question 181

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

Answer 181

Cauda equina syndrome ## Footnote *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.*

Question 182

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

Answer 182

Severe vertigo and nystagmus

Question 183

What is the treatment for restless leg syndrome?

Answer 183

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).

Question 184

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

Answer 184

Tick-borne paralysis ## Footnote *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.*

Question 185

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

Answer 185

Thrombotic (ischemic) stroke ## Footnote *Local in-situ obstruction of an artery occurs.*

Question 186

Diabetic mononeuropathy often involves which cranial nerve?

Answer 186

Cranial nerve III ## Footnote *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.*

Question 187

Muscular dystrophy is associated with which gait abnormality?

Answer 187

Waddling gait ## Footnote *This occurs because of weakness of the gluteal muscles.*

Question 188

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

Answer 188

Embolic (ischemic) stroke ## Footnote *Multiple infarcts within different vascular territories occur.*

Question 189

What is the first-line treatment for essential tremor?

Answer 189

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

Question 190

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?

Answer 190

Below the pons ## Footnote *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.*

Question 191

Upper motor neuron lesions are associated with which gait abnormality?

Answer 191

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

Question 192

What risk factor has the highest association with a stroke?

Answer 192

Hypertension ## Footnote *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.*

Question 193

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

Answer 193

Lateral medullary infarct (Wallenberg syndrome) ## Footnote *This occurs due to occlusion of the posterior inferior cerebellar or vertebral artery.*

Question 194

Elevated intraocular pressure is the hallmark of what?

Answer 194

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

Question 195

Parkinsonism is associated with which gait abnormality?

Answer 195

Hypokinetic (shuffling) gait ## Footnote *This is typically narrow-based gait.*

Question 196

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

Answer 196

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_.*

Question 197

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

Answer 197

Cerebellar dysfunction

Question 198

The following findings are consistent with what diagnosis? 1. Encephalopathy (altered mental state) 2. Oculomotor dysfunction 3. Gait ataxia

Answer 198

Wernickes's encephalopathy ## Footnote *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.*

Question 199

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

Answer 199

Malignant hyperthermia ## Footnote *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.*

Question 200

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

Answer 200

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

Question 201

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

Answer 201

Lumbar puncture ## Footnote *Oligoclonal bands are characteristic of Multiple Sclerosis.*

Question 202

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

Answer 202

Nondominant frontal lobe ## Footnote *This is called motor aprosodia. Contralateral weakness and apraxia can also occur with a lesion in this location.*

Question 203

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

Answer 203

Trochlear nerve ## Footnote *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.*

Question 204

What is the treatment for normal pressure hydrocephalus?

Answer 204

Large volume lumbar punctures and if successful, ventriculoperitoneal shunting

Question 205

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

Answer 205

1. Protein: High 2. WBC: High 3. RBC: Variable 4. Glucose: Low

Question 206

What are 4 most serious complications of heat stroke?

Answer 206

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*

Question 207

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

Answer 207

Carpal tunnel syndrome ## Footnote *This is the most common mononeuropathy of the upper extremity.*

Question 208

Herpes mainly affects which lobe of the brain?

Answer 208

Temporal lobe ## Footnote *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.*

Question 209

What condition presents as chronic neck pain and unilateral radiculopathy?

Answer 209

Cervical nerve root compression

Question 210

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

Answer 210

Syringomyelia ## Footnote *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.*

Question 211

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

Answer 211

Cerebral toxoplasmosis

Question 212

What is pseudodementia?

Answer 212

Memory loss in an elderly patient with depression ## Footnote *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.*

Question 213

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

Answer 213

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

Question 214

Cerebellar infarction of the medial vermis results in what symptoms?

Answer 214

Severe vertigo and nystagmus

Question 215

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?

Answer 215

Central Nervous System lymphoma ## Footnote *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.*

Question 216

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

Answer 216

Cerebellar tumors ## Footnote *The ataxia is usually ipsilateral.*

Question 217

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

Answer 217

Ipsilateral deviation of the eyes to the side of the lesion

Question 218

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

Answer 218

Hypothalamus

Question 219

Vestibular ataxia is associated with which gait abnormality?

Answer 219

En-bloc gait ## Footnote *This is characterized by minimal movements of the head during walking. It is typically a staggering gait, and is accompanied by vertigo and nystagmus.*

Question 220

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

Answer 220

Posterior limb of internal capsule

Question 221

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

Answer 221

Primary CNS lymphoma

Question 222

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

Answer 222

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

Question 223

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

Answer 223

Hemorrhage at the location of the putamen ## Footnote *This is the most common site of hypertensive hemorrhage.*

Question 224

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

Answer 224

Myasthenia gravis ## Footnote *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.*

Question 225

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?

Answer 225

Chronic subdural hematomas

Question 226

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

Answer 226

Cranial nerve VII ## Footnote *Facial weakness may be the only neurological sign of Lyme disease.*

Question 227

What is the most appropriate treatment for Progressive multifocal leukoencephalopathy?

Answer 227

Highly active antiretroviral therapy (HAART)

Question 228

What is the appropriate treatment for Toxoplasma gondii infection?

Answer 228

Sulfadiazine and pyrimethamine

Question 229

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

Answer 229

The lung ## Footnote *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.*

Question 230

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

Answer 230

At the gray-white junction

Question 231

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?

Answer 231

Parinaud syndrome ## Footnote *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.*

Question 232

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

Answer 232

Posterior inferior cerebellar artery (PICA) ## Footnote *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.*

Question 233

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

Answer 233

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

Question 234

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

Answer 234

Cortical white matter *The brainstem and cerebellum can be involved too*.