Life Long Learning in Neurology - part 7 pps

Patients whose dystonia responds to an antidopaminergic drug, such as risperidone, are unlikely to respond to levodopa which, in any case, is effective for only a small fraction of patie

E 15%

Correct Answer: The correct answer is B In the Spanish study referred to (Sempere et al, 2005), a large group of almost 1900 patients was studied, over 99% of whom had normal neurological examinations Imaging studies (primarily CT, although about one third of patients had MRI) detected "significant lesions" in 1.2% of patients; however, of the patients who had normal neurological examinations, the rate of "significant intracranial abnormalities" was 0.9%

Correct Answer: Sempere AP, Porta-Etessam J, Medrano V, et al Neuroimaging in the evaluation of patients with non-acute headache Cephalalgia 2005;25:30-35

29 The risk of stroke in patients with a history of migraine, although debated in several large epidemiological studies, is felt to be higher than that of nonmigraineurs Among women with migraine with aura who are under age

45 and smoke, use of which of the following substances is felt to further increase the risk of stroke?

A Beta-blockers

B Ergotamine

C Opioids

D Oral contraceptives

E Triptans

Correct Answer: The correct answer is D Several large epidemiological studies regarding the relative risk of stroke

in patients with a history of migraine have been undertaken, not all of which have shown consistent results However, the data overall suggest that women under age 45 who smoke, use oral contraceptives, and have a history

of migraine with aura have the highest relative risk of stroke among migraine patients and a significantly higher risk than those without migraine Ergotamine has no known association to migraine-related stroke, and there is no reason to expect this with opioids Beta-blockers are used for migraine prevention

30 A 45-year-old male engineer is seen in an urgent care clinic because of a severe headache that began when he awakened that day He reports that 2 to 3 times a year he experiences a throbbing headache, typically on the right side Those headaches are associated with nausea, vomiting, and prostration; he feels that they may be caused by stress He takes an over-the-counter tablet comprised of aspirin, acetaminophen, and caffeine on average 2 times a week for more minor headaches On this occasion, he had felt well until 2 days before when he was diagnosed with gastroenteritis, which caused him to stop everything by mouth and stop smoking for 1½ days, as he had multiple episodes of vomiting and diarrhea His medical history is significant for hypertension, treated with atenolol He smokes half a pack of cigarettes daily, drinks 6 to 8 cups of coffee daily, and drinks wine with dinner On physical examination, vital signs are: blood pressure 120/70, pulse 80, respiration 15, afebrile He is not orthostatic He prefers to lie still in a dark room Pupils, fundi, and visual fields are normal A noncontrast CT of the brain is normal There is no nuchal rigidity He is treated with intravenous metoclopramide and meperidine with relief of his symptoms Withdrawal from which of the following substances is the most likely cause of this patient's headaches?

A Acetaminophen

B Alcohol

C Atenolol

D Caffeine

E Nicotine

Correct Answer: The correct answer is D This patient does not appear to be overusing his over-the-counter medication (acetaminophen) and does not present with other signs consistent with withdrawal from heavy alcohol use Withdrawal from a beta-blocker (atenolol) might cause a spike in blood pressure or elevated pulse rate, but these are not seen Nicotine withdrawal presumably could be tolerated and not lead to a migraine-type headache However, the patient's caffeine use is substantial and abruptly curtailed by his illness Of the substances presented, this would be the most likely cause of his acute headache

31 In a short-duration study of prevalence of risk factors precipitating migraine, several categories of risk factors were studied Of those considered, which was the most likely to be associated with the onset of a migraine?

A Change in weather

B Food triggers

C Menses

D Psychological stress

E Sleep disturbances

Correct Answer: The correct answer is E A 3-month study by patient diary of prevalence of headache-associated risk factors among migraineurs found sleep disturbances and fatigue to be the leading risk factor by a large margin, followed by psychological stress, change in weather, food triggers, and menses Interestingly, a counterargument

has been advanced by some clinicians that the items identified as risk factors in some situations may represent a

prodrome of a headache waiting to happen rather than a precipitant

32 A 44-year-old woman treats her migraine attacks successfully with an oral triptan However, at least once

yearly, she has an attack that is unresponsive to her oral triptans, either because she awakens with the attack or

because she fails to take the oral medication early and misses her "window." The migraine then builds to vomiting,

and she ends up going to an emergency department What home treatments might keep her out of the emergency

department and give her relief?

A Butalbital, aspirin, caffeine, and codeine

B Celecoxib 200 mg repeated 2 times daily

C Hydromorphone suppository

D Intranasal butorphanol

E Subcutaneous sumatriptan and promethazine suppository

Correct Answer: The correct answer is E Opioids should be avoided both at home and in the emergency

department for rescue, and oral COX-2 inhibitors would be inadequate for rescue Optimal treatments for rescue

include parenteral sumatriptan, suppository neuroleptics, self-injected dihydroergotamine, or oral steroids if the

patient can take by mouth

33 A 50-year-old man comes to the emergency department (ED) 1 day after the onset of a very severe headache

He has been in good health and is treated only for hyperlipidemia with a statin medication One day ago, he was out

jogging when he experienced a lightninglike headache that caused him to vomit He attributed this to dehydration

and returned home where he drank a lot of fluids and rested His headache persisted, however, and he noted pain

upon flexing his neck In the ED, vital signs are within normal limits except for a temperature of 38°C He is lying

in a dark room and is reluctant to move Flexion at the neck is resisted, and he grimaces No other focal signs are

found on neurological examination ED personnel are concerned about subarachnoid hemorrhage and order a

noncontrast CT scan of the brain What is the approximate probability of detecting subarachnoid blood in this

patient at this time if he actually had experienced a subarachnoid hemorrhage?

A 30%

B 50%

C Between 50% and 75%

D Between 75% and 95%

E 99%

Correct Answer: The correct answer is D Several series involving detection rates for subarachnoid hemorrhage by

CT are reviewed by the author, including a large series of over 3500 patients (1990) and a smaller prospective

series of 175 patients (1995) Consistently, the detection rate of subarachnoid hemorrhage by CT on the day of the

ictus was around 95% This number drops substantially to 50% after the first week; the percentage of normal CTs

by day 1 almost doubled in the large series above Per Table 11-10, the correct choice is between 75% and 95%

34 The patient in question 33 undergoes a noncontrast CT scan in the emergency department, which reveals acute

hemorrhage in the perimesencephalic region His physical examination is repeated and again shows a stiff neck,

photophobia, and no localizing neurological signs He is sent for four-vessel digital subtraction angiography, which

does not reveal an aneurysm The patient is told that in about 15% of patients with subarachnoid hemorrhage, no

reason is found for the bleed Of these patients, approximately what percentage demonstrates the pathology

described on this patient's CT scan?

A Less than 1%

B 5%

C 10%

D 30%

E 50%

Correct Answer: The correct answer is E Perimesencephalic subarachnoid hemorrhage is felt possibly to be due to

a vein rupture and is cited as the leading cause of angiogram-negative subarachnoid hemorrhage

TYPE R QUESTIONS (EXTENDED MATCHING)

Theme: Pathophysiology of migraine

A Acetylcholine

B Butorphanol

C Calcitonin-gene related peptide (CGRP)

D Dopamine

E Sumatriptan

For each of the below descriptions of the role of a medication or neurotransmitter in the pathophysiology or treatment of migraine select the most likely medication or neurotransmitter from the list above

35 Stimulation of the trigeminal ganglion produces release of this substance by nerve fibers innervating cerebral vessels

Correct Answer: The correct answer is C CGRP, substance P, and vasoactive intestinal polypeptide have all been shown in experimental models to be released after stimulation of the trigeminal ganglion At least for CGRP there seems a clear role in migraine since CGRP receptor blockers are effective acute attack therapies These neurotransmitters are thought to produce vasodilatation and a sterile inflammatory response around cerebral vessels

36 Influences the CGRP promoter and regulates CGRP secretion from neurons

Correct Answer: The correct answer is E Sumatriptan has multiple mechanisms for production of analgesia in migraine One mechanism may be regulation of CGRP secretions via 5-hydroxytryptamine (5-HT [serotonin])1B and 5-HT1D receptors CGRP appears to be elevated in the headache phase of migraine

37 Blocks neurogenic plasma extravasation

Correct Answer: The correct answer is E Sterile neurogenic inflammation appears to play a role in the pain of migraine and is associated with plasma extravasation from trigeminal-innervated cerebral vessels Plasma extravasation is blocked by ergots, indomethacin, acetylsalicylic acid, and 5HT1B/1D agonists, including sumatriptan

Theme: International Classification of Headache Disorders 2nd Edition (ICHD-II) Classifications

A Chronic migraine (1.5.1)

B Chronic tension-type headache (2.3)

C Episodic cluster headache (3.1.1)

D Hypnic headache (4.5)

E Medication overuse headache (8.2)

F Migraine without aura (1.1)

G Paroxysmal hemicrania (3.2)

H Primary stabbing headache (4.1)

I Short-duration, unilateral, neuralgic, conjunctival injection and tearing headache (SUNCT) (3.3)

For each of the following clinical cases select the most appropriate ICHD-II classification for the patient's current headache problem

38 A 40-year-old woman reports a 20-year history of headache Initially these headaches were episodic (usually perimenstrual), unilateral, throbbing, and associated with nausea and photophobia Over the past 5 years they have become more frequent (4 to 5 days each week), holocephalic, nonthrobbing, and unassociated with nausea or photophobia Currently she is taking 6 to 8 tablets of acetaminophen or a tablet combining acetaminophen, aspirin, and caffeine Her current headaches are not associated with exertion or Valsalva The neurological examination is normal, and a CT scan of the head is unremarkable The patient's headache frequency is markedly reduced 2 weeks after discontinuing over-the-counter medications

Correct Answer: The correct answer is E This patient has probable medication overuse headache (rebound headache) The diagnosis requires that the headache be present for greater than 15 days per month and occur in conjunction with chronic (usually daily) use of analgesic medications These headaches typically occur in patients with preexisting headache syndromes, particularly migraine The diagnosis at this time is "probable" medication overuse headache A definite diagnosis of this headache type requires that the headache resolve within 2 months of discontinuance of the responsible analgesic medication

39 A 50-year-old woman has had recurrent episodic right-sided headaches for the past 4 months She has no previous history of headache Her current headache comes on suddenly and is usually centered over the right frontotemporal or orbital region Each headache lasts only 5 to 10 minutes but may recur as many as 20 times per day Associated with the headache, she notes tearing and conjunctival injection of the right eye Her headaches have been prevented by indomethacin, although her internist has advised that she not take this medication due to reduced renal function The neurological examination and MRI of the head are normal

Correct Answer: The correct answer is G The clinical features suggest paroxysmal hemicrania These headaches usually have their onset in adulthood and are more common in women They are characterized by brief (2 to 30 minutes) one-sided headaches usually located around the eye or in the temporal region Like cluster headache, they are often associated with lacrimation, rhinorrhea, miosis, or ptosis The headaches are shorter lasting and more frequent than cluster headache The diagnosis of paroxysmal hemicrania requires an absolute response to indomethacin

40 A 39-year-old man reports a 1-year history of sharp, stabbing pain behind or above the left eye lasting 5 to 10 seconds and recurring multiple times per day The patient estimates that he has had as many as 100 attacks on a

given day, and he has never gone more than a week without a bout of these head pains He notes tearing and

redness of the left eye associated with the headache The pain is not triggered by eating, touching his face, or

putting in his contacts His neurological examination is normal as is neuroimaging

Correct Answer: The correct answer is I This patient likely has short duration unilateral, neuralgic, conjunctival

injection and tearing (SUNCT) The syndrome is characterized by very brief (5 to 240 seconds) stabbing pain in the

orbital or temporal region with tearing and redness of the ipsilateral eye The attacks are much briefer than those

seen in any other trigeminal autonomic cephalalgia The headaches typically occur multiple times per day The pain

is unilateral and is usually not triggered by touching the face, eating, drinking, or talking, as in trigeminal neuralgia

Causes of secondary headache must be ruled out with clinical examination and neuroimaging Lesions of the

posterior fossa and pituitary gland have been reported to mimic SUNCT

Movement disorder Feb 2007

TYPE A QUESTIONS (ONE BEST ANSWER)

1 An 11-year-old boy comes to the office with his parents because of a 14-month history of motor and vocal tics,

which currently include eye blinking and barking sounds; when his symptoms began he had other motor tics,

including shoulder shrugging and forehead raising Although his tics can vary in severity, they occur frequently

during the day, and he has never been tic free for more than a few weeks during the past year He has no other past

medical history and takes no medication Other than his tics, his neurological examination is normal Which of the

following is the most appropriate description of this patient's symptoms?

A Chronic tic disorder

B Compulsions

C Stereotypies

D Tourette syndrome

E Transient tic disorder

Correct Answer: The correct answer is D This patient meets criteria for Tourette syndrome, defined by a

combination of multiple motor tics with at least one vocal tic, lasting at least 1 year Transient tic disorders are tics

that are present for more than 1 month but less than 1 year Chronic motor or vocal tic disorders are defined by tics

that are present for more than 1 year, but there may be periods of up to 3 months without tics Unlike tics,

compulsions involve the accomplishment of a specific goal, usually associated with a sense of worry or magical

thinking, and typically do not require a particular form of the movement to occur Stereotypies, which can be a

feature of normal behavior, include behaviors such as shaking-shuddering spells, repetitive flapping or twisting arm

movements, or leg kicking

2 A 9-year-old girl with generalized involuntary movements first became symptomatic at age 5, when she began to

experience inversion of the left foot while running This progressed over the following year to the point that any

movement of the left leg would precipitate foot inversion She then began to develop the same symptoms in her

right foot Over the next 3 years, the abnormal movements spread to involve her trunk, neck, and left arm, and she

became confined to a wheelchair Attempts to stand exacerbated her trunk spasms DYT-1 testing was positive She

did not tolerate trihexyphenidyl, baclofen, or risperidone, although her symptoms responded to each of these

medications Which of the following treatment approaches is most likely to be effective for her widespread

movements?

A Botulinum toxin

B Clonidine

C Deep brain stimulation

D Levodopa

E Physical therapy

Correct Answer: The correct answer is C Deep brain stimulation is indicated in patients with primary generalized

dystonia if they have not responded to or tolerated medical therapy Patients whose dystonia responds to an

antidopaminergic drug, such as risperidone, are unlikely to respond to levodopa (which, in any case, is effective for

only a small fraction of patients with dystonia) Clonidine does not appear to be effective in patients with

generalized dystonia Botulinum toxin may be useful in treating a particular area of the body that is severely

affected, but widespread injections in multiple regions are not feasible Physical therapy has been reported to be

helpful primarily in patients with focal dystonia

3 A 12-year-old boy with Tourette syndrome comes to the office because of abnormal movements that began 2

days ago He had been taking pimozide for the past year; because of significant improvement in his tics, pimozide

was discontinued 1 week ago He is afebrile with normal vital signs Examination shows moderate diffuse chorea

of his arms and legs Which of the following is the most likely explanation for his current symptoms?

A Acute dystonic reaction

B Neuroleptic malignant syndrome

C Tardive dyskinesia

D Tourette exacerbation

E Withdrawal emergent syndrome

Correct Answer: The correct answer is E The withdrawal emergent syndrome is a self-limited chorea that occurs with rapid withdrawal of antidopaminergic therapy in patients who do not have tardive dyskinesia The coincidence

of the acute occurrence of his chorea upon discontinuation of dopamine receptor blocker therapy makes tardive chorea less likely His symptoms are not suggestive of tic recurrence His symptoms are also not consistent with an acute dystonic reaction or the neuroleptic malignant syndrome; in addition, these syndromes are associated with use

of neuroleptics rather than their discontinuation

4 A 70-year-old man with Parkinson's disease has grown progressively more demented over the past year, and in the last 2 months he has been experiencing increasingly frequent hallucinations that make him very agitated and even violent His only medication is carbidopa/levodopa, and he has been unable to tolerate all attempts to reduce the dose Which of the following medications would be the best choice for treating his hallucinations?

A Chlorpromazine

B Donepezil

C Haloperidol

D Olanzapine

E Quetiapine

Correct Answer: The correct answer is E Quetiapine and clozapine are the two antipsychotic medications most likely to reduce hallucinations without worsening motor features of Parkinson's disease Of the two, quetiapine is usually preferred because clozapine requires frequent blood monitoring

5 A 62-year-old woman calls the office with the complaint of nausea, vomiting, and unsteadiness that began 2 hours after taking her first dose of primidone (25 mg), which was prescribed for her essential tremor She has a history of hypertension for which she takes enalapril Which of the following is the most likely explanation for her symptoms?

A Acute adverse reaction to primidone

B High blood levels of 2-ethyl-2-phenylmalonamide (PEMA)

C High blood levels of phenobarbital

D Histamine-mediated allergy to primidone

E Interaction between enalapril and primidone

Correct Answer: The correct answer is A Approximately 20% of patients starting primidone are bothered by an acute adverse reaction consisting of nausea, vomiting, or ataxia, which may require discontinuation of the drug Toxicity due to increased blood levels of phenobarbital or PEMA, metabolites of primidone, is unlikely since this patient was given an appropriately very low initial starting dose of primidone Her symptoms are not suggestive of medication allergy

6 A 54-year-old woman has been noted to have abnormal movements of her arms and inability to stand for the last week Nine days ago she had a cardiopulmonary arrest related to a severe asthma attack; she was intubated and mechanically ventilated for 2 days On examination she is awake and alert, without obvious cognitive deficit She has severe action and intention myoclonus when attempting to reach for or hold an object or when performing the finger-to-nose maneuver She is unable to stand without her legs buckling A trial of clonazepam is only mildly effective A trial of which of the following medications would be most appropriate?

A Carbamazepine

B Carbidopa/levodopa

C Levetiracetam

D Phenytoin

E Pramipexole

Correct Answer: The correct answer is C This patient has posthypoxic myoclonus (Lance-Adams syndrome) Patients with posthypoxic myoclonus may require a combination of medications to treat this disabling condition In addition to clonazepam, other medications that have been found to be potentially effective include levetiracetam, valproic acid (not listed), and zonisamide (not listed) The other medications listed above are not useful for posthypoxic myoclonus

7 Which of the following blood tests should be monitored in patients who have recently started taking tolcapone?

A Creatinine

B Glucose

C Liver enzymes

D Platelet count

E White blood cell count

Correct Answer: The correct answer is C Because of the rare but potentially fatal side effect of hepatic failure, the

use of tolcapone is reserved for patients whose Parkinson's disease cannot be adequately controlled with other

medications Written informed consent should be obtained before starting tolcapone, and liver enzymes should be

monitored every 2 to 4 weeks for the first 6 months of therapy and periodically thereafter

8 Deep brain stimulation (DBS) of the globus pallidus interna (GPi) may be more effective than DBS of the

subthalamic nucleus (STN) in treating which of the following features of Parkinson's disease?

A Bradykinesia

B Dementia

C Dyskinesias

D On-off fluctuations

E Tremor

Correct Answer: The correct answer is C GPi and STN are the preferred targets for DBS in patients with

Parkinson's disease Stimulation at either site can improve parkinsonian symptoms (including tremor, bradykinesia,

rigidity, gait disturbance, and postural instability) and reduce on-off fluctuations, dyskinesias, and dystonia

According to some studies, STN stimulation produced slightly greater improvement in motor scores, tremor, and

bradykinesia, although other studies found no significant differences between the two target sites in treating these

symptoms Dyskinesia management may be slightly superior with GPi stimulation Dementia does not respond to

stimulation at either site

9 Which of the following is most likely to be a side effect of pramipexole?

A Hepatic failure

B Pathological gambling

C Retroperitoneal fibrosis

D Serotonin syndrome

E Valvular heart disease

Correct Answer: The correct answer is B Pathological gambling may be associated with dopamine agonists,

especially pramipexole, usually at high doses Patients taking pergolide or other ergot alkaloids have an increased

incidence of valvular heart disease, retroperitoneal fibrosis, and pleural fibrosis Hepatic failure is a rare but

potentially fatal complication of tolcapone use Serotonin syndrome can occur in patients taking serotonin selective

reuptake inhibitors together with selegiline or other inhibitors of monoamine oxidase

10 A 68-year-old woman comes to the office because of a 6-year history of arm and voice tremors that have been

worsening over the past 2 years She is particularly bothered by her extremity tremors, which are embarrassing for

her and impair her writing and eating She has type 2 diabetes, for which she takes insulin Examination shows a

moderately severe postural and action tremor in the upper extremities as well as a mild head and voice tremor No

other abnormalities are seen, and gait is normal Which of the following is the most appropriate treatment for her

tremors?

A Botulinum toxin

B Carbidopa/levodopa

C Pramipexole

D Primidone

E Propranolol

Correct Answer: The correct answer is D This patient has essential tremor, for which primidone and propranolol

are the mainstays of treatment This patient's diabetes relatively precludes the use of propranolol; therefore

primidone is the more appropriate option for her The antiparkinsonian agents carbidopa/levodopa and pramipexole

are not indicated for essential tremor Botulinum toxin can be effective therapy for essential tremor, especially for

severe head and voice tremors; however, this patient has not had a trial of medication, and she is most bothered by

her extremity tremors

11 An asymptomatic 24-year-old woman with a family history of Huntington's disease (HD) in her mother

undergoes genetic testing for HD after pretest genetic counseling Her HD gene testing shows 34 repeats Which of

the following is the most appropriate interpretation of her gene test results?

A She may develop a mild form of HD

B She will not develop HD, and there is no risk to her children

C She will not develop HD, but her children are at risk for a full-length mutation

D She will develop HD, but not likely until her fifth decade of life

E She will develop HD, most likely while still in her 20s

Correct Answer: The correct answer is C Persons with 30 to 35 repeats, such as this patient, will not develop HD, but their offspring may carry a full-length mutation due to repeat instability Normal huntingtin alleles have less than 30 CAG repeats; 36 to 39 repeats are incompletely penetrant; 40 or more repeats are fully penetrant; repeat lengths greater than 60 are associated with disease onset earlier than age 21; and patients with 80 or more repeats usually present in the first decade

12 Which of the following requirements must patients with Parkinson's disease usually meet in order to be eligible for deep brain stimulation?

A Age greater than 60 years

B Disease duration less than 5 years

C Gait impairment unresponsive to levodopa

D Greater than 30% improvement in Unified Parkinson Disease Rating Scale (UPDRS) motor scores between on and off states

E Total off time per day no more than 25% of total on time per day

Correct Answer: The correct answer is D Except for tremor (which may respond to deep brain stimulation [DBS] even when it does not respond to medication), only those symptoms that respond to levodopa (when the patient is on) will respond to DBS Thus, a greater than 30% improvement in UPDRS motor scores between off and on state

is generally required for a patient to be a candidate for DBS As long as their symptoms respond to levodopa, patients who are in an off state for a considerable portion of the day are likely to benefit from DBS The younger the patient, the better the chances of good outcome from DBS A disease duration of at least 5 years is generally desirable before proceeding with DBS, primarily to increase the certainty of the diagnosis of Parkinson's disease as opposed to some other parkinsonian condition

13 A 64-year-old woman with Parkinson's disease began yelling and punching her husband while she was sleeping When he was able to get her to respond to his questions, she told him that she had been dreaming that kidnappers had been threatening their granddaughter Her only current medications are selegiline and carbidopa/levodopa Which of the following medication adjustments would be most likely to be effective for this condition?

A Add amantadine

B Add amitriptyline

C Add clonazepam

D Stop carbidopa/levodopa

E Stop selegiline

Correct Answer: The correct answer is C This history is consistent with REM sleep behavior disorder (RBD), in which patients fail to develop atonia during REM sleep so they act out their dreams This condition has been reported in 25% to 50% of patients with Parkinson's disease (sometimes as the initial clinical manifestation); it is typically treated with clonazepam

14 A 32-year-old professional violinist comes to the office because of a 4-year history of intermittent tremors The tremors occur mainly when she drinks coffee or she feels she is under stress; they are particularly bothersome when she performs onstage Examination shows a very mild postural tremor of her arms Which of the following is the most appropriate treatment?

A Gabapentin daily

B Primidone daily

C Propranolol as needed

D Reassurance alone

E Topiramate daily

Correct Answer: The correct answer is C This patient has enhanced physiological tremor Enhanced physiological tremor is often worse after ingestion of stimulants such as caffeine or during periods of stress Treatment of enhanced physiological tremor is often reassurance; however, in this patient with a situational cause of symptoms, a low dose of propranolol or benzodiazepine (not listed) can be helpful when taken prior to the situation that induces the tremor, such as 30 to 60 minutes before her performances The other medications listed are options for essential tremor and are not appropriate for this patient, who only requires occasional medication to suppress her anxiety-induced, enhanced physiological tremor

15 About an hour after receiving codeine and promethazine for a severe headache, nausea, and vomiting, a 22-year-old pregnant woman suddenly began to arch her neck and twist it to one side She reported excruciating pain, and she became very anxious and agitated, saying that she could not control her head position and that she felt

"possessed." Which of the following medications should be administered acutely in order to alleviate her symptoms?

A Dantrolene

B Diphenhydramine

C Haloperidol

D Levodopa

E Naloxone

Correct Answer: The correct answer is B Medications that block dopamine receptors, including antipsychotic and

antiemetic medications, can result in acute dystonic reactions These usually respond to anticholinergic medications

such as diphenhydramine, benztropine, or trihexyphenidyl Although prodopaminergic and antidopaminergic drugs

may be beneficial for some patients with chronic dystonia, these agents are not typically used for acute dystonic

reactions

16 A 34-year-old woman comes to the office for evaluation of a 2-year history of cognitive problems, progressive

unsteadiness of gait, and abnormal movements of her arms and legs Her father had a similar illness and died in his

50s Examination shows slurred speech, problems with memory and concentration, choreoathetosis in the

extremities, and gait ataxia MRI shows severe diffuse periventricular white matter abnormalities on T2-weighted

images Genetic testing for Huntington's disease is negative Which of the following is the most likely diagnosis?

A Dentatorubral-pallidoluysian atrophy (DRPLA)

B Huntington's disease-like (HDL) illness-1

C HDL-2

D HDL-3

E HDL-4

Correct Answer: The correct answer is A This patient's symptoms and signs are consistent with DRPLA, a

dominantly inherited neurodegenerative disorder characterized by progressive dementia associated with either

chorea (with or without ataxia) or myoclonic epilepsy MRI in DRPLA may show extensive areas of

demyelination, as seen in this case The HDL illnesses are a group of rare choreiform disorders that clinically

resemble Huntington's disease but do not carry the huntingtin mutation; none of these disorders is associated with

the white matter abnormalities that can be seen in DRPLA

17 Patients taking rasagiline should limit consumption of which of the following dietary constituents?

A Aged cheese

B Phenylalanine

C Saturated fats

D Sodium

E Vitamin E

Correct Answer: The correct answer is A Rasagiline is a relatively selective inhibitor of monoamine oxidase-B

(MAO-B) Monamine oxidase-A (MAO-A) in the gut metabolizes ingested tyramine The dose at which rasagiline

loses its MAO-B selectivity and begins to inhibit MAO-A has not yet been clearly delineated It is currently

recommended, therefore, that patients taking rasagiline should avoid foods rich in tyramine (such as red wine, tap

beer, aged cheese, and yeast) because they could precipitate a hypertensive crisis

18 A 19-year-old man comes to the office because of a 2-year history of progressive clumsiness of the extremities,

gait problems, and dysarthria Examination shows dysarthria, mild diffuse chorea, and a spastic gait with episodes

of freezing T2-weighted MRI shows a central region of hyperintensity surrounded by a rim of hypointensity in the

globus pallidus Peripheral blood smear shows acanthocytes Which of the following is the most likely diagnosis?

A Chorea-acanthocytosis (ChAc)

B McLeod syndrome

C Neurodegeneration with brain iron accumulation (NBIA)

D Pantothenate kinase-associated neurodegeneration (PKAN)

E Wilson's disease

Correct Answer: The correct answer is D This patient has characteristic symptoms and signs of PKAN, including

the "eye of the tiger" sign in the globus pallidi on T2-weighted MRI images Some patients with PKAN have

acanthocytes in their peripheral smear, as in this patient ChAc and McLeod syndrome are neuroacanthocytosis

syndromes that would not be associated with the MRI changes seen in this patient NBIA is a group of disorders

with iron accumulation in the basal ganglia but without the imaging or genetic changes seen in PKAN Wilson's

disease can cause chorea, although typically tremor and dystonia are more common; the MRI findings seen in this

patient are not seen in Wilson's disease

19 A 7-year-old girl who "never seemed to walk quite right" has developed worsening gait over the past year She

is fine every morning, but her legs get increasingly stiff as the day proceeds Her examination in the late afternoon

is notable for a stiff awkward gait with fluctuating plantar flexion and eversion of her feet and inversion at her

wrists and elbows She also has reduced facial expression and bradykinesia Which of the following treatments is most likely to be beneficial?

A Botulinum toxin injections

B Levodopa

C Physical therapy

D Tetrabenazine

E Zinc

Correct Answer: The correct answer is B The prominent diurnal fluctuation is typical of dopa-responsive dystonia, which usually presents in childhood with dystonia and parkinsonian features The hallmark of this condition is a dramatic and sustained response to low-dose levodopa

20 Randomized controlled trials of initial therapy with pramipexole, ropinirole, or pergolide (versus levodopa monotherapy) in patients with mild Parkinson's disease have generally yielded similar results Compared to patients

in the levodopa group, at the study end point patients in the dopamine agonist group had:

A Less dyskinesias and less parkinsonism

B Less dyskinesias and more parkinsonism

C More dyskinesias and less parkinsonism

D More dyskinesias and more parkinsonism

E No difference in dyskinesias or parkinsonism

Correct Answer: The correct answer is B In separate randomized controlled trials comparing dopamine agonists to levodopa for initial treatment of Parkinson's disease, the dopamine agonist groups had a lower incidence of dyskinesias, later onset of dyskinesias, or less severe dyskinesias at the study end point, but their scores on motor ratings of parkinsonism were higher

21 Which of the following features would most likely eliminate a patient with advanced Parkinson's disease from consideration for treatment with deep brain stimulation?

A Autonomic instability

B Dementia

C Depression

D Postural instability

E Tremor unresponsive to medications

Correct Answer: The correct answer is B Most groups are reluctant to perform deep brain stimulation (DBS) in patients with Parkinson's disease who have moderate to severe dementia because patients need to be able to participate in an awake surgery and to report their symptoms reliably during DBS programming sessions Cognitive decline has also been reported following DBS in some patients with preexisting dementia Tremor unresponsive to medications is one of the indications for DBS Postural instability (unresponsive to levodopa) and autonomic instability are unlikely to respond to DBS, but they are not contraindications Depression may improve or worsen after DBS; it is not a contraindication

22 Which of the following symptoms is the most common side effect of botulinum toxin injections for cervical dystonia?

A Constipation

B Dysphagia

C Erectile dysfunction

D Hypothermia

E Somnolence

Correct Answer: The correct answer is B Botulinum toxin interferes with the release of acetylcholine It is avidly taken up by nerve terminals, so although it can produce weakness in muscles near the injection site, it does not cause significant systemic side effects unless injected directly into the bloodstream Cervical injections can result in dysphagia due to weakness of the nearby pharyngeal muscles, reduced salivary production, or both

23 A 36-hour-old newborn boy is noted to have recurrent episodes of apnea The child was born of a normal, spontaneous, vaginal delivery after 9 months of gestation On examination he is jittery and has diffusely increased muscle tone and exaggerated startle responses to tactile stimuli that include periods of apnea His mother says that similar problems affected his 2-year-old brother Which of the following is the most likely diagnosis?

A Hyperekplexia

B Myoclonus-dystonia

C Progressive myoclonic epilepsy

D Propriospinal myoclonus

E Thalamic myoclonus

Correct Answer: The correct answer is A Hyperekplexia, or familial startle syndrome, presents in the neonatal

period with muscular rigidity and exaggerated responses to sudden unexpected acoustic or tactile stimuli; these

responses are mediated by the pontine reticular nucleus Early recognition of hyperekplexia is critical as institution

of clonazepam can prevent apneic spells This patient's symptoms are not suggestive of myoclonus-dystonia, which

is characterized by action myoclonus primarily affecting the head, neck, and proximal shoulder girdle and would

not be expected to present in the newborn period His history is also not consistent with a progressive myoclonic

epilepsy; propriospinal myoclonus occurs in the setting of spinal cord lesions and consists primarily of flexion of

the trunk and hips; thalamic myoclonus is a subcortical myoclonus producing asterixis in an arm

24 A 14-year-old boy comes to the office with his parents because of a 2-year history of abnormal movements

Family history is significant for obsessive-compulsive disorder, anxiety, and alcoholism Examination shows brief,

sudden, involuntary jerks of his head and neck that are triggered when he uses his upper extremities to perform

tasks Which of the following is the most likely diagnosis?

A Huntington's disease

B Hyperekplexia

C Myoclonus-dystonia

D Posthypoxic myoclonus

E Progressive myoclonic epilepsy

Correct Answer: The correct answer is C This patient's symptoms are most consistent with myoclonus-dystonia

(also called essential myoclonus), characterized primarily by action myoclonus primarily affecting the head, neck,

and proximal shoulder girdle; associated focal or segmental dystonia is also often present, although this is not yet

the case in this patient This condition typically begins in childhood or adolescence As in this patient, there is often

a family history of anxiety disorders or obsessive-compulsive disorder; an association with alcoholism has been

described Although myoclonus can be seen in Huntington's disease, the characteristic of his symptoms and the

absence of chorea are more suggestive of myoclonus-dystonia Hyperekplexia (or familial startle syndrome)

typically begins in the neonatal period with stiffness and episodes of myoclonus and apnea There is no history of a

hypoxic event to suggest posthypoxic myoclonus, nor is there is history to suggest the seizures of a progressive

myoclonic epilepsy

25 Apomorphine should be administered in conjunction with which of the following types of medication?

A Anticholinergic

B Antiemetic

C Antihistamine

D Antihypertensive

E Protein pump inhibitor

Correct Answer: The correct answer is B Apomorphine was originally introduced in the mid-20th century, but its

use was limited by side effects, including marked nausea It is now administered with an antiemetic

26 A 44-year-old man comes to the office because of a 4-year history of head tremors He also describes a feeling

of mild pain in his neck and feels that his head is being pulled to the left He has no significant past medical history

and takes no medications Examination shows a continuous but waxing and waning tremor of his head, intermixed

with subtle head turns to the left There is no postural or kinetic tremor in the arms, and the remainder of his

neurological examination is normal Which of the following is the most effective treatment of his tremor?

A Baclofen

B Botulinum toxin

C Clonazepam

D Primidone

E Trihexyphenidyl

Correct Answer: The correct answer is B This patient's symptoms and signs are most consistent with dystonic

tremor Features in this patient that are typical of dystonic tremor of the neck include the feeling of pulling or pain,

as well as the tendency for the movements to occur more in one direction Intramuscular botulinum toxin is the

treatment of choice for dystonic neck tremor Oral medications such as trihexyphenidyl, baclofen, or

benzodiazepines are alternative agents, but the benefits from these agents are generally more modest than chemical

denervation with botulinum toxin This patient does not have essential tremor so primidone is less likely to be

effective

27 A 34-year-old man comes to the office because of a progressive 2-year history of abnormal movements,

behavioral changes, and numbness and weakness in the extremities He has no significant family history of

neurological disease Examination shows diffuse choreoathetosis including movements of the mouth and tongue; he

has evidence of bites to his lips and tongue due to these movements There is diminished pinprick and vibration

sensation in the distal lower extremities, mild weakness in the proximal legs and arms, as well as diffusely