Continnum Aug 2019

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ARTICLE 1: PARKINSON DISEASE
1 A 41-year-old man is seen in clinic for follow-up after a recent diagnosis

of Parkinson disease. At his initial visit 3 months ago, in addition to
discussing his diagnosis, a decision was made to delay treatment
considering the nondisabling nature of his symptoms. He presents with
family members who have questions about the expected prognosis of
his disease. Which of the following most accurately describes the usual
prognosis of patients such as this with Parkinson disease?
A he is at higher risk than most patients with Parkinson disease to
develop cognitive dysfunction and symptoms of dysautonomia
B he will ultimately be more prone to dyskinesias and motor
fluctuations
C his nondisabling symptoms at this point suggest that his
symptoms may remain stable and not progress over time
D his prognosis will be negatively impacted by his decision to delay
dopaminergic therapy at the time of diagnosis
E postural instability will be expected to form within the first 2 to
3 years after diagnosis in this patient
2 A 65-year-old woman is seen in clinic with a 1-year history of resting

tremor in the right arm, associated with a subjective sense of stiffness in
this limb. Examination reveals rigidity and resting tremor of the right
arm, along with overall bradykinesia and mild hypomimia, prompting a
diagnosis of Parkinson disease. Which of the following best describes
the role for brain imaging in the diagnostic workup of this patient?
A the asymmetry of his findings is atypical for Parkinson disease, and
urgent brain imaging is indicated
B a dopamine transporter single-photon emission computed
tomography (SPECT) has high sensitivity and specificity in
distinguishing Parkinson disease from essential tremor, but is not
needed here given the clinical scenario
C a dopamine transporter single-photon emission computed
tomography (SPECT) should be done to differentiate Parkinson
disease from an atypical parkinsonism syndrome
D the lack of cerebellar atrophy on CT or MRI is needed to
distinguish Parkinson disease from multiple system atrophy
E MRI showing atrophy of the bilateral substantia nigra regions is
required for a diagnosis of Parkinson disease
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3 A 70-year-old woman with a 10-year history of Parkinson disease is
seen in clinic follow-up. Her symptoms have been well-controlled on
carbidopa/levodopa therapy in the past, but she is concerned about
worsening balance dysfunction. Her husband notes that, although she
has not fallen, he has needed to catch her on several occasions to
prevent falls. Which of the following best describes the association of
postural and balance dysfunction and Parkinson disease in this patient?
A her falls are unlikely to be related to any Parkinson disease–associated
postural dysfunction, and other etiologies should be investigated
B postural instability is common in Parkinson disease but is rarely
associated with negative outcomes
C postural instability is relatively resistant to increased doses of levodopa
D the presence or absence of postural instability does not correlate
well with overall Parkinson disease severity
E the presence of postural dysfunction in this case should prompt a
revised diagnosis to progressive supranuclear palsy
4 A 67-year-old man with long-standing Parkinson disease presents for

follow-up. He and his family report severe motor fluctuations and
considerable dyskinesias despite repeated adjustments in his
carbidopa/levodopa dosing, the addition of entacapone, and a trial of
extended-release amantadine (which was stopped because of adverse
effects). In addition to his motor symptoms, over the past 2 years he
has developed worsening cognitive dysfunction, and his Montreal
Cognitive Assessment (MoCA) score in clinic is 14/30. His family
inquires about his candidacy for deep brain stimulation treatment.
Which of the following factors is a contraindication to deep brain
stimulation therapy in this patient?
A age older than 65 years
B inability to tolerate a course of extended-release amantadine
C long duration of disease
D presence of severe motor fluctuations
E presence of significant cognitive dysfunction
ARTICLE 2: PROGRESSIVE SUPRANUCLEAR PALSY, CORTICOBASAL
DEGENERATION, AND MULTIPLE SYSTEM ATROPHY
5 Which test may be indicated early in the disease course for patients
with progressive supranuclear palsy?
A CT angiography
B ECG
C echocardiogram
D laryngoscopy
E video fluoroscopic swallowing examination
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6 The presence of which of the following pathologic findings

differentiates corticobasal degeneration from progressive
supranuclear palsy?
A astrocytic plaques
B coiled bodies
C gliosis
D 4-repeat tau
E tufted astrocytes
7 Which of the following disease complications is unique to patients with

multiple system atrophy?
A aspiration pneumonia
B cardiopulmonary arrest
C pressure ulcers
D subdural hematomas
E urinary tract infections
8 The use of levodopa in patients with multiple system atrophy can risk
worsening which of the following symptoms?
A central apnea
B dysmetria
C orthostasis
D rapid eye movement (REM) sleep behavior disorder
E stridor
9 A 78-year-old woman was diagnosed with progressive supranuclear

palsy 5 years ago. She now has severe dysphagia and sialorrhea. Her
other signs and symptoms include apraxia of eyelid opening,
blepharospasm, loss of downgaze, limited upgaze, bradyphrenia,
emotional lability, and an ataxic gait. Botulinum toxin type B is the
preferred choice compared to botulinum toxin type A in this patient
for which of the following reasons?
A botulinum toxin type A is contraindicated in the symptomatic
management of progressive supranuclear palsy
B botulinum toxin type A is more expensive than botulinum toxin
type B
C botulinum toxin type B is less potent than botulinum toxin type A
so has less risk of worsening her dysphagia
D botulinum toxin type B is more efficacious in the treatment of
apraxia of eyelid opening
E botulinum toxin type B is more efficacious in the treatment of
sialorrhea
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ARTICLE 3: TICS AND TOURETTE SYNDROME
10 An 11-year-old boy is seen in clinic for evaluation of repetitive

abnormal movements. He and his parents describe several different
types of movements, including some rapid contortions of his face,
sustained opening of his mouth, and intermittent bending of his knees.
These movements were previously diagnosed as complex motor tics.
Which of the following best describes features of complex motor tics
and their applicability in this case?
A bending of knee is inconsistent with a diagnosis of complex tics
B the combination of repetitive abnormal movements is not seen in
complex tics
C the concept of complex motor tics is not associated with specific
neurologic disorders, and a psychogenic cause should be pursued
D a diagnosis of complex tics requires a component of coprolalia or
copropraxia, so the diagnosis should be reconsidered
E some complex tics can have a sustained or dystonic quality, such
as this patient’s sustained mouth opening
11 A 7-year-old boy is seen in clinic for follow-up of his tic disorder,

diagnosed 1 year earlier after he began displaying eye blinking, head
turning, and shoulder shrugging. His parents inquire about the
expected course of his tics and whether they will persist throughout his
life. Which of the following best describes the most commonly
observed natural history of this clinical presentation?
A he should be monitored for the development of progressive
neurologic symptoms since tics do not usually develop at his age
B he will not develop vocal tics at this point since vocal tics always
precede motor tics
C his tics are likely to resolve completely by age 10
D the likelihood of tic persistence into adulthood is less than 10%,
and any adult tics will be trivial and nondisabling
E tic severity will most likely peak in severity between the ages of
8 and 12 and then improve throughout adolescence and early
adulthood

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12 A 12-year-old girl is seen in clinic for follow-up of her Tourette

syndrome. At prior visits, a mutual decision was made to pursue
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nonpharmacologic therapies and reassurance was provided to the
patient and parents on the expected course of her tics. At today’s visit,
the possibility of pharmacologic therapy is brought up by her parents,
since her older brother required medication to control his tics. The
presence of which of the following patient-specific or disorder-specific
factors would suggest a possible indication to escalate therapy beyond
nonpharmacologic options?
A female sex
B low self-esteem and trouble interacting with peers due to the tics
C need for pharmacotherapy in older sibling
D persistence of tics to age 12
E presence of ongoing complex motor and vocal tics
13 A 13-year-old boy is seen in clinic for follow-up of his Tourette
syndrome. His tics have been slowly worsening in severity over the
past 2 years and have failed to respond effectively to a course of
comprehensive behavioral intervention therapy (CBIT). Although his
tics are not that frequent, the patient, his parents, and his teachers feel
that they are starting to interfere with his school performance and his
interactions with other students. Which of the following would be the
most appropriate course of treatment in this patient?
A continue comprehensive behavioral intervention therapy (CBIT)
and inform patient/family that pharmacotherapy is unlikely to be
beneficial
B initiate guanfacine
C initiate tetrabenazine
D referral to psychology for biofeedback training
E surgical evaluation for deep brain stimulation
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ARTICLE 4: TREMOR
14 A 38-year-old woman presents with a 1-year history of kinetic tremor of

her upper extremities. It has worsened very slightly over the past year.
On examination, her tremor is clearly asymmetric (worse on the right),
with worsening as she approaches the examiner’s finger on the
finger-nose-finger maneuver. The rhythm of her tremor on the right
changes when she is asked to mimic the examiner by tapping her thumb
and index finger with her left hand. She has phasic asynchrony with a
see-saw effect when her arms are held in a wing-beat position. Which
of the characteristics of her tremor is most suggestive of a diagnosis of
psychogenic tremor?
A asymmetry
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B entrainment
C intentional component
D pattern of progression
E phasic asynchrony
15 Which of the following neurologic signs can be seen in patients with
essential tremor?
A ataxia
B dysarthria
C dysphagia
D hyperreflexia
E nystagmus
16 A 55-year-old woman presents with a 10-year history of essential
tremor. She has tried nonpharmacologic strategies such as weighted
utensils, but as her worsening tremor is starting to negatively impact
her quality of life, she is interested in starting pharmacologic therapy,
specifically primidone. According to the author of this article,
pretreating with phenobarbital is most likely to help prevent which
adverse effect of primidone?
A bradycardia
B diplopia
C fatigue
D nausea
E rash

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17 A 75-year-old woman presents with a severe right sided tremor of

recent onset. The tremor is slow and most severe with movement, but
occurs at rest, and there is a postural component as well. The tremor
is disabling as it is very difficult for her to use her arm. Her past medical
history is notable for hypertension, diabetes mellitus, and a left
thalamic stroke that she experienced 1 year previously. Treatment with
which of the following pharmacologic agents may alleviate her tremor?
A carbamazepine
B carbidopa/levodopa
C diazepam
D levetiracetam
E pregabalin
ARTICLE 5: THE DYSTONIAS
18 A 36-year-old woman is seen in neurology clinic for longstanding

generalized dystonia that started in childhood and worsened through
adolescence. Because of a presumed diagnosis of possible dopa-

responsive dystonia, she is started on carbidopa/levodopa 25 mg/
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100 mg, 1 tablet twice daily. When seen in follow-up 1 month later,
her symptoms and examination are not significantly changed.Which of
the following is the best next step in management?
A begin carbamazepine for kinesigenic components of her dystonia
B continue to increase dosage up to total levodopa dose of
1000 mg/d if tolerated, and monitor for improvement
C increase dosage to 3 times daily and continue indefinitely
D refer for tendon release surgery to prevent limb contractures
E stop carbidopa/levodopa since dopa-responsive dystonia should
be responsive to low-dose levodopa
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19 A 56-year-old woman presents for evaluation of abnormal neck

movements. Her examination is notable for sustained rotation of
the head to the left and relative hypertrophy of the right
sternocleidomastoid muscle, most consistent with cervical dystonia
with primarily torticollis. She was prescribed low-dose clonazepam by
her primary care physician without clear benefit. Which of the
following best describes possible treatment options in this patient?
A botulinum toxin injections are less helpful for this patient given her
torticollis
B botulinum toxin injections should be considered the treatment of
choice for a focal dystonia such as this
C clonazepam is the treatment of choice, and the dose should be
escalated
D given her failure to respond to clonazepam, she should be
referred for possible deep brain stimulation
E muscle relaxants such as cyclobenzaprine should be prescribed
on a scheduled basis
20 A 34-year-old woman with a history of generalized dystonia is seen

in clinic for a second opinion. She has had minimal response
to conservative medical treatments, including a trial of carbidopa/
levodopa with a dose escalation up to 1000 mg/d of levodopa. She asks
about the role for surgical intervention, specifically deep brain
stimulation, as a treatment option in her case. Which of the following
patient characteristics is generally associated with better outcomes
following surgical treatment for dystonia?
A associated clinical features such ataxia and spasticity
B male sex
C presence of tonic postures or contractures
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D shorter disease duration
E very young age at time of surgery

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ARTICLE 6: CHOREA
21 A 45-year-old Chinese woman presents to the emergency department

with flailing of her right arm that began acutely 2 days ago. It improves
during sleep. On examination, she also has abnormal movements of her
right face and leg, but these are less severe. Which of the following
laboratory studies is most likely to reveal the underlying etiology of
her symptoms?
A blood glucose
B complete blood cell count
C serum calcium level
D thyroid-stimulating hormone (TSH)
E urine human chorionic gonadotropin
22 A 64-year-old man presents with a 1-year history of progressive
memory loss and more recent onset of abnormal movements of his
arms, legs, and trunk that resulted in him retiring earlier than planned
from his job as an electrician. He has also recently been diagnosed
with obstructive sleep apnea, and his wife, who accompanies him to the
visit, describes odd movements during sleep in which he appears to
be performing his previous work activities, such as threading wires.
On examination, he has a vertical gaze palsy. What is the most
likely diagnosis?
A ADCY5-related dyskinesia
B anti–contactin-associated proteinlike 2 (CASPR2) encephalitis
C anti-IgLON5 disease
D dentatorubral-pallidoluysian atrophy
E pantothenate kinase-associated neurodegeneration
23 Tetrabenazine carries a US Food and Drug Administration (FDA)
boxed warning for which of the following adverse events?
A agranulocytosis
B all-cause mortality
C hepatic failure
D suicidality
E toxic epidermal necrolysis
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ARTICLE 7: ATAXIA
24 A 74-year-old woman presents to clinic for follow-up of a several-year

history of ataxia. She first started noticing gait dysfunction in her
late sixties, which has been slowly worsening, although she is still able
to carry out all usual tasks. Her examination is notable for subtle gait
and truncal ataxia and no signs of parkinsonism; these findings have
been stable since she was first seen 5 years prior. Which of the
following best describes the most likely diagnosis and prognosis in
this patient’s case?
A adult-onset Friedreich ataxia, and she is at risk for development of
a cardiomyopathy
B idiopathic late-onset cerebellar ataxia, and no major impact on life
expectancy should occur
C irrespective of the cause of her ataxia, she has a high likelihood of
developing rapid eye movement (REM) sleep behavior disorder
D multiple system atrophy, and she will be expected to develop
parkinsonism in the next several years
E spinocerebellar ataxia type 1, and there is a high likelihood she will
need nutrition assistance for dysphagia in the next several years
25 In patients presenting with gait ataxia, which of the following best
describes how a cerebellar ataxia, as opposed to a sensory neuropathy,
can be distinguished as the cause of symptoms?
A ataxia rarely develops after age 50; hence sensory neuropathy
should be considered much more likely in this setting
B cerebellar atrophy is seen in all patients with ataxia
C dysarthria is uncommon in both conditions and should not be used
as a diagnostic tool
D falls are rarely seen in sensory neuropathy syndromes
E nystagmus and abnormal saccades can be seen in cerebellar
ataxia but not in sensory neuropathy syndromes

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26 A 35-year-old man with chronic alcoholism and malnutrition is seen in

consultation in the hospital for mental status changes and gait changes.
Examination shows impaired attention, limited abduction of both
eyes with prominent nystagmus on right and left gaze, and marked
truncal ataxia. Which of the following MRI findings is most likely in
this patient?
A hot cross bun sign in the pontine white matter
B prominent cerebellar atrophy
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C severe bihemispheric white matter hyperintensity on T2/fluid-
attenuated inversion recovery (FLAIR) sequences
D superficial siderosis on gradient recall echo (GRE) or

susceptibility-weighted imaging (SWI) sequences
E T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities in
the mammillary bodies and paraventricular thalamus
27 A 15-year-old boy presents with several years of progressive gait
dysfunction and dysarthria. His medical history is notable for type 1
diabetes mellitus; he has two older siblings with similar gait. General
examination is notable for pes cavus and scoliosis; neurologic
examination shows dysarthria, square-wave jerks and nystagmus,
prominent gait instability, and loss of vibration sense at the great toe
and medial malleolus bilaterally. Which of the following is the most
likely diagnosis?
A fragile X tremor-ataxia syndrome
B Friedreich ataxia
C Kearns-Sayre syndrome
D sensory ataxic neuropathy, dysarthria, and ophthalmoplegia
(SANDO) syndrome
E spinocerebellar ataxia type 2
ARTICLE 8: MYOCLONUS
28 Which of the following electrophysiologic findings confirms

identification of cortical myoclonus?
A enhanced long-latency EMG reflexes to peripheral nerve
stimulation
B enlarged cortical waves in somatosensory evoked potentials
C epileptiform activity on EEG that correlates with the myoclonus
EMG discharges
D increased corticomuscular coherence
E myoclonic EMG discharges of less than 100 ms
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29 A 22-month-old boy presents with several months of irregular, rapid

eye movements and generalized myoclonus. Which of the following
conditions must be definitively ruled out?
A Lyme Disease
B neuroblastoma
C sarcoidosis
D strep throat
E testicular embryonal carcinoma
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30 A 15-year-old boy experienced a respiratory arrest due to an asthma
attack. At what point would a chronic multifocal action myoclonus be
expected to develop?
A following partial recovery of mental status
B 5 minutes after the start of respiratory arrest
C 15 minutes after the start of respiratory arrest
D prior to arrest once oxygen saturation reaches 65 mm Hg
E within 24 to 28 hours of arrest only if the patient remains
unresponsive
31 A 75-year-old woman with a 5-year history of corticobasal
degeneration presents with worsening myoclonus. She reports this
symptom is most impactful to her quality of life. She has no significant
past medical history, specifically no psychiatric history. Which
medication should be tried first?
A carbamazepine
B levetiracetam
C perampanel
D valproic acid
E zonisamide
ARTICLE 9: TARDIVE SYNDROMES
32 Which of the following patient characteristics is considered to have the

strongest support from observational data as a risk factor for the
development of a tardive movement syndrome?
A African American race
B concomitant use of anticholinergic medications
C female sex
D older age at time of neuroleptic exposure
E presence of coexistent HIV infection

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33 A 46-year-old man with longstanding schizophrenia is seen in clinic for

abnormal movements of the face and limbs. His examination shows
frequent lip smacking, tongue protrusion, and athetoid movements of
the arms and legs. He has been treated with a variety of neuroleptic
medications in the past, most recently olanzapine. If possible, changing
to which of the following antipsychotic medication regimens should
be considered in order to minimize the risk of worsening neurologic
side effects?
A aripiprazole
B clozapine
C haloperidol
D risperidone
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E ziprasidone
ARTICLE 10: MOVEMENT DISORDERS IN CHILDREN
34 Which of the following conditions, although usually benign, requires

further workup with imaging as it may be associated with an
underlying malignancy?
A benign neonatal sleep myoclonus
B idiopathic dystonia of infancy
C paroxysmal tonic upgaze of infancy
D Sandifer syndrome
E spasmus nutans
35 Next-generation sequencing methods will detect which of the
following genetic disorders?
A ADCY5-related dyskinesia
B dentatorubral-pallidoluysian atrophy
C fragile X syndrome
D Friedreich ataxia
E Huntington disease
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36 A 10-year-old boy presents with myoclonus and dystonia that began

approximately 3 years ago. The myoclonus preceded the dystonia by
a year, and both symptoms are most prominent in the arms and neck.
His symptoms are exacerbated during soccer practice and games and
when he is taking tests at school. He is a fifth grader and is performing
well in the gifted track but seems to get very worried about his
assignments and tests. His myoclonus has been captured on EEG and
did not reveal any associated epileptiform activity (overall normal
study). Neuroimaging was normal. Evaluation should be performed in
this patient for which possible comorbidities?
A cardiomyopathy
B depression and anxiety
C migraine
D retinopathy
E ulcerative colitis
37 The neurologic symptoms of ataxia-telangiectasia may be preceded by

which of the following recurrent systemic symptoms?
A appendicitis
B bronchitis/sinusitis
C cellulitis
D cholecystitis
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E pyelonephritis
ARTICLE 11: PSYCHOGENIC (FUNCTIONAL) MOVEMENT DISORDERS
38 A 23-year-old woman presents for a variety of neurologic symptoms,

including episodic tremor of the left arm, stuttering speech, left-sided
numbness, and a bouncy gait, which have been present for the past
5 years. Based on these findings, a psychogenic etiology for her
symptoms is thought to be most likely. Which of the following best
describes the expected prognosis for this patient with a syndrome of
psychogenic neurologic symptoms?
A her symptoms should be expected to improve rapidly after the
diagnosis of psychogenic disorder is made
B longer duration of symptoms is the greatest predictor of poor
outcome in her case
C the presence of a multitude of symptoms is a positive prognostic
factor in her case
D a second MRI is needed for her symptoms to begin improving
E younger age of the patient is a negative prognostic factor in her
case

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39 Which region of the brain has been suggested to contribute to the

development of psychogenic movement disorders through its
involvement in the modulation of the neural circuit for self-agency?
A hypothalamus
B mamillary bodies
C prefrontal cortex
D temporoparietal junction
E thalamic reticular nucleus
40 A 16-year-old girl presents for a third neurologic opinion for fixed

plantar flexion and inversion of her right foot that began acutely
4 months ago. Although initially she would attempt to weight bear, she
very quickly progressed to assistive devices and now uses a rolling
walker owing to severe pain with walking attempts. Her parents would
like assistance in obtaining an electric wheelchair. She has had an
extensive workup. On examination she moans, grimaces, and cries
when she attempts to straighten out her foot. She also has giveway
weakness in the right leg and arm but demonstrates full strength with
encouragement. She drags her foot behind her when she walks, but she
is able to walk normally when the examiner walks alongside her and
lightly touches the outside of her foot with his own after suggesting
this often helps. What is the most appropriate next step in the
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management of this patient?
A assist the patient in obtaining an electric wheelchair
B functional MRI of the brain
C hydrocodone-ibuprofen 1 tablet every 6 hours as needed for
severe pain
D motor retraining
E referral to rheumatology
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Postreading
Self-Assessment and
CME Test—Preferred
Responses
By Adam G. Kelly, MD; Allison L. Weathers, MD, FAAN
MOVEMENT DISORDERS
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ARTICLE 1: PARKINSON DISEASE
1 The preferred response is B (he will ultimately be more prone to dyskinesias

and motor fluctuations). The prognosis of Parkinson disease can be difficult to
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estimate in a specific individual patient, although some general principles can
be applied to most patients. Patients such as this who are relatively younger at
the time of diagnosis will be at higher risk of developing levodopa-induced
dyskinesias and motor fluctuations over time, while older patients will be at
SELF-ASSESSMENT
AND CME

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higher risk for cognitive symptoms and dysautonomia. Postural instability is in

most cases a relatively later manifestation of Parkinson disease, often occurring
10 or more years after diagnosis. His symptoms should be expected to gradually
progress, although this progression is not based on a decision to institute
levodopa or other treatment options at this stage. For more information, refer to
page 901 of the Continuum article “Parkinson Disease.”
2 The preferred response is B (a dopamine transporter single-photon emission

computed tomography [SPECT] has high sensitivity and specificity in
distinguishing Parkinson disease from essential tremor, but is not needed here
given the clinical scenario). Parkinson disease remains a diagnosis made almost
exclusively on clinical grounds, without specific requirements on either brain
imaging or laboratory studies. Particularly in a case such as this, with a clinical
history and examination that are highly supportive of a Parkinson disease
diagnosis, many providers may elect not to perform brain imaging at all. The
asymmetric presence of her tremor is quite usual for Parkinson disease, and this
finding does not suggest that imaging is mandatory. Dopamine transporter
SPECT can be considered as an ancillary test for the diagnosis of Parkinson
disease, although the strongest evidence for this test is in the distinction
between Parkinson disease, and essential tremor; since the diagnosis of
essential tremor is unlikely in this case based on clinical grounds, this testing is
not likely to add significantly to the diagnostic process. Of note, dopamine
transporter scan is not intended to be used to distinguish Parkinson disease,
from other degenerative atypical parkinsonism syndromes. For more
information, refer to page 906 of the Continuum article “Parkinson Disease.”
3 The preferred response is C (postural instability is relatively resistant to

increased doses of levodopa). This patient with a relatively long history of
Parkinson disease is now experiencing increasing balance and postural
dysfunction. This is a common manifestation of Parkinson disease, particularly as
disease severity and duration increase, and should not prompt a revision in the
diagnosis. Unlike other common manifestations of Parkinson disease (eg, tremor,
bradykinesia), postural instability is relatively nonresponsive to levodopa and
other treatment options. It is associated with a variety of negative outcomes,
including falls, hip fractures, and loss of independence, so evaluations by
rehabilitation specialists (eg, physical therapy) should be considered in patients
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like this. For more information, refer to pages 898–899 of the Continuum article
“Parkinson Disease.”
4 The preferred response is E (presence of significant cognitive dysfunction).

This patient is presenting with increasing severity of a number of symptoms
related to his Parkinson disease or subsequent treatment. Deep brain stimulation
is an option for some patients such as this, who have severe progressive motor
symptoms despite intensive medical management. However, there are some
contraindications/exclusions to deep brain stimulation therapy, the most
important of which in this patient is the presence of significant cognitive
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dysfunction. Other exclusion criteria include an atypical (Parkinson-plus)

syndrome, unstable psychiatric illness, or major medical comorbidities. Advanced
age is also a relative contraindication although many centers use an age cutoff
of 75 years, so this may not be a particular factor in this patient’s case. For more
information, refer to pages 912–913 of the Continuum article “Parkinson Disease.”
ARTICLE 2: PROGRESSIVE SUPRANUCLEAR PALSY, CORTICOBASAL

DEGENERATION, AND MULTIPLE SYSTEM ATROPHY
5 The preferred response is E (video fluoroscopic swallowing examination).

Patients with progressive supranuclear palsy can develop dysphagia early in
the course of their disease and are at risk for aspiration pneumonia. Inspiratory
stridor, cerebrovascular complications, and cardiac abnormalities are not
commonly associated with progressive supranuclear palsy. For more
information, refer to page 921 of the Continuum article “Progressive
Supranuclear Palsy, Corticobasal Degeneration, and Multiple System Atrophy.”
6 The preferred response is A (astrocytic plaques). The typical glial finding in

corticobasal degeneration is astrocytic plaques, tau-containing processes
surrounding astrocytes. The astrocytic tau accumulations that occur in
progressive supranuclear palsy are tufted astrocytes. 4-Repeat tau is present in
both, but the ultrastructure of the insoluble tau fragments is different between
the two diseases. Coiled bodies, tau fibrils around nuclei of oligodendroglia, are
present in both diseases. Gliosis is also present in both diseases. For more
information, refer to page 925 of the Continuum article “Progressive
7 The preferred response is B (cardiopulmonary arrest). Patients with multiple

system atrophy are at risk of sudden death both from cardiopulmonary arrest
due to severe autonomic dysfunction as well as from anoxic injury due to
Page 16
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laryngeal stridor. The other complications listed may occur in all atypical
parkinsonian syndromes. For more information, refer to page 927 of the
Continuum article “Progressive Supranuclear Palsy, Corticobasal Degeneration,
and Multiple System Atrophy.”
8 The preferred response is C (orthostasis). Patients with multiple system atrophy

who have resting tremor may experience some benefit from levodopa early in
their course. However, this benefit is usually only transient, and patients often
experience worsening orthostasis, requiring use of blood pressure support
agents, which in turn may cause hypertension. The other symptoms and signs of
multiple system atrophy are not treated or worsened by the use of levodopa.
For more information, refer to page 932 of the Continuum article “Progressive

is prohibited.
9 The preferred response is E (botulinum toxin type B is more efficacious in the

treatment of sialorrhea). Botulinum toxin type B is more effective than
botulinum toxin type A for the treatment of sialorrhea, although the
administration of botulinum toxin type B is more painful than that of botulinum
toxin type A. There is no significant difference in cost. Both types can worsen or
lead to the development of dysphagia in patients with progressive supranuclear
palsy, and caution should be used. Treatment should be initiated with lower
doses than are used in patients with Parkinson disease. For more information,
refer to page 932 of the Continuum article “Progressive Supranuclear Palsy,
Corticobasal Degeneration, and Multiple System Atrophy.”
ARTICLE 3: TICS AND TOURETTE SYNDROME
10 The preferred response is E (some complex tics can have a sustained or

dystonic quality, such as this patient’s sustained mouth opening). This patient
is presenting with repetitive motor movements that have features most
consistent with a diagnosis of complex motor tics. Tics of this type can be
completely nonpurposeful or have an appearance of purposefulness, such
as this patient’s knee bending. Complex motor tics can also have a more
sustained or dystonic quality, so this patient’s prolonged mouth opening is
consistent with a complex tic. Coprolalia and copropraxia are relatively
uncommon manifestations of tics and are not required for a diagnosis of
complex tics. For more information, refer to page 937 of the Continuum
article “Tics and Tourette Syndrome.”
11 The preferred response is E (tic severity will most likely peak in severity
between the ages of 8 and 12 and then improve throughout adolescence and
early adulthood). This patient is presenting with stereotyped movements that
seem highly consistent with simple motor tics. The development of symptoms
Page 17
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at age 6 is very common and not predictive of any underlying progressive
degenerative condition. The most common pattern of tics is worsening severity
around ages 8 through 12 with gradual improvement after that. Roughly one-third
of patients with tics will have complete resolution, one-third will have
improvement over time, and one-third will have persistent and fluctuating tics
into adulthood. This patient may yet develop vocal tics since motor tics most
commonly precede the onset of vocal tics. For more information, refer to
page 938 of the Continuum article “Tics and Tourette Syndrome.”
12 The preferred response is B (low self-esteem and trouble interacting with

peers due to the tics). Tics and Tourette syndrome are relatively common and,
in many cases, do not require treatment, particularly pharmacologic treatment.
However, if patient/family education and other conservative approaches are
unsuccessful, other options may need to be considered. Specific indications
for tic-suppressing therapy include the presence of psychosocial distress (such
as the poor self-esteem and difficulty with social interactions described here),
1166 AUGUST 2019

is prohibited.
difficulty with work or school performance, and tic-induced physical

dysfunction. The sex of this patient, the persistence of her tics to her current
age of 12 (which is not uncommon), the specific nature of the tics, and the
presence of an older sibling who required treatment do not represent clear
indications to begin pharmacologic treatment for tics. For more information,
refer to pages 948–949 of the Continuum article “Tics and Tourette syndrome.”
13 The preferred response is B (initiate guanfacine). This patient has a known

diagnosis of Tourette syndrome and has been experiencing worsening of
symptoms in his preadolescent years, which is a common observation in
patients with this disorder. His tics are now starting to interfere with his school
performance and his psychosocial function, so treatment is indicated in his
case. Since he has not responded to a more conservative course of
comprehensive behavioral intervention therapy (CBIT), a trial of
pharmacotherapy is reasonable. Given the relatively mild nature of his
symptoms and the fact that he has not tried medications in the past, a tier 1
medication, such as guanfacine, is the preferred option in this case. Tier 2
medications, such as tetrabenazine and antipsychotic medications, should be
reserved for more severe cases given their side effect profile. Deep brain
stimulation should only be considered in more severe cases in which medical
management is unsuccessful. At present, limited evidence exists to support the
role of biofeedback training in Tourette syndrome. For more information, refer
to page 950 of the Continuum article “Tics and Tourette syndrome.”
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ARTICLE 4: TREMOR
14 The preferred response is B (entrainment). Entrainment (the ability to bring the

tremor into a specific rhythm on examination) along with distractibility and
suggestibility are suggestive of psychogenic tremor. The kinetic tremor of
essential tremor is often slightly asymmetric and can be markedly asymmetric or
even unilateral in a small percentage of patients. Approximately half of patients
with essential tremor will have an intentional component to their tremor, and
phasic asynchrony is common. Patients with psychogenic tremor often report an
acute onset of their tremor with maximal severity at the beginning; however,
gradual and subtle worsening does not definitively rule out psychogenic tremor.
For more information, refer to page 969 of the Continuum article “Tremor.”
15 The preferred response is A (ataxia). While the ataxia in most patients with
essential tremor is mild, it may be moderate in severity. Patients with essential
tremor have an increased number of falls and near falls compared to
age-matched controls. The ataxia may only be apparent on examination on
tandem gait. The other signs (dysarthria, dysphagia, hyperreflexia, and
nystagmus) are not seen in patients with essential tremor, and their presence
should raise concern for an alternative diagnosis, such as a spinocerebellar ataxia.
For more information, refer to page 963 of the Continuum article “Tremor.”

is prohibited.
16 The preferred response is D (nausea). Acute nausea is a common adverse

effect with starting primidone, occurring in approximately one-fourth of
patients. A lower starting dose does not prevent this adverse effect, but
pretreating with phenobarbital can be an effective strategy. It will not prevent
diplopia or fatigue, which can occur at higher doses of primidone, or a rash from
an allergic reaction. Bradycardia is an adverse effect of propranolol, another
medication with efficiency in the treatment of essential tremor. For more
information, refer to page 964 of the Continuum article “Tremor.”
17 The preferred response is B (carbidopa/levodopa). This patient’s tremor is

consistent with postinfarction Holmes tremor, which can occur after a latency
of up to 2 years following an infarct in the pontine-midbrain region, cerebellum,
or thalamus. With this type of tremor, the dopaminergic system may be
involved; therefore, carbidopa/levodopa has been reported to be beneficial as
it improves all three components of the tremor (kinetic, resting, and postural).
Medications used for the treatment of essential tremor, including beta-

blockers and primidone, may also be effective in alleviating the postural and
kinetic components of the tremor. For more information, refer to page 969 of
the Continuum article “Tremor.”
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ARTICLE 5: THE DYSTONIAS
18 The preferred response is B (continue to increase dosage up to total levodopa

dose of 1000 mg/d if tolerated, and monitor for improvement). This patient has
a presentation that is consistent with a dopa-responsive dystonia. Although
some patients with dopa-responsive dystonia can have a clinical response at
low doses of levodopa, many patients require higher doses before seeing an
effect. If the clinical suspicion remains high, levodopa dosage should be
titrated up to a dose of 1000 mg/d (in adults) if tolerated in order to provide an
adequate levodopa trial. There is no indication in this patient’s case for a
referral for tendon release surgery or empirically trying carbamazepine. For
more information, see page 991 of the Continuum article, “The Dystonias.”
19 The preferred response is B (botulinum toxin injections should be considered

the treatment of choice for a focal dystonia such as this). This patient’s
presentation is classic for cervical dystonia, which responds well to botulinum
toxin injections; this approach is considered the standard of care for cervical
dystonia and other focal or segmental dystonias. Botulinum toxin is less
effective for patients with anterocollis, prominent tremor with their dystonia,
and those with long-standing fixed postures. Clonazepam and other
benzodiazepines can be habit forming and should be avoided if other options
are possible, and muscle relaxants can help with some discomfort but may not
affect the underlying movement disorder to a significant degree. While some
patients with dystonia may respond to deep brain stimulation, this should only
1168 AUGUST 2019

is prohibited.
be considered in patients who do not respond to more conservative approaches.

For more information, see page 993 of the Continuum article, “The Dystonias.”
20 The preferred response is D (shorter disease duration). Deep brain stimulation

and other surgical approaches are effective options for some patients with
dystonia who do not respond to conservative medical management. The
specific approach and expected outcome vary across patients, but some
general principles for predicting outcome do exist. Patients with shorter
disease duration and those with a mobile dystonia (ie, without tonic postures or
contractures) tend to have better outcomes than patients with longer disease
duration and fixed postures, respectively. No clear association with age of
treatment is seen other than the observation that very young patients (younger
than 12 years of age) are at higher risk for surgical complications. While the
presence of associated clinical features such as spasticity and ataxia does not
present an absolute contraindication to surgery, in general, patients with
Page 20
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isolated dystonia are more likely to respond favorably to surgery. For more
information, see page 994 of the Continuum article, “The Dystonias.”
ARTICLE 6: CHOREA
21 The preferred response is A (blood glucose). This patient has hemichorea.

While the subthalamic nucleus is often mentioned as a classic location in most
teaching, the lesion does not have to be in the subthalamic nucleus. However,
hemichorea (or markedly asymmetric generalized chorea) can also be caused
by systemic disorders, including nonketotic hyperglycemia. This etiology has
been reported to occur in increased frequency in Asian women, even those
without a prior diagnosis of type 2 diabetes mellitus. In this case, out of the
studies listed, blood glucose testing is most likely to reveal the diagnosis.
Polycythemia vera can also cause hemichorea, and a complete blood cell count
should be checked. Chorea gravidarum is chorea that occurs during pregnancy
(although is not usually markedly asymmetric) but is not a diagnosis in itself,
and a workup should be undertaken for an underlying cause. Therefore, a urine
pregnancy test and thyroid-stimulating hormone (TSH) are appropriate in this
patient. Hypocalcemia can also cause chorea, but this is also not usually
markedly asymmetric. For more information, refer to page 1005 of the
Continuum article “Chorea.”
22 The preferred response is C (anti-IgLON5 disease). Anti-IgLON5 disease is a

recently described autoimmune disorder characterized clinically by dementia,
chorea, parasomnia and obstructive sleep apnea, and vertical supranuclear
gaze palsy. Patients may display behaviors during sleep consistent with
performing common work activities, called finalistic behaviors. Studies have
shown a neurodegenerative component (a tauopathy) as well. ADCY5-related
dyskinesia is an autosomal disorder due to mutations in the ADCY5 gene and is
clinically associated with facial dyskinesia, hypotonia, and delayed motor

is prohibited.
milestones. Anti–contactin-associated proteinlike 2 (CASPR2) encephalitis is

another autoimmune etiology of chorea but is not associated with obstructive
sleep apnea and the described parasomnia. Dentatorubral-pallidoluysian
atrophy is a rare autosomal disorder due to a CAG repeat expansion in the
ATN1 gene. Patients with the late-onset form can have chorea, ataxia,
parkinsonism, and dementia. Chorea is rare in pantothenate kinase-associated
neurodegeneration but is more commonly seen in neuroferritinopathy and
aceruloplasminemia. For more information, refer to page 1009 of the
23 The preferred response is D (suicidality). Tetrabenazine can cause depression

and suicidality in addition to parkinsonism and akathisia. Cytochrome P450 2D6
Page 21
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isozyme (CYP2D6) testing is recommended for doses higher than 50 mg/d to
assess whether a patient is a poor, intermediate, or extensive metabolizer, and
doses should be adjusted accordingly. In poor metabolizers, doses higher than
50 mg/d should not be used. For more information, refer to page 1030 of the
ARTICLE 7: ATAXIA
24 The preferred response is B (idiopathic late-onset cerebellar ataxia, and no

major impact on life expectancy should occur). This patient has had a
relatively slowly progressive ataxia starting in her sixties, without other
neurologic abnormalities such as parkinsonism, or sensory neuropathy. The
most likely diagnosis is idiopathic late-onset cerebellar ataxia, and the lack
of any significant progression (and particularly the lack of developing
parkinsonism) suggests a benign prognosis that should not limit her life
expectancy. The other options listed are causes of ataxia but are less likely
based on the patient’s history and examination. Rapid eye movement (REM)
sleep behavior disorder is commonly seen in multiple system atrophy but is
not seen in most other causes of ataxia, including idiopathic late-onset
cerebellar ataxia. For more information, refer to page 1050 of the Continuum
article “Ataxia.”
25 The preferred response is E (nystagmus and abnormal saccades can be seen in

cerebellar ataxia but not in sensory neuropathy syndromes). Ataxia can be
seen from a cerebellar disorder or from profound sensory dysfunction as a
result of a sensory neuropathy. Gait changes can be similar in both disorders,
and the presence or absence of falls is not a discriminating feature. The
presence of nystagmus, abnormal saccades (hypermetric or hypometric), and
dysarthria (scanning quality) can make the diagnosis of a cerebellar ataxia more
likely. The absence of cerebellar atrophy does not rule out a cerebellar ataxia
since atrophy is often not present early in the course of some disorders. For
more information, refer to pages 1037–1038 of the Continuum article “Ataxia.”
1170 AUGUST 2019

is prohibited.
26 The preferred response is E (T2/fluid-attenuated inversion recovery [FLAIR]

hyperintensities in the mammillary bodies and paraventricular thalamus).
This patient is described as having a classic presentation of Wernicke
encephalopathy (mental status changes, ophthalmoparesis, and gait ataxia) in
the setting of known risk factors for this disorder (alcoholism and malnutrition).
Imaging is usually not needed to confirm this diagnosis, and empiric treatment
with thiamine should be started in cases where Wernicke encephalopathy is
suspected. That being said, in patients with Wernicke encephalopathy, imaging
Page 22
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will most commonly show T2/fluid-attenuated inversion recovery (FLAIR)
changes in the mammillary bodies and paraventricular thalamus. The other
options listed are imaging findings that can be seen in other causes of ataxia
but would not be seen in the setting of Wernicke encephalopathy. For more
information, refer to pages 1042–1043 of the Continuum article “Ataxia.”
27 The preferred response is B (Friedreich ataxia). This case describes a patient

with symptoms of a cerebellar disorder plus an associated sensory neuropathy;
this presentation, along with the associated history of type 1 diabetes mellitus
and an autosomal recessive inheritance pattern, is strongly suggestive of
Friedreich ataxia. Sensory ataxic neuropathy, dysarthria, and ophthalmoplegia
(SANDO) syndrome can also present with ataxia and sensory neuropathy but
usually presents in adulthood as opposed to Friedreich ataxia, which usually
presents in the childhood or teenage years. Kearns-Sayre syndrome is a
mitochondrial disorder and would have a maternal inheritance pattern, and
spinocerebellar ataxia type 2 (along with other spinocerebellar ataxias) is a
trinucleotide repeat disorder inherited in an autosomal dominant pattern.
Fragile X tremor-ataxia syndrome is an X-linked disorder and would also not
have the inheritance pattern described here. For more information, refer to
pages 1045–1046 of the Continuum article “Ataxia.”
ARTICLE 8: MYOCLONUS
28 The preferred response is C (epileptiform activity on EEG that correlates

with the myoclonus EMG discharges). Identification of cortical myoclonus may
be confirmed with epileptiform activity on EEG that correlates with the
myoclonus EMG discharges either grossly or with back averaging. The other
electrophysiologic findings support a cortical source of myoclonus but are
not confirmatory. For more information, refer to page 1059 of the Continuum
article “Myoclonus.”
29 The preferred response is B (neuroblastoma). The patient in this case is a

young child with opsoclonus-myoclonus syndrome. Opsoclonus-myoclonus
syndrome in young children is most commonly paraneoplastic in etiology with
neuroblastoma being the frequently associated malignancy. In adults, the
differential diagnosis is broader, both in terms of the associated malignancy in
cases of paraneoplastic etiology (including lung, breast, and kidney) and in

is prohibited.
terms of other possible etiologies (eg, infectious, autoimmune). For more

information, refer to page 1071 of the Continuum article “Myoclonus.”
30 The preferred response is A (following partial recovery of mental status).

The chronic posthypoxic myoclonus, known as Lance-Adams syndrome, occurs
Page 23
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after partial recovery of mental status. While it is usually a multifocal action
myoclonus, negative myoclonus and cerebellar ataxia may develop as well. The
acute myoclonus that occurs after severe hypoxia is usually spontaneous or
stimulus triggered and generalized. For more information, refer to page 1072 of
the Continuum article “Myoclonus.”
31 The preferred response is B (levetiracetam). Unless contraindications exist,

levetiracetam is used as the first-line agent for the symptomatic management
for cortical myoclonus, as is seen in patients with corticobasal degeneration.
Clonazepam and valproic acid are second-line agents. While perampanel has
shown some efficacy, it is not a first- or second-line agent. Zonisamide is used
to treat subcortical and nonsegmental myoclonus such as the type observed in
opsoclonus-myoclonus and myoclonus-dystonia. Carbamazepine is tried in segmental
and peripheral myoclonus; however, botulinum toxin is often more effective.
For more information, refer to page 1076 of the Continuum article “Myoclonus.”
ARTICLE 9: TARDIVE SYNDROMES
32 The preferred response is D (older age at time or neuroleptic exposure). Many

different risk factors for the development of tardive syndrome movement
disorders have been proposed and studied, although most have relatively weak
evidence to support their role. Much of this uncertainty is related to difficulty
accounting for prescribing patterns, neuroleptic medication exposures, and
other factors. Even with that caveat, most studies support older age at time of
neuroleptic use as a risk factor for developing a tardive syndrome: an older
patient exposed to a neuroleptic medication for the same time period as a
younger patient will be more likely to develop tardive features. The other
options listed may have some data supporting their role as a risk factor but
the data supporting age is more convincing. For more information, refer to
pages 1093–1094 of the Continuum article “Tardive Syndromes.”
33 The preferred response is B (clozapine). This patient’s clinical presentation,

including an extensive prior history of exposure to neuroleptic antipsychotic
medications, is highly suggestive of a tardive syndrome movement disorder,
with primarily dyskinetic movements. Minimizing exposure to offending
medications is a critical step in management, although this is not always
possible given the underlying psychiatric or medical illness requiring certain
medications. For patients requiring neuroleptic medications for schizophrenia
or other related mental illnesses, changing to clozapine or quetiapine should
1172 AUGUST 2019

is prohibited.
be considered since these medications are rarely, if ever, associated with

Page 24
{10461233974242065076
the development of tardive movements. For more information, refer to
page 1095 of the Continuum article “Tardive Syndromes.”
ARTICLE 10: MOVEMENT DISORDERS IN CHILDREN
34 The preferred response is E (spasmus nutans). Spasmus nutans, the triad of

head nodding, nystagmus, and head tilt that occurs in late infancy, requires
neuroimaging as it is occasionally associated with optic pathway gliomas.
Sandifer syndrome is torticollis or opisthotonic posturing in the setting of
gastroesophageal reflux but is not associated with malignancy. The other
conditions listed are not associated with any underlying malignancies or
other systemic conditions. For more information, refer to page 1103 of the
Continuum article, “Movement Disorders in Children.”
35 The preferred response is A (ADCY5-related dyskinesia). Next-generation

sequencing techniques, including multigene panels and whole exome
sequencing, are now readily available and being used earlier in the workup of
pediatric patients with suspected genetic disorders. However, a key limitation
of this testing, is that next-generation sequencing methods are not able to
detect triplet repeat expansions and therefore will not reliably detect
trinucleotide repeat disorders. Out of the options listed only ADCY5-related
dyskinesia is not caused by a triplet repeat expansion. For more information,
refer to page 1115 of the Continuum article, “Movement Disorders in Children.”
36 The preferred response is B (depression and anxiety). The patient’s clinical

presentation is most consistent with myoclonus-dystonia. This rare
nondegenerative disorder has a symptom onset at a mean age of 6 years,
and patients have a normal life span. The myoclonus, which is nonepileptic, and
the dystonia are usually most prominent in the arms and neck. Patients have
normal cognition, but psychiatric comorbidities such as depression, anxiety,
and obsessive-compulsive disorder are common. Given the association of
depression and anxiety with this disorder and this patient’s school-related
anxiety, further formal evaluation with a child psychiatrist is warranted. The
other listed conditions are not associated with myoclonus-dystonia. For
more information, refer to page 1116 of the Continuum article, “Movement
Disorders in Children.”
37 The preferred response is B (bronchitis/sinusitis). Ataxia-telangiectasia is a

chronic, progressive ataxia that presents in early childhood and is associated
with dystonia and chorea, progressive ataxia, and oculocutaneous
telangiectasias. There are a number of systemic complications including
immunodeficiency (low numbers of circulating T lymphocytes and B
lymphocytes and decreased serum immunoglobulin levels) and increased

is prohibited.
Page 25
{10461233974242065076
susceptibility to malignancy. Patients may have a history of recurrent

sinopulmonary infections (bronchitis, sinusitis, and pneumonia) prior to the
onset of neurologic symptoms. For more information, refer to page 1115 of
the Continuum article, “Movement Disorders in Children.”
ARTICLE 11: PSYCHOGENIC (FUNCTIONAL) MOVEMENT DISORDERS
38 The preferred response is B (longer duration of symptoms is the greatest

predictor of poor outcome in her case). This patient has historical and
examination findings consistent with a psychogenic etiology to her symptoms.
Longer duration of symptoms at the time of diagnosis is considered the
strongest predictor of poor outcome in psychogenic disorders, with briefer
duration of symptoms being associated with better outcomes. The presence or
absence of several neurologic symptoms is not clearly associated with
prognosis in cases such as this, and no evidence suggests that repeat brain
imaging will alter prognosis. For more information, refer to page 1122 of the
Continuum article “Psychogenic (Functional) Movement Disorders.”
39 The preferred response is D (temporoparietal junction). Self-agency is a

person’s sense of control over voluntary actions. Impairment of self-agency
is thought to contribute to the development of psychogenic movement
disorders. Movements that occur through voluntary circuits are perceived by
patients with psychogenic movement disorders as being involuntary. The
temporoparietal junction is involved in the modulation of the neural circuit for
self-agency, with functional MRI showing decreased connectivity between the
right temporoparietal junction and the bilateral sensorimotor regions in patients
with psychogenic movement disorders. For more information, refer to page 1135
of the Continuum article “Psychogenic (Functional) Movement Disorders.”
40 The preferred response is D (motor retraining). The patient’s presentation is

consistent with a diagnosis of psychogenic dystonia. This diagnosis is
supported by the acute onset, rapid progression to a fixed dystonia, early
severe pain, and suggestibility of her symptoms. Although there is not one clear
best practice for the treatment of psychogenic dystonia, physical therapy
utilizing motor retraining, which emphasizes regaining voluntary control over
movements, is likely beneficial in most patients. While studies utilizing
functional MRI have helped elucidate the possible pathophysiology of
psychogenic movement disorders, it is not recommended in the routine
evaluation of patients with psychogenic movement disorders. Assistive
devices should not be encouraged, and the use of opioids should be strictly
avoided. Nothing in the patient’s presentation suggests a rheumatologic
disorder and therefore referral to rheumatology is not appropriate.
Neurologists should provide ongoing care to patients with psychogenic
movement disorders. For more information, refer to page 1134 of the
Continuum article “Psychogenic (Functional) Movement Disorders.”

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1174 AUGUST 2019
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ARTICLE 1: PARKINSON DISEASE

1 A 41-year-old man is seen in clinic for follow-up after a recent diagnosis

2 A 65-year-old woman is seen in clinic with a 1-year history of resting

1148 AUGUST 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

4 A 67-year-old man with long-standing Parkinson disease presents for

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

6 The presence of which of the following pathologic findings

7 Which of the following disease complications is unique to patients with

9 A 78-year-old woman was diagnosed with progressive supranuclear

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

ARTICLE 3: TICS AND TOURETTE SYNDROME

10 An 11-year-old boy is seen in clinic for evaluation of repetitive

11 A 7-year-old boy is seen in clinic for follow-up of his tic disorder,

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

12 A 12-year-old girl is seen in clinic for follow-up of her Tourette

1152 AUGUST 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

14 A 38-year-old woman presents with a 1-year history of kinetic tremor of

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

17 A 75-year-old woman presents with a severe right sided tremor of

18 A 36-year-old woman is seen in neurology clinic for longstanding

adolescence. Because of a presumed diagnosis of possible dopa-

1154 AUGUST 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

19 A 56-year-old woman presents for evaluation of abnormal neck

20 A 34-year-old woman with a history of generalized dystonia is seen

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

21 A 45-year-old Chinese woman presents to the emergency department

1156 AUGUST 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

24 A 74-year-old woman presents to clinic for follow-up of a several-year

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

26 A 35-year-old man with chronic alcoholism and malnutrition is seen in

D superficial siderosis on gradient recall echo (GRE) or

28 Which of the following electrophysiologic findings confirms

1158 AUGUST 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

29 A 22-month-old boy presents with several months of irregular, rapid

ARTICLE 9: TARDIVE SYNDROMES

32 Which of the following patient characteristics is considered to have the

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

33 A 46-year-old man with longstanding schizophrenia is seen in clinic for

34 Which of the following conditions, although usually benign, requires

1160 AUGUST 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

36 A 10-year-old boy presents with myoclonus and dystonia that began

37 The neurologic symptoms of ataxia-telangiectasia may be preceded by

ARTICLE 11: PSYCHOGENIC (FUNCTIONAL) MOVEMENT DISORDERS

38 A 23-year-old woman presents for a variety of neurologic symptoms,

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

39 Which region of the brain has been suggested to contribute to the

40 A 16-year-old girl presents for a third neurologic opinion for fixed

1162 AUGUST 2019

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ARTICLE 1: PARKINSON DISEASE

1 The preferred response is B (he will ultimately be more prone to dyskinesias

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higher risk for cognitive symptoms and dysautonomia. Postural instability is in