Self-Assessment and CME

Postreading
Self-Assessment and
CME TestPreferred
Responses
D. Joanne Lynn, MD, FAAN; James W. M. Owens Jr, MD, PhD

Following are the preferred responses to the questions in the Postreading

Self-Assessment and CME Test in this Continuum issue. The questions and
answer options are repeated, and the preferred response is given, followed
by an explanation and a reference with which you may seek more specific
information. You are encouraged to review the responses and explanations
carefully to evaluate your general understanding of the course material.
The comments and references included with each question are intended
to encourage independent study.
Participants who complete the Postreading Self-Assessment and CME Test
and issue evaluation online at www.aan.com/continuum/cme may earn up
to 12 AMA PRA Category 1 Creditsi toward SA-CME. Participants have up to
3 years from the date of publication to earn CME credits. No SA-CME will be
awarded for this issue after February 28, 2019.

b 1. Which of the following pharmacologic agents is most likely to be associated

with interictal epileptiform discharges?
A. allopurinol
B . bupropion
C. donepezil
D. lisinopril
E . metformin
The preferred response is B (bupropion). Bupropion is well known to be
associated with drug-induced symptomatic seizures and may be associated
with interictal epileptiform discharges. Other medications that may induce
epileptiform discharges include cefepime, clozapine, lithium, and tramadol.
Various metabolic encephalopathies may also be associated with interictal
epileptiform discharges. For more information, refer to page 25 of the
Continuum article Diagnosis of Epilepsy and Related Episodic Disorders.

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Postreading TestPreferred Responses

b 2. A 26-month-old girl is brought to the emergency department after having a

10-minute generalized convulsive seizure at home. She had been coughing
for several days and had a temperature of 38.6-C (101.5-F) 1 hour prior to the
seizure. She is a normally developing child who was born at term without
complications. She had two similar events in the past, at ages 16 months and
22 months, both selfYlimited generalized convulsive seizures associated
with fever. No first-degree relatives have epilepsy. On examination, the child
is fussy but readily comforted by her parents. Her neurologic examination is
unremarkable. Which of the following medications should be recommended
to the family at this time?
A. daily oxcarbazepine
B. daily phenobarbital
C. daily valproic acid
D. oral diazepam when ill
E . rectal diazepam as needed for seizures lasting longer than 5 minutes
The preferred response is E (rectal diazepam as needed for seizures lasting
longer than 5 minutes). This patient has experienced three simple febrile
seizures. Of the options listed, rectal diazepam to be given in the event of
another seizure would be the most clearly indicated. There is no evidence
to suggest a daily anticonvulsant at this point given the potential for side
effects and the lack of evidence that any significant harm is prevented.
Oral diazepam administered only during illness may help to prevent febrile
seizures but has significant potential side effects and is difficult to justify
given the overall low likelihood of febrile seizure occurrence with any
given febrile illness. For more information, refer to pages 57Y58 of the
Continuum article Febrile Seizures.
b 3. A 32-year-old left-handed woman is referred from a sleep clinic after a
polysomnogram failed to reveal the cause of her disrupted sleep and daytime
somnolence. She reports a 7-year history of brief nocturnal episodes
described by her bed partner as focal jerking of her left arm lasting for 10 to
15 seconds and occurring 3 to 10 times per night. At times, the jerking will
spread from her left arm to her left face. No other features have ever been
seen. She does not distinctly recall the jerking but does know when she has
had more episodes because of more noticeable daytime sleepiness. While
these episodes were captured during the polysomnogram, the EEG montage
used was limited, and the waveforms were obscured by movement and
myogenic artifact. A routine interictal EEG was normal, and she has had a
normal MRI of the brain. Assuming that these episodes are epileptic, from
which of the following locations would such seizures most likely arise?
A. cingulate
B . dorsolateral frontal
C. frontopolar
D. insular
E . parietal

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The preferred response is B (dorsolateral frontal). The brevity and

nocturnal predilection of this patients seizures strongly suggest a frontal
localization, and the prominent hemiclonic symptomatology with spread to
contiguous areas of motor cortex points toward a dorsolateral frontal focus.
A normal interictal EEG is commonly seen in frontal lobe epilepsy. For
more information, refer to page 108 of the Continuum article Adult
Focal Epilepsies.
b 4. Which of the following antiepileptic drugs is most likely to be associated
with autoinduction of its own metabolism?
A. carbamazepine
B . eslicarbazepine acetate
C. lamotrigine
D. topiramate
E . valproate
The preferred response is A (carbamazepine). Carbamazepine induces
its own metabolism via hepatic clearance. This process of autoinduction
results in increased clearance with lower serum levels and shortened half-life
over the first month of administration. For more information, refer to
page 140 of the Continuum article Antiepileptic Drugs.
b 5. A 58-year-old man develops convulsive status epilepticus. The emergency
medical technicians are the first to assess and treat him. IV access proves
difficult to establish. Intramuscular injection of which of the following
medications has the most evidence to support its use as initial treatment?
A. diazepam
B . fosphenytoin
C. lorazepam
D. midazolam
E . phenobarbital
The preferred response is D (midazolam). The Rapid Anticonvulsant
Medications Prior to Arrival Trial (RAMPART) demonstrated superior efficacy
for IM midazolam compared with IV lorazepam. Other treatments that are
currently being used for prehospital treatment of status epilepticus include
intranasal and buccal midazolam and rectal diazepam. For more information,
refer to page 179 of the Continuum article Epilepsy Emergencies.
b 6. A 17-year-old girl with epilepsy seeks advice regarding methods of
contraception. She is currently treated with carbamazepine. Which of the
following methods of contraception would be optimal for this patient?
A. etonogestrel subdermal implant
B . intrauterine device
C. oral contraceptive pills
D. transdermal estrogen patch
E . vaginal ring

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The preferred response is B (intrauterine device). The intrauterine device (IUD)

is a highly reliable form of reversible contraception and is considered the
method of choice for most women with epilepsy. It has been recommended by
the American Academy of Pediatrics as the optimal method of contraception for
teenage girls. Enzyme-inducing antiepileptic drugs induce the metabolism of
estrogens and progestins and are associated with an increased rate of contraceptive
failure for oral contraceptive pills, the birth control patch, and the vaginal ring.
The efficacy of etonogestrel subdermal implants may also be reduced when
used in association with these antiepileptic drugs. For more information, refer to
pages 215Y217 of the Continuum article Managing Epilepsy in Women.
b 7. A 72-year-old man with Alzheimer disease but no other serious medical
conditions is brought by his family to the emergency department with ongoing
generalized convulsive seizure activity that began approximately 20 minutes
earlier and for which the patient has not yet received any treatment. He has
no history of epilepsy and is not taking an antiepileptic medication. Which of
the following IV medications would be best to administer first?
A. diazepam
B . levetiracetam
C. lorazepam
D. propofol
E . valproic acid
The preferred response is C (lorazepam). The best currently available evidence
indicates that lorazepam, phenytoin, and phenobarbital are all effective in
the treatment of generalized convulsive status epilepticus in elderly patients.
Limited data are available with regard to valproic acid, levetiracetam and
other newer agents. For more information, refer to page 248 of the Continuum
article Special Issues in Epilepsy: The Elderly, the Immunocompromised,
and Bone Health.
b 8. A 20-year-old previously healthy man presents to the emergency department
after having a 15-minute apparently generalized convulsive seizure. The patient
was awake and talking with friends when he suddenly let out a load groan, stiffened,
and fell to the floor with what was described as shaking and twitching of all
four limbs. Following the seizure, he was somnolent but arousable and gradually
returned to normal over the course of 1 hour. He had never had an episode
like this nor any events concerning for seizures in the past. An EEG performed
in the emergency department 3 hours after the event reveals only nonspecific
generalized theta slowing of the background without epileptiform features. An
MRI of the brain with and without contrast reveals scattered patchy nonenhancing
juxtacortical and periventricular T2 hyperintensities in the right hemisphere,
felt to be consistent with a remote injury. His neurologic examination 2 weeks
following the seizure is entirely normal. Which of the following clinical features
are associated with an increased risk of seizure recurrence in this patient?
A. age
B . EEG findings
C. MRI findings
D. seizure duration
E . seizure symptomatology

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The preferred answer is C (MRI findings). This patients MRI reveals findings
consistent with a remote brain injury, a feature that increases his risk of
recurrence. There is nothing about his age (20 years), seizure symptomatology,
or the seizure duration (15 minutes) that would increase this risk. His EEG
findings are nonspecific and, in the absence of focal features or epileptiform
activity, do not increase his risk of recurrence. For more information, refer to
pages 43Y45 of the Continuum article Management of a First Seizure.
b 9. A 6-month-old infant is admitted to the hospital with several minute-long
clusters of abnormal movements characterized by sudden axial flexion lasting
1 to 2 seconds occurring most commonly upon awakening. The movements
are stereotyped, and no focal features are noted. An EEG is performed, and
a cluster of events is captured. The interictal background is hypsarrhythmia,
and an electrodecrement is seen in association with the events. The patient
was born at term after an uncomplicated pregnancy and went home after
36 hours. She had been developing normally until these events started
approximately 3 weeks ago. Since that time she has stopped sitting up unaided
and is not rolling. There is no family history of seizures or neurodevelopmental
problems. On examination, she demonstrates poor visual attention and
hypotonia with no focal findings. A Woods lamp examination does not reveal
any suspicious skin findings. Her evaluation has thus far included an MRI,
which was normal, as well as normal serum lactate, serum amino acids, urine
organic acids, aspartate transaminase, alanine transaminase, and ammonia.
What is the most appropriate next diagnostic step?
A. comparative genomic hybridization array
B . CSF neurotransmitters
C. magnetic resonance spectroscopy
D. muscle biopsy
E . serum !-aminoadipic semialdehyde level
The preferred response is A (comparative genomic hybridization array).
This 6-month-old presents with a history and neurophysiologic findings
typical for West syndrome: epileptic spasms, hypsarrhythmia, and developmental
regression. Her initial evaluation thus far has been unrevealing. At this point,
genetic studies, starting with a comparative genomic hybridization array,
would be higher yield than further metabolic testing. A muscle biopsy would
not be indicated without further less-invasive investigations and other evidence
supportive of a mitochondrial process. This story would not be typical for
pyridoxine dependent epilepsy, the condition for which serum !-aminoadipic
semialdehyde testing would be useful. For more information, refer to
pages 61Y64 of the Continuum article Infantile, Childhood, and
Adolescent Epilepsies.

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b 10. A 15-year-old girl is being evaluated for recurrent episodes of sudden loss
of posture with jerking that have been increasing in frequency. A routine
awake/asleep EEG, during which an event was not captured, was normal.
A 24-hour video EEG study captured a single habitual event. Clinically, while
standing the patient looked briefly dazed and then slowly lost postural tone
and fell forward with 5 seconds of multifocal proximal and distal arrhythmic
jerks after hitting the floor. She was back to full consciousness within
40 seconds of the onset of the fall. The EEG during the event initially showed
theta slowing followed by generalized delta activity and then a brief period
of suppression before returning to her prior normal background. The family
reports that this episode was characteristic of her spells of concern. Which
of the following diagnoses is most likely in this patient?
A. atonic drop attacks
B . cataplexy
C. psychogenic nonepileptic seizures
D. syncope
E . tonic drop attacks
The preferred response is D (syncope). This patient had an event typical
of syncope, including the duration of 40 seconds and the presence of motor
symptoms. The EEG changes described were also typical of syncope. The
cause of syncope in this patient could be vasovagal or due to other causes
(eg, other cardiac causes). With psychogenic nonepileptic seizures, no EEG
changes (other than movement and myogenic artifact) would be seen. Atonic
and tonic drop attacks would have associated epileptiform EEG changes
and would not commonly occur in a patient with a normal interictal EEG.
For more information, refer to page 119 of the Continuum article
Diagnosis and Treatment of Nonepileptic Seizures.
b 11. A 15-year-old boy continues to have frequent primary generalized
tonic-clonic seizures despite trials of gabapentin and oxcarbazepine. He was
born at term after an uncomplicated pregnancy and has had no other significant
medical problems. He is doing well in regular classes in the 10th grade. An
MRI of the brain performed with and without contrast is normal, and an interictal
EEG reveals brief bursts of anterior dominant generalized spike and polyspike
and slow wave activity. His neurologic examination is entirely normal. What
would be the best next management step for this patient?
A. referral for ketogenic diet initiation
B . referral for vagus nerve stimulator placement
C. trial of carbamazepine
D. trial of lamotrigine
E . trial of pregabalin
The preferred response is D (trial of lamotrigine). This patient has primary
generalized tonic-clonic seizures, and the two medications that have been
tried are not efficacious for this condition. Therefore, he has not yet met
criteria for intractability, and a trial of an appropriate anticonvulsant such as
lamotrigine would be warranted, as well as a second appropriate medication
trial should the first medication prove ineffective. Neither carbamazepine nor

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pregabalin would be appropriate for primary generalized epilepsy. Vagus

nerve stimulation would be considered only in a patient who has medically
refractory epilepsy. For more information, refer to pages 157Y158 of the
Continuum article Management of Drug-Resistant Epilepsy.
b 12. Which of the following epilepsy localizations or etiologies is associated
with the highest frequency of anxiety disorders?
A. left mesial frontal lobe focus
B . left mesial temporal lobe focus
C. primary generalized epilepsy
D. right lateral temporal lobe focus
E . right orbitofrontal focus
The preferred response is B (left mesial temporal lobe focus). An increased
rate of many different mood disorders exists in people with epilepsy.
Depression is the most frequent psychiatric comorbidity, followed by various
anxiety disorders. A higher frequency of both anxiety disorders and psychosis
exists in patients with left mesial temporal lobe epilepsy compared with
other localization-related epilepsies or generalized epilepsy. For more
information, refer to pages 196Y200 of the Continuum article Management
of Epilepsy Comorbidities.
b 13. A 27-year-old woman presents with 2 weeks of behavioral changes,
hallucinations, and prominent memory impairment followed by the
development of generalized tonic-clonic seizures. Her status worsens
despite initiation of antiepileptic drugs. Brain MRI is normal. CSF is
remarkable for a mild lymphocytic pleocytosis. An extensive evaluation
yields the finding of N-methyl-D-aspartate (NMDA) receptor antibodies
in CSF. Her status continues to decline despite treatment with
high-dose corticosteroids and IVIg. Evaluation for malignancy is
negative. Which of the following treatment options is most appropriate
at this time?
A. azathioprine
B . cyclophosphamide or rituximab
C. methotrexate
D. mycophenolate mofetil
E . surgical hemispherotomy
The preferred response is B (cyclophosphamide or rituximab). First-line
treatments for autoimmune encephalitides include corticosteroids, IVIg, and
plasma exchange. However, cyclophosphamide and rituximab have been
reported to be effective as second-line treatments for patients with NMDA
receptor encephalitis and are being tried for other types of autoimmune
encephalitis. Surgical hemispherotomy is indicated for treatment of Rasmussen
encephalitis. For more information, refer to pages 241Y242 of the
Continuum article Autoimmune Epilepsy.

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b 14. Which of the following clinical presentations meets the 2014 International
League Against Epilepsy (ILAE) definition of epilepsy?
A. recurrent provoked seizures
B . a single provoked seizure
C. a single unprovoked seizure
D. a single unprovoked seizure with an estimated 60% or greater
risk of recurrence
E . two seizures 1 hour apart
The preferred response is D (a single unprovoked seizure with an estimated
60% or greater risk of recurrence). The 2014 ILAE definition of epilepsy
includes either recurrent unprovoked seizures at least 24 hours apart or a
heightened tendency toward recurrent unprovoked seizures with an estimated
risk of 60% or more of a recurrence in the next 10 years. One can have
recurrent provoked seizures without a diagnosis of epilepsy. A single
unprovoked seizure would not qualify for the diagnosis of epilepsy without
clinical, EEG, or imaging features that would indicate a heightened risk for
recurrence. For more information, refer to page 16 of the Continuum
article Diagnosis of Epilepsy and Related Episodic Disorders.
b 15. A three-and-a-half-year-old girl is brought to clinic with a history of three
febrile seizures: one at age 26 months and two on a single day approximately
1 month ago. Each seizure lasted around 10 minutes and was entirely
generalized in symptomatology. Both the seizure at 26 months and the two
more recent events were associated with a febrile viral syndrome with
maximal measured temperatures of 38.4-C (101.2-F). Her mother had a single
simple febrile seizure at age 2. The child has been neurodevelopmentally
normal, and her neurologic exam is entirely normal. Which of the following
features of this patients history would qualify her most recent febrile seizure as
complex rather than simple?
A. age over 3 years
B . duration of 10 minutes
C. family history of febrile seizures
D. low-grade fever at seizure onset
E . recurrence within 24 hours
The preferred response is E (recurrence within 24 hours). Febrile seizures
that are focal, prolonged (greater than 15 minutes), or recurrent within
24 hours are considered complex. Age is a consideration as to whether or not
a seizure can be described as a febrile seizure since this is an age-dependent
phenomenon (occurring between 6 months and 5 years) but does not relate
to the complex versus simple distinction. Temperature at the time of the seizure
is also not relevant to this categorization. Having had a complex febrile seizure
increases the risk of subsequent epilepsy, so appropriate classification is
important in prognostication. For more information, refer to page 53 of the
Continuum article Febrile Seizures.

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b 16. A 27-year-old right-handed woman has medically refractory seizures that

began 5 years ago. Her seizures begin with a strange sensation in her abdomen
followed 10 to 15 seconds later by loss of consciousness. Her mother reports
that during this period of unresponsiveness, the patient makes chewing
movements, often while picking at her shirt with her left hand. This is followed
by the patients right arm twisting up into a weird stiff posture. The mother
says she knows the seizure is over when the right arm untwists and the patient
wipes her nose with her left hand. These seizures last 1 to 2 minutes, occur
2 to 4 times per month, and are followed by 10 to 20 minutes of gradually
resolving postictal confusion. A recent EEG reveals spikes and sharp waves in
F7/T7 and F8/T8, which occur more frequently during drowsiness and sleep.
What is the most likely dominant seizure focus in this patient?
A. dominant lateral temporal lobe
B . dominant mesial temporal lobe
C. nondominant lateral temporal lobe
D. nondominant mesial temporal lobe
E . temporal pole
The preferred response is B (dominant mesial temporal lobe). The
symptomatology of this patients seizures is very typical of mesial temporal
lobe epilepsy: an epigastric aura followed by unresponsiveness with orofacial
and ipsilateral hand automatisms evolving to dystonic posturing of the
contralateral arm. Features that point to a dominant hemisphere focus include
unresponsiveness with prominent orofacial and manual automatisms.
Additionally, her left-sided manual automatisms, right-sided dystonic posturing,
and left-hand postictal nose wiping all point strongly to a left hemisphere origin.
The bilateral anterior temporal interictal activity strongly implicates the mesial
temporal structures. This is a common finding in mesial temporal lobe
epilepsy. For more information, refer to pages 95Y105 of the Continuum
article Adult Focal Epilepsies.
b 17. A 45-year-old woman developed focal seizures with secondary generalization
after a closed-head injury sustained during a motor vehicle accident. She was
started on lamotrigine monotherapy, which has resulted in a significant
reduction in seizure frequency but not complete control. Sometimes, she misses
a dose. The dose is titrated up to the point that she has developed side effects
of mild blurred vision. She does not wish to stop this medication. Of the
following medications, which one would be most appropriate to add to her
regimen as a second agent?
A. carbamazepine
B . eslicarbazepine
C. levetiracetam
D. oxcarbazepine
E . phenytoin
The preferred response is C (levetiracetam). All of the choices above except
for levetiracetam are medications that share a mechanism of action of
sodium channel blockade, the same mechanism of action as lamotrigine.
Antiepileptic drug combinations with different mechanisms of action may be

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more effective and less toxic than combinations of drugs with the same
mechanism of action. Levetiracetam has a different mechanism of action
(binding of synaptic vesicle protein SV2A) than these other agents and would
make a reasonable second agent in this setting. For more information, refer
to pages 152Y153 of the Continuum article Antiepileptic Drugs.
b 18. A 35-year-old woman with complex partial epilepsy well controlled on
lamotrigine presents with 3 months of worsening dysphoria, sleep disturbance,
weight gain, and anhedonia. Screening indicates moderate levels of depression.
Which of the following would be the most appropriate first-line treatment for
her depression?
A. amitriptyline
B . bupropion
C. doxepin
D. maprotiline
E . sertraline
The preferred response is E (sertraline). Depression is very common in
patients with epilepsy. The frequency of depression is reported to be 10%
to 20% in patients with well-controlled epilepsy and much more in those
whose epilepsy is poorly controlled. Selective serotonin reuptake inhibitors
(SSRIs) such as sertraline are first-line treatments for depression for patients
with epilepsy. SSRIs are unlikely to worsen seizure frequency and have few
significant interactions with epilepsy medications. Tricyclic antidepressants
are not recommended as first-line agents because of side effects and
drug-drug interactions with antiepileptic drugs, especially in children. However,
low doses are unlikely to increase seizure frequency in adults. Antidepressant
medications in the monoamine oxidase inhibitor and serotonin-norepinephrine
reuptake inhibitor classes are generally safe for use in patients with epilepsy.
Bupropion is well known to lower the seizure threshold and should be avoided
in patients with epilepsy. Electroconvulsive therapy can be utilized for
refractory depression. For more information, refer to pages 196Y198 of the
Continuum article Management of Epilepsy Comorbidities.
b 19. A 61-year-old man presents with new-onset seizures that have been
worsening over the past 2 months. His illness began with the onset of
recurrent brief contractions of his arm and face on one side or the other as
well as episodes marked by confusion and decreased responsiveness. He
recently experienced two generalized seizures. He presented to the emergency
department after the most recent seizures and was noted to have a serum
sodium level of 128 mEq/L. Which of the following antibodies is most likely
to be found on serum and CSF testing of this patient?
A. !-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) receptor
B . amphiphysin
C. CV2/collapsin response mediator protein-5 (CRMP-5)
D. GABA-A receptor
E . leucine-rich, glioma inactivated 1 (LGI1)

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The preferred response is E (leucine-rich, glioma inactivated 1 [LGI1]).

Antibodies directed against the components of the voltage-gated potassium
channel complex can cause a syndrome of limbic encephalitis. Awareness of
several distinctive clinical features can aid diagnosis of the syndrome of LGI1
antibodyYassociated encephalitis and initiation of immune treatment before
significant cognitive impairment develops. Faciobrachial dystonic seizures are
brief tonic contractions of the face and arm involving one side or alternating
sides of the body that are typical of this illness and are consistent with this
patients clinical presentation. Faciobrachial dystonic seizures occur early in
this illness, followed by the development of other features of limbic
encephalitis. Seizures may be partial, with altered cognition, or secondarily
generalized. Seizures may be associated with ictal autonomic manifestations
and hyponatremia due to the syndrome of inappropriate secretion of
antidiuretic hormone (SIADH). The other autoantibodies associated with
autoimmune encephalitis do not share these specific characteristics. For more
information, refer to page 229 of the Continuum article Autoimmune Epilepsy.
b 20. A 68-year-old woman with pneumonia has protracted bouts of coughing.
During one bout of coughing, she is witnessed falling to the floor and is
unresponsive to verbal stimuli for approximately 2 minutes. She has convulsive
movements of both arms for a few seconds. She maintains continence of her
bowel and bladder. She is rapidly oriented upon recovery of consciousness
and is without evidence of postevent confusion. If an EEG had been obtained
during this spell, what features would be mostly likely seen?
A. diffuse triphasic waves
B . focal cerebral slowing
C. generalized slowing or suppression
D. polymorphic delta activity
E . spike-wave discharges
The preferred response is C (generalized slowing or suppression). This
patients episode of altered consciousness is most consistent with syncope
induced by coughing. During a syncopal event, an EEG may demonstrate
generalized slowing or suppression if cerebral blood flow is significantly
reduced or interrupted. For more information, refer to page 20 of the
Continuum article Diagnosis of Epilepsy and Related Episodic Disorders.
b 21. A 76-year-old man with hypertension, hepatitis C, and chronic liver
dysfunction has had two focal seizures with secondary generalization thought
to be related to a remote cerebral infarction. Which of the following
antiepileptic medications would be best to try first in this patient?
A. carbamazepine
B . levetiracetam
C. phenobarbital
D. phenytoin
E . valproic acid
The preferred response is B (levetiracetam). Of the antiepileptic medications
listed, all but levetiracetam would be relatively contraindicated in a patient

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with significant liver disease given that they are hepatically metabolized. For
more information, refer to page 249 of the Continuum article Special Issues
in Epilepsy: The Elderly, the Immunocompromised, and Bone Health.
b 22. A 30-month-old boy is brought to clinic with a history of three febrile
seizures. All three lasted less than 10 minutes, appeared generalized, and
were associated with febrile upper respiratory infections. The first occurred at
18 months of age, and the next two occurred at 24 and 28 months. The
patients language development is delayed, with a current vocabulary of only
20 words and no two-word sentences. Besides receptive and expressive
language, his neurologic examination is normal. No history of febrile seizures
or epilepsy exists in the family. Which of the following clinical features places
this patient at higher risk for the development of epilepsy?
A. age at time of first febrile seizure
B . generalized symptomatology
C. history of developmental delay
D. male sex
E . recurrent febrile seizures
The preferred response is C (history of developmental delay). The only
feature of this patients history that clearly places him at higher risk of
developing epilepsy is his history of developmental delay. His febrile seizures
have all been simple: less than 15 minutes, generalized, and not recurrent
within 24 hours. While four or more febrile seizures may be related to an
increased risk of developing epilepsy, no evidence exists that three simple
febrile seizures increases the risk. The age at which his first febrile seizure
occurred (18 months) is at the peak age of incidence (18 to 24 months) and
would not be considered an additional risk factor for the development
of epilepsy. No evidence shows that sex plays a role in the risk of epilepsy in
patients with febrile seizures. For more information, refer to pages 55Y57
of the Continuum article Febrile Seizures.
b 23. A 27-year old-woman has completed 2 days of a planned 5-day video
EEG monitoring unit admission without yet capturing one of her habitual
episodes. Her events of concern are strongly suspected to be psychogenic
nonepileptic seizures given self-reported symptoms of frequent prolonged
hypermotor events with a waxing and waning character, crying during the
event, preservation of consciousness throughout the event, and minimal
postevent confusion. She has had a normal interictal awake and asleep
EEG without activation procedures, and a normal MRI in the past. What
would be the best approach to improve the chances of capturing a typical
event during the remaining 3 days of her admission?
A. perform a normal saline induction
B . perform hyperventilation and photic stimulation
C. tell the patient, erroneously, that the video is not working
D. tell the patient that the study will be ending in 12 hours
E . withhold a dose of the patients fluoxetine

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The preferred response is B (perform hyperventilation and photic

stimulation). Given this patients hypermotor symptomatology and lack
of prior activation procedures, the chance of successful induction with
standard hyperventilation is good. Performing a normal saline induction or
telling the patient that the video is not working would be deceptive and are
therefore ethically questionable. No evidence exists that suggesting a
premature end to the study or withholding a dose of antidepressant
medication would facilitate event capture, and these options are also ethically
problematic. For more information, refer to pages 119Y120 of the Continuum
article Diagnosis and Treatment of Nonepileptic Seizures.
b 24. A 13-year-old girl continues to have frequent primary generalized
tonic-clonic seizures despite adequate trials of lamotrigine, valproic acid,
zonisamide, and levetiracetam. She was born at term after an uncomplicated
pregnancy and has had no other significant medical problems. She has
required some assistance with reading but is otherwise in regular classes. An
MRI of the brain performed with and without contrast is normal. A recent
video-EEG monitoring study captured several typical seizures with generalized
onset and interictal bursts of anterior-dominant generalized spike and polyspike
and slow wave activity. Her neurologic examination is entirely normal. What
would be the best next management step for this patient?
A. corpus callosotomy
B . responsive neurostimulator placement
C. trial of oxcarbazepine
D. trial of vigabatrin
E . vagus nerve stimulator placement
The preferred response is E (vagus nerve stimulator placement). This
patient has medically refractory primary generalized epilepsy with tonic-clonic
seizures. Of the options listed, vagus nerve stimulator placement is most
likely to be efficacious. Responsive neurostimulation is indicated for focal and
not generalized epilepsy. Corpus callosotomy would usually best be reserved
for atonic seizures. Vigabatrin and oxcarbazepine are not efficacious in primary
generalized epilepsy. For more information, refer to pages 165Y166 of the
Continuum article Management of Drug-Resistant Epilepsy.
b 25. Which of the following is the most frequent epilepsy-related cause of death?
A. drowning
B . falls
C. motor vehicle accidents
D. status epilepticus
E . sudden unexpected death in epilepsy
The preferred response is E (sudden unexpected death in epilepsy).
Sudden unexpected death in epilepsy (SUDEP) is the most frequent of
the epilepsy-related causes of death, and the incidence is more than 20 times
that of sudden death in the general population. The risk of falls, drowning,
and other accidents are increased in people with epilepsy and require
counseling about risks and precautions to reduce risk. The relative risk of being
involved in a motor vehicle accident has been estimated to be as high
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as 2.0 in people with epilepsy compared with the general population.

People with epilepsy also have higher rates of cardiovascular, respiratory,
and inflammatory disorders that may lead to death. For more information,
refer to pages 191Y192 of the Continuum article Management of
Epilepsy Comorbidities.
b 26. A delta brush pattern on EEG is associated with autoimmune encephalitis
related to antibodies to which of the following?
A. amphiphysin
B . contactin-associated protein-like 2 (Caspr2)
C. GABA-B receptor
D. Hu
E . N-methyl-D-aspartate (NMDA) receptor
The preferred response is E (N-methyl-D-aspartate [NMDA] receptor).
NMDA receptor encephalitis is associated with a specific EEG feature
of extreme delta brushes in approximately half of cases. For more
information, refer to pages 229Y233 of the Continuum article
Autoimmune Epilepsy.
b 27. A 7-year-old neurodevelopmentally normal boy is brought to the
emergency department after a flurry of three tonic-clonic seizures over the
course of 2 hours at home. He was somnolent and confused after each
seizure for a few minutes but has a normal neurologic examination by the
time he reaches the emergency department. Careful history taking elucidates
no prior events concerning for seizures. He was born at term without
complications. An MRI of the brain and an EEG obtained 2 weeks later are
unremarkable. What is the approximate risk of seizure recurrence in this child?
A. 5%
B . 10%
C. 30%
D. 70%
E . 90%
The preferred response is C (30%). This patient had three convulsive
seizures within a 2-hour period. However, the fact that multiple seizures
occurred does not change his risk of seizure recurrence. Given his normal
development, neurologic examination, MRI, and EEG, his risk of recurrence
is approximately 30% to 40%. If all of those clinical factors were abnormal,
his recurrence risk would be closer to 90%. Given a single event that did not
fit into a defined electroclinical syndrome and a low risk of recurrence
(well below 60%), he would not meet current diagnostic criteria for epilepsy.
For more information, refer to pages 39Y40 of the Continuum article
Management of a First Seizure.

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b 28. A 7-year-old boy is seen in clinic for a recent decline in school

performance. He has had epilepsy for a few years, with brief hemiconvulsive
seizures, managed with carbamazepine monotherapy with a seizure every
2 to 3 months. Nothing has recently changed in regard to his seizures or his
carbamazepine dosing. He has been a neurodevelopmentally normal child,
doing well in regular classes until the past several months. His teachers report
that he is often inattentive and seems to just stare off. He can be redirected
and is not unresponsive. His schoolwork has suffered, with concern that he
may need to repeat second grade. An EEG is performed, which continues to
show right centroparietal spike wave discharges, as has been seen in prior
EEGs, but now also shows significant increase in the frequency of occurrence
of these discharges during sleep, occupying approximately 90% of epochs.
What would be the best therapeutic intervention at this point?
A. add pyridoxine
B . add vigabatrin
C. change to levetiracetam
D. change to oxcarbazepine
E . change to phenobarbital
The preferred response is C (change to levetiracetam). This patients story
is concerning for electrical status epilepticus in slow sleep (ESES) and
particularly for continuous spike and wave in slow sleep (CSWS).
The child had a fairly unremarkable history of focal epilepsy but now has
significant cognitive clouding in association with nonYrapid eye movement
sleepYrelated potentiation of interictal activity. The carbamazepine that the
child is taking can exacerbate this condition, as could oxcarbazepine.
Levetiracetam has shown benefit in small case series as have several other
anticonvulsant drugs. High-dose diazepam given at night is also often used
as a first-line agent in this condition, as are oral steroids. Phenobarbital does
not have demonstrated efficacy nor does pyridoxine. Vigabatrin would properly
be considered in the treatment of infantile spasms for a patient with
tuberous sclerosis. For more information, refer to page 73 of the
Continuum article Infantile, Childhood, and Adolescent Epilepsies.
b 29. A 27-year-old man presents to clinic with a 3-year history of episodes of
shaking and unresponsiveness that are triggered by fluorescent lights, but only
those at work. The onset of the convulsive activity is sudden, with each event
lasting approximately 30 seconds, and they usually occur once per month.
During times of significant emotional stress, the episodes can occur much more
frequently, up to 2 times per week. Which of the following features of this
patients history would be more consistent with psychogenic nonepileptic
seizures than with epileptic seizures?
A. duration
B . exacerbation by emotional stressors
C. frequency
D. nature of specific trigger
E . sudden onset
The preferred response is D (nature of specific trigger). While reflex
epilepsies can occur, seizures triggered by fluorescent lights only in one
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particular context would be highly unusual. Other elements of the patients

history, however, are consistent with epileptic seizures: the duration
of the events is brief, the frequency is low but increases during times of
emotional stress, and the onset of the event is sudden rather than
a gradual crescendo. For more information, refer to page 117 of the
Continuum article Diagnosis and Treatment of Nonepileptic Seizures.
b 30. A 35-year-old right-handed man is referred for a neurologic consultation
because of seizures characterized by a sensation of laryngeal constriction and
choking associated with clouding of consciousness. No other manifestations
have been noted by either the patient or his wife, who has witnessed many
typical events. These seizures last for 1 to 2 minutes and occur 1 to 3 times
per week. From which of the following locations would such seizures most
likely arise?
A. insula
B . mesial temporal lobe
C. occipital lobe
D. parietal lobe
E . supplementary sensory motor area
The preferred response is A (insula). The symptoms of laryngeal constriction
with choking sensations would be most typically seen with insular seizures.
Mesial temporal seizures usually involve a typical aura with dyscognitive features
as well as orofacial and manual automatisms. Occipital seizures may involve
visual phenomena, while parietal seizures may have somatosensory auras
followed by a wide variety of ictal manifestations depending upon the region
to which the abnormal activity spreads. Supplementary sensory motor area
seizures typically manifest as asymmetric tonic posturing. For more information,
refer to page 113 of the Continuum article Adult Focal Epilepsies.
b 31. An 8-year-old boy is brought to the emergency department after
awakening with right mouth tingling followed by about 1 minute of right
facial twitching and drooling as well as difficulty speaking. He had fallen
asleep on the couch next to his mother, and she witnessed the entire
episode, reporting that it began not long after he began to doze. Immediately
following the seizure, he was somnolent but not confused. He was born at
term with no complications. He did have one other concerning episode
2 years ago in which he awoke to find that he had wet the bed and bitten
his tongue. He has otherwise developed normally and has always done very
well in school. His neurologic examination is entirely normal. An EEG is
obtained 1 week later. Given this childs history, which of the following
EEG findings would be most likely?
A. anterior-dominant generalized 3-Hz spike and slow-wave bursts
B . centrotemporal spike-and-slow-wave discharges
C. generalized paroxysmal fast activity
D. generalized paroxysmal 4-Hz to 6-Hz polyspike and slow-wave bursts
E . intermittent centroparietal theta rhythm

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The preferred response is B (centrotemporal spike-and-slow-wave discharges).

This patients history is strongly suggestive of benign epilepsy with
centrotemporal spikes (also known as benign rolandic epilepsy), in
particular the hemifacial sensory and motor manifestations, occurrence
around sleep onset, age of onset, and infrequent occurrence. An EEG would
be most likely to show centrotemporal spikes with associated slow waves.
One would also expect to see a frontal dipole in association with these
transients. Anterior dominant generalized 3-Hz spike and slow-wave
bursts would be an interictal finding seen in childhood absence epilepsy,
while generalized paroxysmal fast activity is often seen in Lennox-Gastaut
syndrome, neither of which fits this patients history. Generalized paroxysmal
4-Hz to 6-Hz polyspike and slow-wave bursts are seen in juvenile myoclonic
epilepsy. Intermittent centroparietal rhythmic theta activity, while a nonspecific
finding, can be seen in Panayiotopoulus syndrome, another benign focal
epilepsy of childhood that would be typically associated with seizures having
prominent autonomic features. For more information, refer to pages 69Y70 of
the Continuum article Infantile, Childhood, and Adolescent Epilepsies.
b 32. A 6-year-old boy with Lennox-Gastaut syndrome continues to have
frequent generalized seizures despite adequate trials of several appropriate
medications. A vagus nerve stimulator was placed 1 year ago and has
significantly reduced his atonic drop seizures as well as his atypical absence
seizures. The patient is significantly cognitively impaired and has a
gastrostomy tube. An MRI reveals mild to moderate global atrophy. A recent
video-EEG study captured several of his typical generalized tonic and
generalized tonic-clonic seizures, revealing a generalized pattern at onset as
well as a slow and disorganized waking background with frequent multifocal,
bisynchronous, and generalized interictal epileptiform spikes. What would
be the best next management step for this patient?
A. corpus callosotomy
B . ketogenic diet
C. resective surgery evaluation
D. responsive neurostimulator placement
E . trial of cannabidiol
The preferred response is B (ketogenic diet). This patient has Lennox-Gastaut
syndrome with persistent generalized tonic and generalized tonic-clonic
seizures despite adequate medication trials and vagus nerve stimulator
placement. Given this constellation of findings, the ketogenic diet would be
the option most likely to result in a meaningful reduction in his seizure
frequency. Use of the diet would be facilitated by the fact that the patient has a
gastrostomy tube. Corpus callosotomy would be best considered for a patient
with atonic drop attacks, which have already significantly diminished for this
patient. Given the global MRI changes and the generalized onset of the
patients seizures, no clear role exists for resective surgery or a responsive
neurostimulation. Marijuana derivatives such as cannabidiol, while
interesting potential treatments, do not currently have sufficient evidence
to be recommended. For more information, refer to page 169 of the
Continuum article Management of Drug-Resistant Epilepsy.
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b 33. Which of the following antiepileptic medications is associated with the

highest rates of major congenital malformations?
A. carbamazepine
B . lamotrigine
C. levetiracetam
D. topiramate
E . valproate
The preferred response is E (valproate). Valproate has been associated with
the highest rates of major congenital malformations in multiple registries and
studies, with frequencies ranging from approximately 5% to 14%. Neural tube
defects are the most common major malformation seen with valproate;
however, rates of cardiac defects, clefts, and hypospadias are also highest
for valproate when compared with the other drugs listed. Lamotrigine,
levetiracetam, and carbamazepine have relatively lower rates of associated
malformations. More research is needed on the risk of major malformations
with topiramate; however, recent research suggests the risk is higher than
these three drugs but likely lower than the risk seen with valproate. In addition,
exposure to valproate during pregnancy has been associated with a decrease
in mean IQ and an increased risk of autism. For more information, refer to
pages 205Y208 of the Continuum article Managing Epilepsy in Women.
b 34. A 42-year-old woman is being treated for a malignant glioma. She
initially presented with focal seizures and headache, but her seizures had
been entirely controlled on phenytoin monotherapy with levels in the middle
of the therapeutic range. Recent imaging performed because of increased
somnolence and worsening headache revealed tumor extension into the
corpus callosum with edema, so she was started on dexamethasone. Five
days later, she has a series of breakthrough seizures identical to her initial
seizures. Her examination is unchanged, and she is afebrile with no nuchal
rigidity. What is the most likely etiology of these breakthrough seizures?
A. central nervous system infection
B . change in seizure type
C. drug-drug interaction
D. need for a second antiepileptic
E . tumor expansion
The preferred response is C (drug-drug interaction). This patients
breakthrough seizures are likely to be caused by a drug-drug interaction between
dexamethasone and phenytoin. Dexamethasone may reduce serum phenytoin
levels. The patients seizure symptomatology has not changed, making a new
type of seizure unlikely. There is no evidence at present of a central nervous
system infection, although this would always be important to consider in an
immunocompromised patient. While the patient may eventually need a
different antiepileptic agent, there is no reason yet to declare phenytoin a
failure given initial treatment success with serum levels in the middle of the
therapeutic range. Finally, her seizures are unlikely to be due to extension of the
tumor into deep subcortical white matter structures. For more information,
refer to page 255 of the Continuum article Special Issues in Epilepsy:
The Elderly, the Immunocompromised, and Bone Health.

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b 35. An 11-year-old neurodevelopmentally normal girl is brought to clinic after

having had a single 15-minute generalized tonic-clonic seizure at night that
began during sleep and which stopped without intervention. She was born
at term without complications and has an unremarkable medical history.
Her neurologic examination is entirely normal, as is an MRI of the brain
performed with and without contrast. An EEG performed 2 hours after the
seizure demonstrates a mildly and diffusely slow background for the patients
age but is otherwise within the range of normal for age. Which of the
following clinical features of this patients story are associated with
an increased risk for seizure recurrence?
A. age
B . EEG findings
C. occurrence at night
D. seizure duration
E . seizure symptomatology
The preferred response is C (occurrence at night). The fact that the
seizure occurred at night during sleep increases the risk of recurrence.
Age has not been shown to be an independent risk factor for recurrence.
A seizure duration of 15 minutes, although longer than most seizures,
does not represent status epilepticus and would not increase the risk of
recurrence. Furthermore, status epilepticus has been shown to increase
recurrence risk in patients with remote symptomatic etiologies which
would not appear to be relevant to this neurodevelopmentally normal
child with a normal brain MRI. The apparently generalized nature of the
seizure does not alter recurrence risk nor do the EEG findings of mild
postictal generalized slowing. For more information, refer to pages 45Y46
of the Continuum article Management of a First Seizure.
b 36. A 10-month-old boy who has had febrile otitis media for 2 days is brought
to the emergency department following a 25-minute generalized convulsive
seizure soon after spiking a fever to 39.7-C (103.5-F). He is now somnolent
but arousable, with an otherwise normal examination. His past medical
history has been unremarkable to date and no history of seizures exists in
the immediate family. Which of the following clinical features places this
patient at higher risk of febrile seizure recurrence?
A. age
B . duration longer than 15 minutes
C. generalized symptomatology
D. high temperature at time of seizure
E . no family history of febrile seizures
The preferred response is A (age). This patient has presented with his
first febrile seizure at the age of 10 months, a relatively young age that puts
him at increased risk for recurrence. Seizure duration is not predictive of
recurrence, and generalized symptomatology would be more reassuring
than a focal presentation. A low-grade fever at the time of the seizure would
indicate a higher recurrence risk, as would a family history of febrile seizures.
For more information, refer to page 55 of the Continuum article
Febrile Seizures.
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b 37. A 4-year-old boy is brought to the epilepsy clinic for evaluation and
management of multiple seizure types that began approximately 1 year ago
with tonic seizures occurring at night. Soon after these seizures began, he
developed atonic seizures and occasional myoclonic seizures. He has had
several generalized tonic-clonic seizures with one requiring intranasal
midazolam because of a duration longer than 5 minutes. He was born at term
without complications but has developed slowly, in particular with regard
to language. However, while his development seems to have plateaued, no
evidence of regression exists. His examination is remarkable only for abnormal
expressive language, which is poorly articulated with a limited vocabulary. EEG
demonstrates a diffusely slow background with high-amplitude 1.5-Hz to 2.5-Hz
generalized spike and polyspike and slow-wave discharges on the awake EEG
as well as frequent bursts of generalized paroxysmal fast activity during sleep.
Which of the following is the most likely diagnosis?
A. Dravet syndrome
B . Lennox-Gastaut syndrome
C. myoclonic atonic epilepsy
D. Panayiotopoulos syndrome
E . progressive myoclonic epilepsy
The preferred response is B (Lennox-Gastaut syndrome). This patients history
is typical of patients with Lennox-Gastaut syndrome. Particularly suggestive
are the abnormal development at baseline, multiple generalized seizure types,
and EEG findings of generalized paroxysmal fast activity in sleep, along with
slow spike and slow wave upon a diffusely slow background in wakefulness.
In Dravet syndrome, the seizures begin earlier and are usually febrile and
hemiconvulsive at first. Also, as in myoclonic atonic epilepsy, development is
typically normal until the seizures start, without preceding developmental
delay. Panayiotopoulos syndrome is generally seen in neurodevelopmentally
normal children and is most often characterized by prolonged seizures with
prominent autonomic features. Progressive myoclonic epilepsy is a class of
disorders that share the features of progressive decline, myoclonic epilepsy, and,
often, ataxia. For more information, refer to pages 76Y78 of the Continuum
article Infantile, Childhood, and Adolescent Epilepsies.
b 38. A 23-year-old man with focal epilepsy is referred for persistent seizures
despite trials of two antiepileptic medications. He was first tried on an
appropriate dose of oxcarbazepine without seizure freedom, despite good
compliance. Levetiracetam was tried next, but the patient was unable to tolerate
doses larger than the starting dose because of significant irritability. MRI of his
brain with and without contrast is normal, and a recent EEG revealed right
parietooccipital epileptiform sharp waves. His neurologic examination is
normal. What would be the best next management step for this patient?
A. referral for resective epilepsy surgery
B . referral for responsive neurostimulator placement
C. referral for vagus nerve stimulator placement
D. trial of another antiepileptic agent
E . trial of cannabidiol

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The preferred response is D (trial of another antiepileptic agent).

Although this patient has tried two antiepileptic drugs with demonstrated
efficacy for focal seizures, the trial of levetiracetam was limited by side
effects. For this reason, the patient cannot yet be considered medically
refractory, and a trial of another antiepileptic drug is warranted. Vagus nerve
stimulation and responsive neurostimulation do not yet have a role in
this patient given that it is not yet clear that his epilepsy is medically
refractory or if it might be surgically amenable. Marijuana derivatives such
as cannabidiol, while interesting potential treatments, do not currently
have sufficient evidence to be recommended. For more information,
refer to pages 157Y160 of the Continuum article Management of
Drug-Resistant Epilepsy.
b 39. A 42-year-old man is being evaluated for convulsive seizures that have not
abated with adequate trials of three well-chosen antiepileptic agents. His
spouse shows a video of a typical event from the onset of the clinically
manifest stage to completion, which reveals findings typical of psychogenic
nonepileptic seizures (PNES). His neurologic examination is entirely normal.
A routine EEG is performed without video and his typical clinical event is
captured, provoked by hyperventilation, without evident epileptiform activity.
The patient and his wife now return for a follow-up visit. What would be the
best next step in this patients management?
A. offer a trial of another antiepileptic medication before making an
affirmative diagnosis
B . order a video EEG monitoring unit study to confirm the diagnosis before
discussing PNES with the patient
C. provide the patient with an affirmative diagnosis and explanation of PNES
D. refer the patient to a psychiatrist, who will be better able to convey
the diagnosis
E . refer to a social worker to assess for evidence of primary gain and malingering
The preferred response is C (provide the patient with an affirmative
diagnosis and explanation of PNES). This patient meets criteria for clinically
established PNES; therefore, providing the patient with an affirmative
diagnosis and explanation would be the best next step. There is no reason
to await video EEG monitoring or to obtain other consultations before
communicating this diagnosis. Referral to a psychiatrist and social worker may
be appropriate but not for communicating or making the diagnosis. With
regard to assessing for evidence of feigning symptoms, this is no longer a
component of the diagnostic criteria for PNES. For more information,
refer to page 121 of the Continuum article Diagnosis and Treatment
of Nonepileptic Seizures.

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b 40. Which of the following systemic complications is most commonly

described as a complication of long-term use of phenobarbital?
A. decreased bone density
B . hyperammonemia
C. hyponatremia
D. peripheral edema
E . thrombocytopenia
The preferred response is A (decreased bone density). Decreased bone
density may be associated with long-term use of phenobarbital, as are
Dupuytren contractures, plantar fibromatosis, joint pain, and frozen shoulder.
Common adverse effects include sedation, mood changes, and decreased
concentration/attention. Because of these common side effects, phenobarbital
is infrequently used as first-line therapy in the United States. Hyperammonemia
is more likely to occur with valproate or topiramate. Hyponatremia is a
well-described side effect of carbamazepine or oxcarbazepine treatment.
Peripheral edema is a side effect of pregabalin, gabapentin, and valproate.
Thrombocytopenia is a dose-dependent adverse effect with valproate.
For more information, refer to pages 132Y134 of the Continuum article
Antiepileptic Drugs.

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