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1. Which of the following antiepileptic drugs C. The audiogenic seizure model in mice
would be the best initial choice in an adoles- predicts efficacy against focal seizures
cent girl with juvenile myoclonic epilepsy? D. Levetiracetam was effective in both MES
and PTZ models
A. Carbamazepine
E. The 6 Hz model is predictive of efficacy
B. Pregabalin
against absence seizures
C. Lacosamide
D. Valproic acid
4. The cooperative VA studies showed:
E. Levetiracetam
A. Carbamazepine and phenytoin were
2. A 26-year-old female had frequent episodes
overall more efficacious than phenobar-
of focal left hand shaking followed by gen-
bital and primidone
eralized tonic–clonic seizure activity. On
B. Phenobarbital and primidone were less
two occasions, she broke her jaw and her
well tolerated than carbamazepine and
right shoulder. Routine EEG is normal, and
phenytoin
brain imaging is unremarkable. Which
C. Phenobarbital was more efficacious than
would be most appropriate antiepileptic drug
primidone
for this patient?
D. Primidone was better tolerated than
A. Oxcarbazepine phenobarbital
B. Lamotrigine E. Primidone was more efficacious than
C. Topiramate phenobarbital
D. Phenobarbital
E. Valproate 5. Which of the following is not a 1,4
benzodiazepine?
3. Which is true about animal models of
epilepsy? A. Diazepam
B. Clonazepam
A. The PTZ model helps predict efficacy C. Lorazepam
against absence seizures D. Clobazam
B. The kindling model typically has spon- E. Clorazepate
taneously recurring seizures
254 Multiple Choice Questions for Part IV
6. Which of the following benzodiazepines has 11. Urinary retention is most likely to occur with
important active metabolites?
A. Perampanel
A. Clonazepam B. Levetiracetam
B. Clobazam C. Ezogabine
C. Diazepam D. Felbamate
D. B and C E. Pregabalin
E. All the above
12. Which is not likely to happen with the
7. Dupuytren’s contractures and plantar fibro- addition of felbamate?
matosis are chronic adverse effects of
A. Increased phenobarbital level
A. Tiagabine B. Increased phenytoin level
B. Phenobarbital C. Increased carbamazepine level
C. Vigabatrin D. Increased valproate level
D. Clobazam E. Toxicity related to carbamazepine epoxide
E. Clorazepate
13. A 4-month-old infant is diagnosed with
8. Simvastatin efficacy is reduced with: infantile spasms. He did not respond to
2-week course of ACTH at 150 units/m2.
A. Lamotrigine Which of the following antiepileptic medi-
B. Carbamazepine cations would you use next?
C. Levetiracetam
D. Pregabalin A. Vigabatrin
E. Ezogabine B. Levetiracetam
C. Topiramate
9. An adolescent boy with epilepsy presents D. Clobazam
with overheating and fever following exer- E. Tiagabine
cise. The likely cause is as follows:
14. Irritability is most common with the fol-
A. Levetiracetam lowing AEDs:
B. Lamotrigine
C. Zonisamide A. Lamotrigine and oxcarbazepine
D. Valproate B. Perampanel and levetiracetam
E. Phenobarbital C. Felbamate and zonisamide
D. Phenobarbital and primidone
10. The following antiepileptic drug is FDA E. Valproate and ethosuximide
approved for once-daily dosing:
15. Insomnia is most likely with which of the
A. Perampanel following?
B. Zonisamide
C. Ethosuximide A. Gabapentin
D. Clobazam B. Pregabalin
E. Topiramate C. Ezogabine
D. Perampanel
Multiple Choice Questions for Part IV 255
36. Which of the following is unlikely to exac- 41. Screening for potential psychosocial barriers
erbate absence seizures? to the safety and success of the ketogenic
diet should take place before initiation of
A. Carbamazepine therapy. Which of the following features
B. Oxcarbazepine makes the ketogenic diet a better/easier
C. Valproate treatment option?
D. Tiagabine
E. Vigabatrin A. Presence of a gastrostomy tube
B. Severe failure to thrive/inability to
37. Which of the following antiepileptic drug is maintain adequate nutrition
not known to modulate GABA? C. Potential surgically resectable seizure
focus
A. Phenobarbital D. Multiple siblings in an unstructured
B. Tiagabine home environment
C. Clonazepam E. Available premanufactured formulas
D. Pregabalin
E. Topiramate 42. Prolonged episodes of altered responsive-
ness are most likely with:
38. For which of the following conditions is the
ketogenic diet indicated? A. Tiagabine
B. Perampanel
A. Primary carnitine deficiency C. Vigabatrin
B. Pyruvate carboxylase deficiency D. Clobazam
C. Pyruvate dehydrogenase deficiency E. Pregabalin
D. Porphyria
E. None of the above
258 Multiple Choice Questions for Part IV
43. Screening for disorders of fatty acid meta- D. Deficiency of calcium and vitamin D,
bolism should be performed prior to initia- leading to bone mineralization loss
tion of the ketogenic diet. Specifically, this E. Encephalopathy due to hypoglycemia,
testing could include which of the dehydration, and excessive acidosis
following?
46. Epilepsy is most commonly associated with:
A. Complete blood count and complete
metabolic panel including liver function A. Dementia
tests and BUN and creatinine B. Stroke
B. Acylcarnitine profile, urine organic acids, C. Birth defect
and carnitine D. Bipolar disorder
C. CSF glucose, lactate, folate metabolites, E. Psychosis
amino acids, and neurotransmitters
D. Kidney ultrasound and nephrology 47. There is a need for more research and data
consult regarding the incidence and prevention of
long-term complications in patients who
44. A 23-year-old man with idiopathic general- have been on the ketogenic diet for long
ized epilepsy was initially maintained on durations, for instance greater than 5 years.
lamotrigine. Valproate was added as adjunct Which of the following tests would be rea-
therapy. Which of the following effects sonable to perform in such a patient, who is
may result from interaction between these otherwise asymptomatic?
drugs?
A. Blood draw for amylase, lipase
A. Lamotrigine toxicity because of glu- B. Renal ultrasound looking for renal stone
curonidation inhibition C. Abdominal X-ray for stool
B. Decreased lamotrigine efficacy due to D. Blood draw for 25-hydroxy-vitamin D
inhibition of cytochrome 2C9 and 2C19 and consideration of bone density scan
C. Decreased valproate efficacy due to E. CRP
induction of CYP2C9
D. Increased risk of liver disease 48. The literature supports the probable benefit
E. Valproate toxicity due to inhibition of its of the ketogenic diet in which of the fol-
b-oxidation lowing conditions?
45. With rare exceptions, the ketogenic diet is A. Benign myoclonus of infancy
initiated during an inpatient hospitalization. B. Juvenile myoclonic epilepsy
Complications during the initiation period C. Glucose transporter protein 1 deficiency
could include all of the below, except: D. Pyruvate carboxylase deficiency
A. Vomiting due to hypoglycemia, dehy- 49. A 36-year-old male from Nepal is evaluated
dration, excessive acidosis, constipation, for a generalized tonic seizure. Few weeks
or exacerbation of gastroesophageal ago, he started taking rifampin and isoniazid
reflux for a positive tuberculin test. His neurolog-
B. Precipitation of deterioration in a patient ical examination, EEG, and brain MRI are
with an undiagnosed disorder of fat all normal. What is the likely mechanism
metabolism responsible for his seizure?
C. Excessive metabolic acidosis in a patient
also treated with a carbonic anhydrase A. Reversible inhibition of GABA reuptake
inhibitor B. Impaired pyridoxine synthesis
Multiple Choice Questions for Part IV 259
76. Which is not true about vigabatrin’s visual 81. Which antiepileptic drug blocks T-type cal-
defect? cium channel:
International Ketogenic Diet Study Group’s epilepsy syndromes showed that only
recommendation? 20% had a greater than 75% decrease in
seizures
A. The ketogenic diet should be considered D. There are currently no randomized con-
in a patient whose epilepsy is controlled trolled trials published on the efficacy of
by a single medication, but whose family dietary therapies in epilepsy treatment
prefers “natural” therapies
B. The ketogenic diet should be considered 88. Which antiepileptic drug should be avoided
only as a last resort, since it is hard to in a patient with known sulfa drug allergy?
administer, is unpalatable, and there is no
data from randomized controlled trials to A. Pregabalin
support it B. Zonisamide
C. The ketogenic diet should be considered C. Carbamazepine
in a child who has failed 2–3 anticon- D. Vigabatrin
vulsant therapies, as long as he/she is E. Lamotrigine
older than 1 year and younger than
12 years of age 89. The following statement is false:
D. The ketogenic diet should be considered
in a child who has failed 2–3 anticon- A. A common ratio of the ketogenic diet is
vulsant therapies, regardless of age or 4 g of fat to 1 g of protein plus carbo-
gender hydrate (4:1)
B. In a 4:1 ratio ketogenic diet, approxi-
86. Brivaracetam has the following mechanism mately 90% of energy comes from fat
of action: C. A fasting period during initiation of the
ketogenic diet is necessary to achieve
A. Potassium channel opening ketosis
B. Blocking of sodium channels D. The modified Atkins diet is approxi-
C. Binding to the synaptic vesicle protein mately a 1:1 ratio
D. Binding to the GABAA receptor E. In the low glycemic index treatment,
E. Binding to the synaptic vesicle protein foods are chosen which produce
and blocking of sodium channels slower/steadier changes in blood glucose
87. There has been growing interest and confi- 90. A 72-year-old man is seen in clinic for a new
dence in the use of the ketogenic diet and diagnosis of epilepsy, with recurrent partial
subsequently a growing body of literature. seizures secondary to a right MCA ischemic
Which of the following statements is true? infarct. His medical history includes chronic
afib (for which he takes warfarin), osteo-
A. Four randomized controlled trials porosis, and a history of kidney stones.
showed that at least 38% of patients had Which of the following is the most appro-
a 50% reduction in seizures compared to priate antiepileptic medication for this
controls at 3 months, with this response patient?
maintained for up to a year
B. A meta-analysis has shown that a third of A. Carbamazepine
all patients on the ketogenic diet may B. Levetiracetam
become seizure-free C. Primidone
C. A recent retrospective, multicenter study D. Ethosuximide
assessing the ketogenic diet for various E. Zonisamide
264 Multiple Choice Questions for Part IV
91. In addition to the traditional ketogenic diet, does not typically have spontaneous sei-
alternative dietary therapies have been zures. The audiogenic seizure model is pre-
developed for epilepsy treatment. Which of dictive of efficacy against generalized tonic–
the following is an alternative dietary ther- clonic seizures. Levetiracetam efficacy was
apy for epilepsy treatment? missed by the maximal electroshock model
(MES) and PTZ models. The 6 Hz model is
A. The low glycemic index treatment a model of pharmacoresistant epilepsy.
B. The Atkins diet 4. (B). In the first VA cooperate, all four
C. The Paleo diet antiepileptic drugs compared had equal effi-
D. The short-chain triglyceride diet cacy, but phenobarbital and primidone were
less well tolerated.
92. The blood levels of which antiepileptic will 5. (D). In contrast to the listed benzodi-
not be altered after the addition of azepines, Clobazam is a 1,5 benzodiazepine;
phenytoin? the brand name “On-fi” is derived from
“One-five.”
A. Felbamate 6. (D). Diazepam and clobazam have important
B. Topiramate active metabolites (desmethyldiazepam and
C. Zonisamide desmethylclobazam). Clonazepam is con-
D. Tiagabine verted to an inactive metabolite.
E. Pregabalin 7. (B). Connective tissue diseases in particular
Dupuytren’s contractures and plantar fibro-
matosis may be seen with long-term phe-
Answers nobarbital use.
8. (B). Enzyme-inducing antiepileptic medica-
1. (E). In this case, levetiracetam is the most tions can induce the metabolism of other
appropriate choice for treating juvenile concomitant medications such as carba-
myoclonic epilepsy in a woman of child- mazepine and phenytoin and can lower the
bearing age. Pregabalin and carbamazepine serum concentration of simvastatin by about
are typically efficacious against partial–onset 50%.
seizures and may potentially exacerbate 9. (C). Both zonisamide and topiramate are
generalized seizures, notably absence and mild carbonic anhydrase inhibitors. The
myoclonic seizures. Lacosamide lacks evi- resulting decreased sweat may lead to over-
dence to support its use in this condition, heating and possible heatstrokes especially
while valproate should be avoided in women in children.
of childbearing age due to significant risk of 10. (A). While some of the other antiepileptic
teratogenicity. drugs have a long half-life (such as zon-
2. (A). The clinical scenario describes partial isamide, clobazam, and ethosuximide), they
seizures with secondarily generalization. do not have FDA approval for once-daily
Oxcarbazepine is an appropriate drug for dosing. The main reasons are the potential
treating this condition. Lamotrigine and sedation and cognitive or gastrointestinal
topiramate would not be a good choice due side effects that may incur from high doses
to the slow titration schedule. Phenobarbital given once daily.
and valproate must be avoided in women of 11. (C). Ezogabine may be associated with uri-
childbearing age. nary retention in about 2% of patients. It is
3. (A). The pentylenetetrazole (PTZ) model is generally reported within the first six months
predictive of efficacy against absence sei- of treatment, but can also be observed later.
zures, although some effective drugs may be 12. (C). Felbamate reduces carbamazepine level
missed by this model. The kindling model through induction of CYP3A4, but may
Multiple Choice Questions for Part IV 265
mechanism in addition to its SV2A binding. tricarboxylic acid cycle, to be used for
In general, it exhibits a similar profile to energy production or ketone body produc-
levetiracetam general but with a higher tion. A shift to use of fats as the primary
potency (about 10 times higher). energy source in disorders of fat metabolism
31. (B). Valproate can produce tremors and would precipitate deterioration. Lack of
Parkinsonism that is usually dose-dependent carbohydrates would exacerbate acute inter-
and more common in the elderly. It is usu- mittent porphyria.
ally reversible with reduction or elimination 39. (D). Incidence of epilepsy increases with the
of the drug. severity of mental retardation (MR), but it
32. (A). Pentylenetetrazole (PTZ) is an animal varies depending on epidemiological
model of absence seizures, while the other methodologies as well. In population-based
listed animal models are specific for partial studies, 21% of those with mild MR had
seizures. epilepsy. Another study reported epilepsy in
33. (C). Vigabatrin visual toxicity is a slowly 11% of subjects with mild MR and in 23%
progressive, usually irreversible retinopathy in those with severe MR. On the other hand,
that is related to dose and duration of in institution-based studies including
treatment. patients with severe MR, the prevalence of
34. (D). Tiagabine has a short half-life; it is a epilepsy varies from 32 to 34%.
narrow-spectrum agent for partial (focal) 40. (B). Vigabatrin use is associated with visual
seizures, approved only as adjunctive ther- field constriction in about one-third of
apy; it increases GABA levels by inhibiting patients. Periodic visual field monitoring is
its reuptake in the synapse. required for the prescription of vigabatrin.
35. (A). Aplastic anemia and hepatic failure are 41. (E). Premanufactured ketogenic formulas are
unlikely within one month of initiating fel- available, ensuring more accuracy of mea-
bamate therapy. Known risk factors include surements and minimizing barriers such as
prior cytopenia, allergy to or significant food refusal or aversions.
toxicity with other antiepileptic drugs, and 42. (A). Prolonged encephalopathy or noncon-
underlying autoimmune disease. vulsive status epilepticus may be seen as a
36. (C). Valproate is a wide-spectrum dose-related adverse effect of tiagabine.
antiepileptic drug effective against absence 43. (B). This should adequately screen for dis-
seizures; the other listed AEDs may exac- orders of fatty acid metabolism including
erbate absence seizures. carnitine deficiency, CPT I or II deficiency,
37. (D). Unlike other listed antiepileptic drugs, carnitine translocase deficiency, and the
pregabalin does not interact with GABA beta-oxidation defects. The other choices are
receptors. also reasonable considerations for preinitia-
38. (C). In pyruvate dehydrogenase deficiency, tion screening, but for other conditions.
pyruvate cannot be metabolized into 44. (A). Valproate inhibits uridine glucosyl
acetyl-coA. The ketogenic diet bypasses this transferase, the enzyme that metabolizes
step and provides ketones as an alternative lamotrigine. Initiation of valproate therapy
fuel for the brain. All of the other choices are will result in lamotrigine toxicity. This
contraindications to the ketogenic diet. addition usually requires immediate reduc-
Long-chain fatty acids are transported across tion of the dose of lamotrigine by about
the mitochondrial membrane by carnitine 50%.
(helped by CPT I and II and carnitine 45. (D). This would be a longer-term compli-
translocase); once in the mitochondrion, cation. Osteoporosis in the ketogenic diet is
fatty acids are beta-oxidized to 2 carbon contributed to by calcium/vitamin D defi-
units of acetyl-CoA that can then enter the ciency as well as acidosis.
Multiple Choice Questions for Part IV 267
46. (B). Most common known etiology for epi- of choice for pure generalized absence
lepsy is cerebrovascular disease at 11%, seizures.
followed by neurologic deficits from birth, 51. (B). Although the benefit on seizure control
mental retardation, or cerebral palsy at 8%. can be seen within 2 weeks after initiation
47. (D). Long-term complications in children on (in 75% of children in one study), it is rec-
the ketogenic diet for >2 years have not ommended that the ketogenic diet be con-
been systematically reviewed. There may be tinued for 3 months before deciding to
increased fractures and kidney stones. continue or discontinue. Gradual weaning
Symptoms, however, would be expected in rather than abrupt discontinuation is pre-
the setting of pancreatitis, renal calculi, or ferred and may assist with determining
severe constipation. Specific guidelines for whether there has been benefit of the keto-
monitoring of bone health, however, still genic diet on seizure control. The recom-
need to be delineated. mendation is to discontinue after 2 years of
48. (C). In GLUT1 deficiency syndrome, glu- seizure freedom, similar to the time period
cose transport across the blood-brain barrier used for anticonvulsant medications.
is impaired. Since the ketogenic diet pro- 52. (C). The likely diagnosis of this patient is
vides ketones that bypass the metabolic neurocysticercosis. Epilepsy is the most
defect, serving as an alternative fuel to the common presentation (70% of patients) fol-
brain, the ketogenic diet is the treatment of lowed by headache, stroke, and psychiatric
choice for this syndrome. Such epilepsy manifestations. It is more common in
treatment is not necessary for benign myo- Southern America due to ingestion of
clonus of infancy. Although the ketogenic uncooked egg-infected pork meat. Brain
diet may be particularly helpful for gener- imaging often reveals several ring-enhancing
alized epilepsies, there has not been data lesions. Treatment consists of anthelminthic
supporting its use in JME as of yet. The medication (such as albendazole) and ster-
ketogenic diet is contraindicated for pyru- oids (e.g., dexamethasone) to suppress the
vate carboxylase deficiency, which would inflammatory response induced by destruc-
impair tricarboxylic acid cycle function and tion of live cysticerci.
energy production in the ketogenic diet. 53. (D). Valproate exposure during pregnancy is
49. (B). Isoniazid is an antibiotic commonly associated with decreased verbal IQ and
used in treating tuberculosis. It may trigger autism in offsprings. The teratogenic effect is
de novo seizures by competing with the dose-dependent and is irrespective of
mechanism of pyridoxine and its metabo- monotherapy or polytherapy use. Supple-
lites. Pyridoxine is an essential cofactor for mentation with folic acid is not sufficient to
many enzymatic reactions, including GABA reverse the teratogenic effect.
an inhibitory neurotransmitter. 54. (C). This patient is suffering from partial–
50. (B). A large, multicenter, double-blind, ran- onset seizures and postherpetic neuralgia for
domized, controlled trial to compare the which pregabalin is FDA indicated for.
efficacy, tolerability, and neuropsychological Topiramate, lamotrigine, and levetiracetam
effects of ethosuximide, valproic acid, and have not yet been proven effective in this
lamotrigine favored ethosuximide. After setting. Carbamazepine could be helpful but
16 weeks of therapy, the has the risk of drug interactions at the level
freedom-from-failure rates for ethosuximide of hepatic metabolism.
and valproic acid were similar and higher 55. (C). Both valproate and phenytoin are highly
than the rate for lamotrigine. However, protein-bound antiepileptic drugs. When
attentional dysfunction was more common used together or added to a highly
with valproic acid than with ethosuximide. protein-bound medication (such as war-
As a result, ethosuximide became the drug farin), they can compete on protein binding,
268 Multiple Choice Questions for Part IV
increasing the free fraction of either drugs. abnormal initial routine EEG. An additional
Valproate free fraction decreases at higher 35% were identified to have abnormalities
concentrations due to protein saturation. on the second sleep-deprived EEG. An
56. (E). The use of the KD is being investigated in abnormal EEG predicts a higher recurrence
several neurologic conditions beyond epi- rate, and a normal EEG predicts a lower
lepsy and in traumatic brain injury, Alzhei- recurrence rate but does not rule out
mer’s disease, amyotrophic lateral sclerosis, epilepsy.
autism, glial tumors, diabetic nephropathy, 64. (E). Maternal use of valproate during preg-
and Parkinson’s disease. In addition, in nancy was associated with a significantly
development is 2-deoxy-(D)-glucose (2-DG), increased risk of autism spectrum disorder
an agent which is a nonmetabolizable glucose and childhood autism in the offspring.
analog that inhibits glycolysis. 65. (B). Lorazepam and clonazepam do not have
57. (B). Lamotrigine does not affect bone health active metabolites; lorazepam has the short-
while phenytoin, phenobarbital, and topira- est half-life.
mate can, because of their enzyme induction 66. (C). Phenytoin has nonlinear (saturable)
properties. kinetics; its half-life becomes longer after
58. (D). Topiramate should be avoided in elderly the saturation point which is usually within
because it has significant cognitive effects. the recommended therapeutic range.
These can significantly limit or compromise 67. (B). Carbamazepine induces its own meta-
their intellectual reserves. bolism so that its half-life becomes shorter
59. (B). Faciobrachial dystonic seizures are fre- with continued use. The process of autoin-
quent brief dystonic seizures, typically duction is completed over 2–4 weeks.
affecting the ipsilateral arm and face found 68. (C). Recurrence rate after the first seizure
in association with LGI1 antibodies. Facio- averages around 30–40% by two years. The
brachial dystonic seizures often precede risk is higher (approaching 50–60%) when
LGI1-antibody encephalitis. Recognition the EEG or brain MRI is positive.
may lead to early diagnosis and early insti- 69. (A). Primidone is converted into phenobar-
tution of immunotherapy, with improved bital and phenylethylmalonamide (PEMA),
outcome. which is also an active metabolite. Primi-
60. (A). Excluding carbamazepine, all the other done has acute toxic reactions that are dif-
four medications (amiodarone, cimetidine, ferent from phenobarbital. It can produce
fluconazole, and felbamate) may inhibit transient drowsiness, dizziness, ataxia, nau-
phenytoin metabolism and may cause sea, and vomiting that can be debilitating.
phenytoin accumulation. These reactions are present even before
61. (B). Excluding ceftriaxone, all the other phenobarbital has appeared as a metabolite.
three medications (erythromycin, fluoxetine, 70. (D). Among the listed antiepileptic drugs,
and propoxyphene) and grapefruit juice may gabapentin is mostly excreted in urine;
inhibit carbamazepine metabolism and may hence, its dose should be reduced according
cause carbamazepine accumulation. to the renal function.
62. (D). Estrogen may be proconvulsant as it 71. (D). Overall, risk factors that carry higher
may reduce inhibition at the GABAA seizure recurrence rate include focal-onset
receptor and also inhibits the synthesis of seizures, status epilepticus or cluster seizures
GABA. On the other hand, progesterone at first seizure, abnormal EEG with epilep-
may be anticonvulsant as it enhances inhi- tiform activity (sharp waves or spikes), and
bition at the GABAA receptor and increases abnormal brain MRI or neurological
GABA synthesis. examination.
63. (B). In one pooled analysis of 1766 subjects, 72. (E). Levetiracetam does not interact with
51% of patients (pooled analysis) had an oral contraceptive medications, while all the
Multiple Choice Questions for Part IV 269
other choices can lower oral contraceptive tend to offer pharmacological advantages in
medication levels. regard to lower protein binding, minimal
73. (B). Antiepileptic drugs with potential oph- drug–drug interaction, and absence or mini-
thalmological adverse effects include topi- mal liver inhibition/induction. They are,
ramate (acute open-angle glaucoma), however, more expensive than older AEDs.
vigabatrin (peripheral visual constriction), 81. (A). Ethosuximide and valproate are both
and ezogabine (photoreceptor damage). known to block T-type calcium channels,
74. (C). Zonisamide and topiramate are mild which conveys efficacy against absence
carbonic anhydrase inhibitors and are known seizures.
to decrease sweat production (causing 82. (C). HLA allele B*1502 is a marker for
anhydrosis or hypohydrosis). This may par- carbamazepine-induced Stevens–Johnson
ticularly be dangerous in patients with high syndrome and toxic epidermal necrolysis,
physical activity, without compensatory particularly in Han Chinese. The FDA rec-
hydration. ommends genotyping all Asians for the
75. (B). Risk of seizure recurrence after first allele before treatment initiation.
symptomatic seizure is about 33% in an 83. (E). St John’s wort (or Hypericum perfora-
acute (less than 7 days) stroke setting and tum) is a popular medicinal herb used for the
70% in a remote setting (more than 7 days). treatment of depression. It is known to
Risk of seizure recurrence after first symp- induce cytochrome P450 affecting the phar-
tomatic seizure is about 13% in the setting of macokinetics of several AEDs such as phe-
acute TBI and 45% in the setting of remote nobarbital, carbamazepine, and phenytoin,
TBI. resulting in adverse events.
76. (B). Vigabatrin’s visual field defect may 84. (A). Overall, 11–41% of patients will relapse
affect about one-third of patients with vari- after antiepileptic drug discontinuation. The
able severity. It primarily consists of relapse rate tends to be lower in children
peripheral visual field constriction. Risk (*20%) and higher in adults (*40).
factors include longer duration of treatment 85. (D). This is the consensus statement made
and higher dosage. by the International Ketogenic Diet Study
77. (C). All of the listed antiepileptic drugs are Group.
considered to be broad-spectrum except 86. (E). Brivaracetam binds to the synaptic
pregabalin which targets focal-onset seizures vesicle protein as well as blocks sodium
and may alternatively worsen generalized channels.
seizures especially myoclonic seizures. 87. (A). Compared to the Cochrane Review
78. (C). Several clinical trials were concluded or performed in 2003, in which no RCTs were
underway for (up to the current writing) the available, the Cochrane Review in 2012
treatment of acute repetitive seizures. This reviewed 4 RCTs, which showed that at least
includes intranasal diazepam and midazo- 38% of patients had a 50% decrease in sei-
lam, intramuscular diazepam (by autoinjec- zures at 3 months, with this positive
tor), and buccal midazolam. response maintained for a year. Hender-
79. (B). Zonisamide does not have an extended son CB et al’s meta-analysis in 2006 showed
release preparation, but has a prolonged 1/3 of patients having a >90% decrease in
half-life, obviating the need for such a seizures. Caraballo R et al’s multicenter
preparation. retrospective study in 2011 showed 22%
80. (C). Overall, when compared to older AEDs, with seizure freedom and 56% with greater
newer AEDs tend to have better safety profile than 75% decrease.
and tolerability (except for felbamate, viga- 88. (B). Zonisamide’s chemical structure
batrin) but comparable efficacy. They also includes a sulfa moiety and thus should be
270 Multiple Choice Questions for Part IV
avoided in patients with known history of exclusively against absence seizures. Car-
sulfa allergy. bamazepine and primidone are liver enzyme
89. (C). Traditionally, the patient would fast for inducers that can decrease warfarin efficacy
24–48 h. Once urine ketones appeared, the and worsen osteoporosis. Zonisamide can
ketogenic diet would then be initiated grad- precipitate kidney stones.
ually. Data support the fact that ketosis 91. (A). The Modified Atkins diet, the medium-
occurs without this initial fasting period and chain triglyceride diet, and the low glycemic
that tolerance of the diet may be higher index treatment are alternative dietary ther-
without this fasting period. apies developed for epilepsy treatment.
90. (B). In this case, levetiracetam is the most 92. (E). Pregabalin is not metabolized and is not
appropriate choice for treating partial sei- affected by phenytoin and other enzyme
zures. Ethosuximide is efficacious inducers.