A 25-year-old man presents with a generalized tonic-clonic
(GTC) seizure that occurred after a late night out. This was his
second GTC seizure; the first occurred about 1 year ago. He
also describes episodes of brief staring since he was 15 years
old and occasional jerking (usually in the morning) for the
past 12 years. He does not report any cognitive issues. Which
electroencephalographic (EEG) pattern is most commonly
associated with this patient’s presentation?
a. Generalized 3-Hz spike-and-wave discharges
b. Generalized slow spike-and-wave discharges (<2.5 Hz)
c. Generalized 4- to 6-Hz polyspike-and-wave discharges
d. Focal right centrotemporal spikes
e. Right frontal sharp waves
Answer c.
The scenario is typical for juvenile myoclonic epilepsy
(JME). Patients with JME often present as developmentally
normal teenagers. Patients have a combination of myo-
clonic, GTC, and absence seizures. Myoclonic seizures often
occur shortly after waking. GTC seizures occur in more than
90% of patients with JME. Conversely, absence seizures are
present in only about one-third of JME patients. The EEG
typically shows generalized 4- to 6-Hz polyspike-and-wave
discharges interictally. Generalized discharges and myo-
clonic seizures are photosensitive in about 30% of patients.
A 1-year-old girl was brought to the clinic after her mother
noticed sudden abnormal movements, including forward
bending of the head and torso. This has been occurring for
the past 2 months in clusters of 10 to 20, several times daily.
The child has not reached several developmental milestones.
Electroencephalography (EEG) showed high-amplitude, asyn-
chronous slow waves with multifocal spikes and sharp waves.
What is the most likely diagnosis?
a. West syndrome
b. Early infantile epileptic encephalopathy (Ohtahara syndrome)
c. Severe myoclonic epilepsy in infancy (Dravet syndrome)
d. Myoclonic-atonic epilepsy (Doose syndrome)
e. Panayiotopoulos syndrome
Answer a.
West syndrome is clinically characterized by infantile
spasms and developmental arrest or regression. Infantile
spasms are a type of epileptic spasms that occur during
infancy and often occur in clusters. In patients with West
syndrome, the interictal EEG shows hypsarrhythmia, which
consists of high-amplitude, asynchronous slow waves with
multifocal spikes and sharp waves (Figure 81.6).
Electrodecrement is typically seen on EEG during spasms.
A 13-year-old boy is brought to the clinic by his parents, who
noticed that he had restless movements during sleep with an
inability to respond, but he had preserved awareness. A previ-
ous routine electroencephalogram (EEG) during wakefulness
was normal. After he was admitted to the epilepsy monitoring
unit, spells recorded during sleep included lower limb bend-
ing, jerking, and rocking movements. The corresponding EEG
showed frontal lobe–onset seizures. Several of his family
members have seizures that occur during sleep. A mutation of
which gene is associated with this disease?
a. LGI
b. CHRNA2
c. SCN1A
d. STXBP1
e. KCNQ2
Answer b.
In patients with autosomal dominant nocturnal frontal lobe
epilepsy, seizures begin in late childhood or adolescence and
persist into adulthood. This syndrome is associated with an
autosomal dominant mutation in the genes coding for neuro-
nal nicotinic acetylcholine receptor subunits, including
CHRNA2, CHRNA4, and CHRNB2. Seizures are usually focal
aware with hyperkinetic or tonic movements and occur dur-
ing non–rapid eye movement sleep. Patients frequently have
sensory or psychic auras. The interictal EEG is usually nor-
mal; the ictal EEG may show frontal discharges (or they may
be obscured by artifact), but it can also be normal.
Which of the following seizures would be considered unpro-
voked?
a. A seizure in a patient who has severe hyponatremia
b. A seizure in a patient who slept only 4 hours the night before
c. A seizure in a patient who abruptly missed several doses of
scheduled clonazepam
d. A seizure within 48 hours after a patient had an acute stroke
e. A seizure in a patient undergoing alcohol withdrawal
Limited sleep deprivation can lower the seizure thresh-
old, but it would not be expected to provoke a seizure in
a person who did not have a tendency toward seizures.
All the other choices would be expected to cause acute
symptomatic seizures.
VIII.5. Which patient would have the highest risk for new unpro-
voked seizures?
a. A 68-year-old man with a history of a large middle cerebral
artery stroke 2 years earlier
b. A 22-year-old woman with a history of 2 concussions
c. An 8-year-old girl with type 1 diabetes mellitus who recently
began insulin therapy
d. A 9-month-old boy with a febrile illness
e. A 56-year-old with a family history of epilepsy
Children and older persons are the most likely to receive
a new diagnosis of epilepsy. The stroke in the 68-year-old
man increased his risk. Concussion is not a risk factor for
seizures. Hypoglycemia or febrile illnesses can cause sei-
zures that are provoked rather than unprovoked. A family
history of epilepsy is a risk factor for epilepsy, but the risk
is not as high as the risk with stroke.
VIII.6. Which of the following characteristics would be most consis-
tent with autoimmune epilepsy?
a. Generalized epilepsy with onset in adolescence
b. A high seizure burden of 2 types of focal seizures and a per-
sonal history of rheumatoid arthritis
c. Drug-resistant epilepsy with infrequent seizures
d. Multifocal epilepsy with diffuse polymicrogyria
e. An elderly patient with long-standing, well-controlled epi-
lepsy and gradual cognitive decline
Drug-resistant epilepsy, high seizure burden, multifocal or
evolving seizures, and a history of autoimmune disease are
clinical factors associated with autoimmune epilepsy. The
origins of generalized epilepsies are idiopathic and genetic.
Patients with infrequent seizures are less likely to have auto-
immune epilepsy. Multifocal epilepsy may occur with auto-
immune epilepsy, but a preexisting congenital brain anomaly
(polymicrogyria) would be a more likely cause. Patients with
autoimmune epilepsy may have cognitive impairment, but
an elderly patient with long-standing, well-controlled epi-
lepsy would be more likely to have dementia.
VIII.7. Which type of neurologic spell is sometimes mistaken for sei-
zure and is much more common in the general population?
a. Transient ischemic attack
b. Psychogenic nonepileptic seizure
c. Transient global amnesia
d. Paroxysmal kinesiogenic dyskinesia
e. Syncope
Answer e.
Syncope is sometimes mistaken for seizure and is 60 times
more common. Clinical features more suggestive of syn-
cope include history of cardiac disease or arrhythmia, pal-
pitations, short duration (typically seconds rather than
minutes), and associated autonomic symptoms (see Box
83.4). Convulsive syncope is a particularly perplexing con-
founder when the associated brief myoclonic motor activ-
ity is confused with a seizure. Vasovagal syncope may have
a typical provoking factor, such as pain or needles.
VIII.8. Which of the following is more commonly associated with
psychogenic nonepileptic seizures?
a. Incontinence
b. Lack of stereotypy
c. Bicycling motion of the legs
d. Loud grunt at onset
e. Forced gaze
Psychogenic nonepileptic seizures can resemble epileptic
seizures, but Box 83.7 lists some features more common in
nonepileptic seizures. Lack of stereotypy would be atypi-
cal for epileptic seizures. Incontinence favors a diagnosis
of seizure, although it can occur with nonepileptic sei-
zures. Bicycling motion of the legs can be seen if patients
have a frontal seizure focus, whereas pelvic thrusting
would be unusual. Grunting, screaming, or moaning occurs
more commonly with frontal rather than temporal localiza-
tion, and laughter is sometimes a feature when patients
have hypothalamic lesions (eg, gelastic seizure). Head
turning and gaze deviation are also common with seizures.
VIII.9. A 17-year-old patient had an episode of sudden fright and
then tears while at a sleepover party. After that episode, the
patient was unresponsive and tired. Where is the likely local-
ization of the seizure focus?
a. Supplementary motor area
b. Temporal lobe
c. Parietal lobe
d. Occipital lobe
e. Angular gyrus
VIII.9. Answer b.
Psychic symptoms such as déjà vu, a rising epigastric sen-
sation, powerful emotions (fear or fright), or a noxious
smell can indicate an aura emanating from the temporal
lobe. See Table 83.1.
VIII.10. Which of the following statements is true about women of
childbearing age and antiseizure medication?
a. Lamotrigine carries the highest risk of teratogenicity
b. Folic acid, 0.1 mg daily, is recommended to reduce the risk of teratogenicity in patients who need antiseizure medication
during pregnancy
c. Contraception should be discussed with women of childbear-
ing age when antiseizure medication is prescribed
d. Women with a seizure disorder should stop all antiseizure
medications as soon as they know they are pregnant
e. Dose adjustments of antiseizure medications are not neces-
sary during pregnancy
VIII.10. Answer c.
Contraception should be discussed with all women of
childbearing age when antiseizure medication is pre-
scribed. Oral contraceptives are hepatically metabolized
by the same enzymes as many of the enzyme-inducing
antiseizure medications, so those medications can reduce
the effectiveness of contraception. Folic acid is recom-
mended as a supplement for women with childbearing
potential because supplementation can decrease the rate
of neural tube defects in the growing fetus. A common
dose is 0.4 to 1 mg daily. Although nearly all antiseizure
medications present some risk of teratogenic adverse
effects, valproic acid carries the highest potential harm.
During pregnancy, the dosing of medications may need to
be modified as the patient’s metabolism and volume of
distribution of the medication change. Monitoring drug
levels may be helpful. After delivery, the medications
should be adjusted again to avoid potential adverse effects.
VIII.11. Which of the following patients would be the best candidate
for surgical consideration to prevent seizure?
a. A 16-year-old patient with juvenile myoclonic epilepsy
b. A 75-year-old man with complex partial seizures related to
sulcal subarachnoid blood
c. A 35-year-old woman with left mesial temporal cavernous
malformation and a single seizure without recurrence while
taking a single medication
d. A 25-year-old man with left mesial temporal sclerosis and com-
plex partial seizures that were refractory to 2 medication trials
e. A 35-year-old man with complex partial seizures and no
abnormal findings on magnetic resonance imaging
VIII.11. Answer d.
Surgery is generally reserved for patients with refractory
seizures who have a focal lesion. Up to 70% of patients
with mesial temporal sclerosis can achieve seizure freedom
up to 2 years after surgical resection. Patients with cavern-
ous malformation may undergo surgery if the goal is to
reduce the rate of future hemorrhage. However, if the lesion
has not recently hemorrhaged and the patient responds to a
single medication, resection is not always pursued. Juvenile
myoclonic epilepsy is a generalized seizure disorder. Sulcal
subarachnoid blood is often due to underlying amyloid or
trauma (or both) and is not typically resected.
VIII.12. Which of the following medications may be favored for a
patient with liver disease and complex partial seizures?
a. Cannabidiol
b. Carbamazepine
c. Phenytoin
d. Topiramate
e. Lacosamide
VIII.12. Answer e.
Box 84.1 reviews antiseizure medications and the route of
metabolism. Cannabidiol, carbamazepine, phenytoin, and
topiramate are predominantly metabolized through the
liver. Lacosamide is the only medication listed that is pre-
dominantly renally excreted.
VIII.13. Which of the following medications could be considered for
an elderly patient who receives warfarin and who has com-
plex partial seizures?
a. Phenytoin
b. Levetiracetam
c. Felbamate
d. Divalproex
e. Ethosuximide
VIII.13. Answer b.
Levetiracetam is indicated for complex partial seizures or
generalized seizures. It has minimal drug-drug interac-
tions. Felbamate is not commonly used because of its
potential for severe adverse effects. Divalproex and phe-
nytoin interact with warfarin. Ethosuximide is usually
used for generalized seizures such as absence seizures.
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