Seizures Flashcards
Approximately what proportion of patients with epilepsy have medically refractory seizures?
a. 0-20%
b. 20-40%
c. 40-60%
d. 60-80%
e. 80-100%
b. 20-40%
Which one of the following is a predictor of spontaneous epilepsy remission?
a. Abnormalities on neurological examination or developmental delay
b. Identification of epileptogenic substrate
c. Inadequate seizure control for greater than 4 years
d. Persistent epileptiform abnormalities
on EEG
e. Younger age at onset
e. Younger age at onset
In general, predictors for low probability of
epilepsy remission are (i) symptomatic localized
epilepsy secondary to remote CNS injury,
(ii) abnormalities on neurological examination
or developmental delay, (iii) persistent epileptiform abnormalities on EEG, (iv) older age at onset, (v) inadequate control of seizures for longer than 4 years, (vi) presence of multiple seizure types and frequent generalized tonic-clonic seizures. Seizure duration of over 10 years also decreases likelihood of achieving seizure control in those who undergo epilepsy surgery.
The approximate rate of sudden unexpected death in epilepsy (SUDEP) in patients with medically intractable seizures is which one of the following?
a. 1 in 200 per year
b. 1 in 400 per year
c. 1 in 600 per year
d. 1 in 800 per year
e. 1 in 1000 per year
a. 1 in 200 per year
Overall rates of SUDEP in adults is 1 in 1000 and
lower in children at 0.2-0.4 in 1000 per year.
However, in adults and children with medically
intractable epilepsy rates are higher at 1 in 100-
200 per year. Cause of death in SUDEP is unclear but thought to be due to seizure related cardiac arrhythmias and/or respiratory compromise.
The most reliable risk factor is severity and frequency of seizures (particularly generalized
tonic-clonic seizures). Other risk factors include
nocturnal seizures, young adult, poor adherence
to treatment, earlier age of seizure onset, longer
duration of epilepsy, symptomatic epilepsy and
male gender.
A 7-year-old right-handed girl presented
with onset of seizures at 3-years old. Typically, her eyes rolled up and she often had
either a left or right body twitch. These
events lasted 10-12 s and occurred often in
activities such as eating or talking. Immediately after these seizures, she spontaneously
returned to her baseline. They typically
occurred 6-10 times a day. She had been on
numerous medications in the past but
continues to have frequent breakthrough
seizures on a daily basis. EEG shows a 3Hx spike and wave pattern. Which one of the following is most likely?
a. Breath holding attack
b. Cardiac syncope
c. Childhood absence epilepsy
d. Partial complex seizures
e. Rolandic epilepsy
c—Childhood absence epilepsy
A 12-year-old boy with diagnoses of attention-deficit disorder and hyperactivity was noted to have “staring episodes” beginning over 1 year before this evaluation. About 6 months earlier, the staring episodes became accompanied by some eye blinking and mouth twitching. He has had one generalized tonic-clonic seizure in the last year. Which one of the following is most likely diagnosis?
a. Childhood absence epilepsy
b. Day dreaming
c. Juvenile absence epilepsy
d. Neurocardiogenic syncope
e. Reflex anoxic seizure
c. Juvenile absence epilepsy
Juvenile absence epilepsy is a type of idiopathic
generalized epilepsy. Onset is from age 8 to
age 16 years, with a peak occurrence at 10-12
years of age. The frequency of absence seizures
in juvenile absence epilepsy is lower than that
in childhood absence epilepsy. A higher frequency of generalized tonic-clonic seizures is
seen compared with childhood absence epilepsy,
and there is an increased probability of epilepsy
continuing into the adult years. They also noted
that 11% of patients with the disorder report a
family history of epilepsy. Absence seizures are
predominant. The impairment of consciousness
in juvenile absence epilepsy is moderate and
not generally as severe as in childhood absence
epilepsy. The level of retained consciousness
may vary significantly from seizure to seizure
in the same patient. Unlike childhood absence
epilepsy they may occur once a day or in a cluster in the hour after awakening. The classic clinical feature is “simple absence” with staring and altered alertness (sometimes “complex absence” with blinking or head nodding). Seizures are typically triggered by hyperventilation or sleep deprivation. EEG: Interictal background activity is usual normal, spike and wave slightly faster (3.5-4 Hz).
A 19-year-old man presents with a history of seizures. The first was at age 12 when he got up very early in the morning to play computergames and was found by his mother a few hours later sitting at the computer blinking and unresponsive. Two years later he experiences a generalized tonic-clonic seizure. Currently he has a seizure every 1-3 weeks and can involve a minor jerk of the arms, staring with blinking, or generalized tonic-clonic seizures. He believed that sleep deprivation often precipitated seizures.Which one of the following is most likely diagnosis?
a. Benign familial convulsions
b. Benign myoclonic epilepsy in infancy
c. Epilepsy with myoclonic absences
d. Juvenile absence epilepsy
e. Juvenile myoclonic epilepsy
e. Juvenile myoclonic epilepsy
Juvenile myoclonic epilepsy comprises 5-10%. It is
an idiopathic generalized epilepsy with myoclonic
jerks; may also have typical absence seizures, generalized tonic-clonic seizures, or all three seizure
types. The majority have myoclonic and generalized tonic-clonic seizures shortly after awakening
from sleep. Seizure types have age-specific onset:
absence (5-14 years), myoclonic (9-18 years), and
generalized tonic-clonic seizures (9-26 years).
Sleep deprivation is identified as a precipitating
factor in the large majority (>90%) of individuals.
The EEG showing paroxysmal spike, polyspike,
and wave complexes that may be regular at 3-
5 Hz but often are irregular with 2- to 10-Hz components. A photoconvulsive effect on the EEG has
been described in at least one third of cases.
Hyperventilation less reliably than in childhood
absence epilepsy or juvenile absence epilepsy.
Which one of the following abnormalities is demonstrated in the ictal EEG below?
a. 3-Hz spike and wave discharge
b. Hypsarrhythmia
c. Lambda wave
d. Rhythmic temporal theta burst of
bdrowsiness
e. Sleep spindles
a. 3-Hz spike and wave discharge
Which one of the following seizure types is classically associated with the pathology shown below?
a. Absence seizure
b. Complex partial seizure
c. Focal motor seizure
d. Gelastic seizure
e. Rolandic seizure
d. Gelastic seizure
Hypothalamic (or tuber cinereum) hamartomas
are associated with gelastic seizures, visual problems, central precocious puberty (increased
GnRH) and behavioral change. Gelastic seizures
manifest as typically short (<30 s) bursts of uncontrollable laughter with preservation of
consciousness.
Background interictal awake EEG in a 10-month-old female infant presenting with clusters of arm abduction and head drop. Which one of the following is most likely?
a. Benign Rolandic epilepsy
b. Gelastic epilepsy
c. Landau-Kleffner syndrome
d. Lennox-Gastaut syndrome
e. West syndrome
e. West syndrome
West syndromeis the triad of infantile spasms, hypsarrhythmia on EEG and developmental delay or
regression. Onset is typically around 6 months of
age (almost all begin within the first year of life);
incidence is 1 in 3225 live births. Spasms may
involve brief contractions of predominantly flexor
or extensor muscle groups ranging from large
“jack-knife” type motions to subtle head bobbing.
These movements are at times difficult to differentiate from less serious non-epileptic events
such as gastroesophageal reflux, colic and benign myoclonus of infancy. Precipitating factors mostly
include when falling asleep/waking up, being handled, loud noise, feeding, infection, fever, excitement, hunger and excessive environmental
temperatures. Cognitive disorders may include
mental retardation, speech delay, autistic features
and visuomotor dyspraxia. EEG of hypsarrhythmia
consists typically of a diffuse, very high voltage, disorganized, chaotic and asynchronous pattern of
multifocal spike and wave discharges. This pattern
is seen while the child is both awake and in nonrapid-eye-movement (NREM) sleep. In REM
sleep, there is marked reduction or even disappearance of the HA pattern. EEG with simultaneous
pyridoxine injection is often performed to rule
out pyridoxine-dependent seizures (very rare, <1
in 100,000; treatment is with high doses of vitamin
B6). Video-EEG analysis is considered the gold
standard to diagnose the spasms, and to assess for
focal features of the spasm semiology or EEG tracings. Metabolic screens and LP may help identify
cause. MRI should be done to look for surgical
lesions. Adrenocorticotropic hormone (ACTH) is
effective in the short-term treatment of infantile
spasms and in the resolution of HA (takes about
2 weeks with an “all or nothing” response). Vigabatrin indicated for pediatric patients aged from
1 month to 2 years with infantile spasms (but risk
of irreversible vision loss; need ophthalmology
assessment). If EEG and MRI demonstrate a focal
causative lesion surgical resection can be performed, while functional hemispherectomies may
be used in more diffuse abnormalities (e.g.
Sturge-Weber).
A 12-year-old, right-handed, developmentally delayed boy presented with three seizure types: generalized tonic-clonic, tonic, and atonic. The patient generally had at LEAST three brief seizures (1-
10 s) per day of the tonic and atonic types but rarely had generalized seizures. He presented to the
emergency department after one generalized tonic-clonic seizure followed by a series of atonic drop
attacks concurrent with a streptococcal infection. The patient remained lethargic, and status epilepticus was a concern. Which of the following is the most likely diagnosis?
a. Aicardi syndrome
b. Angelman syndrome
c. Lennox-Gastaut syndrome
d. Otahara syndrome
e. West syndrome
c. Lennox-Gastaut syndrome
Lennox-Gastaut syndrome is characterized by
multiple generalized seizure types that are refractory to treatment, cognitive dysfunction, and an
interictal slow spike and wave pattern with a slow
background on EEG. May be idiopathic or symptomatic (e.g. hypoxic-ischemic, traumatic brain
injury). Onset is generally between 2 and 6 years
of age. A wide range of behavioral problems will
develop in about half of patients. Approximately
25% of patients will have a history of infantile
spasms. The most common manifestation is a drop
attack. Drop attacks may range from a simple head
drop to a fall if proximal legs are involved. Because
the drop attack may be due to a tonic, atonic, or
myoclonic seizure, video-EEG monitoring may
be necessary. Tonic seizures may involve the arms,
legs, or whole body. Tonic seizures often occur in
sleep. Atonic seizures appear as loss of postural tone of the head or whole body. Clonic (clusters of
myoclonus) seizures involving the arms, face, or
legs may occur in isolation or in clusters. The
EEG shows a bisynchronous spike and wave or
polyspikes and wave pattern. Sixty to seventy percent of patients exhibit atypical absence seizures.
Hyperventilation does not provoke this seizure
type. At least one episode of status epilepticus
occurs in more than half of patients over their lifetime. Typical presentations include mental status
changes or persistent tonic seizures. Medications
are often chosen for the most debilitating seizure
type. Broad-spectrum anticonvulsants such as valproic acid have been cited as good choices because
of their efficacy in many seizure types. Surgical
interventions including vagal nerve stimulator
placement and corpus callosotomy have been used
in some patients as palliative procedures with varying degrees of success. Lennox-Gestaut is one of
the few epilepsies particullarily responsive to deep
brain stimulation of the centromedian nucleus
(Velasco et al.).
When the patient, a young boy, was about age 6½ his mother first noticed an episode in which he
failed to respond to her on the telephone. Within 3 months, he had become “deaf” with an auditory
aphasia and poor articulation. An EEG during sleep showed continuous epileptiform discharges
from the posterior left temporal region. His language markedly improved with valproic acid and
prednisone. Which of the following is the most likely diagnosis?
a. Absence seizures
b. Continuous spike and wave during slowdwave sleep syndrome (CSWS)
c. Landau-Kleffner syndrome
d. Parietal lobe epilepsy
e. Rassmussen’s syndrome
f. Temporal lobe epilepsy
c. Landau-Kleffner syndrome
Landau-Kleffner syndrome, or acquired epileptic aphasia, is characterized by a progressive verbal agnosia in a child with previous normal
language development, paroxysmal EEG abnormalities, and epileptic seizures. Associated symptoms include psychomotor and behavioral
disturbances consisting of motor hyperactivity,
impulsivity, and aggressive behavior. There is a
slight male predominance, and it typically presents at between 3 and 8 years of age. Onset is
usually acute and presents initially as difficulty
comprehending language, followed by verbal
agnosia. Subsequently, expressive language is
also affected and may lead to mutism. The
EEG background activity during wakefulness is
usually normal; severe and variable abnormalities
during sleep. Neuroimaging without any structural brain lesion is required for diagnosis.
PET scans have demonstrated regions of hypometabolism predominantly in the temporal
lobes. Seizures are usually well controlled with
traditional antiepileptic drugs but the neuropsychological manifestations have a more variable
response. Valproate, clobazam, and ethosuximide, either monotherapy or in polytherapy,
are successful in controlling seizures and are
occasionally successful in reversing language
regression. Carbamazepine, phenobarbital, and
phenytoin can lead to worsening of seizures
and conversion to electrical status epilepticus
during slow sleep because they may increase
cortical synchronization. Corticosteroids can provide dramatic improvement in language, cognition, and behavior in some children. In CSWS, seizures
are the first symptom in 70-80% of children, followed by neuropsychological regression that may
be global or selective regression of cognitive functions, excluding the acquired aphasia characteristic
of Landau-Kleffner syndrome.
Which one of the following is the most common cause of neonatal seizures?
a. Fetal inflammatory response
b. Hypocalcemia
c. Hypoglycemia
d. Hypoxia-ischemia
e. Pyridoxine-dependent seizures
d. Hypoxia-ischemia
Hypoxia-ischemia (i.e. asphyxia) is traditionally
considered the most common cause associated
with neonatal seizures. Intrauterine factors before
labor can result in fetal asphyxia without later
documentation of acidosis at birth. Both antepartum and intrapartum maternal and placental
illnesses associated with thrombophilia, preeclampsia, or specific uteroplacental abnormalities such as abruptio placentae or cord
compression may contribute to fetal asphyxial
stress leading to metabolic acidosis. Antepartum
maternal trauma and chorioamnionitis are additional conditions that also contribute to the intrauterine asphyxia secondary to uteroplacental
insufficiency. Intravascular placental thromboses
and infarction of the placenta or umbilical cord
documented after birth are markers for possible
fetal asphyxia. Meconium passage into the amniotic fluid also promotes an inflammatory
response within the placental membranes, potentially causing vasoconstriction and resultant
asphyxia. Postnatal include persistent pulmonary
hypertension of the newborn, cyanotic congenital
heart disease, sepsis, meningitis, encephalitis, and
primary intracranial hemorrhage are leading
diagnoses. Fetal inflammatory response may
increase in the risk of unexplained early-onset seizures after intrapartum maternal fever. Other
causes include hypoglycemia, hypocalcemia and
pyridoxine-dependent epilepsy (rare).
A 24-year-old male presents with episodes characterized by facial (mouth and tongue) clonic movements (which may be unilateral), laryngeal symptoms, articulation difficulty, swallowing or chewing
movements and hyper-salivation. Sensory (e.g. epigastric) and experiential (e.g. fear) aura and autonomic (urogenital, gastrointestinal, cardiovascular or respiratory) features are common. Gustatory
hallucinations are particularly common. Which one of the following areas may the seizure focus arise?
a. Cingulate gyrus
b. Dorsolateral frontal
c. Fronto-parietal operculum
d. Frontopolar
e. Motor cortex
f. Orbitofrontal
g. Supplementary sensorimotor area
c. Fronto-parietal operculum
Frontal lobe epilepsy is the second most common
localization-related epilepsy, accounting for
20-30% of surgical series. These types of seizures
are usually short, occur more frequently during
sleep, and tend to cluster. Lateralizing signs
include version and unilateral clonic, tonic, or
dystonic activity that correlate with contralateral
onset. Unless there is secondary generalization,
responsiveness persists throughout the seizure.
Complex partial seizures of frontal lobe origin
are characterized by partial or complete loss of
consciousness. Most patients report an initial aura
of vague general body sensation or unspecified
cephalic aura. The patient initially may develop
staring and behavioral arrest. Motor manifestations consist of prominent semi-purposeful automatism. There are frequently bilateral and
involve both legs and arms with features of running, pelvic thrusts, and bizarre behavior. Upper
extremity automatisms tend to be irregular,
involving proximal muscles. Later during the seizures laughing and crying may be observed.
Finally, hypermotor activity characterized by
complex movements of the proximal segments
of limbs and trunks may occur. In contrast, temporal lobe seizures have early and prominent
oroalimentary automatism and repetitive upper
extremity automatism involving mainly the distal
segments. Consciousness is more frequently
affected or lost in temporal lobe epilepsy.
MRI Abnormalities include encephalomalacia,
neoplasm, vascular malformations, cortical
dysplasias, and migrational disorders.
Which one of the following is the likely origin of this secondarily generalized seizure recorded in the EEG below?
a. Frontal poles
b. Mesial temporal lobe
c. Motor cortex
d. Occipital
e. Parietal
a. Frontal poles
This ictal EEG begins with attenuation and then
low-amplitude fast frequencies frontocentrally
(Fp1-F3, Fp1-F7 and Fp2-F4, Fp2-F8) before
evolving into more generalized irregular polyspike and wave
A 10-year-old right-handed boy presented with episodes of numbness around the mouth, drooling,
right-sided facial twitching, and inability to speak on waking up from sleep. There was retained awareness and memory. Episodes were frequently followed by headaches. He experienced several episodes
per week lasting up to 1-2 min. Sleep EEG shown below. Which one of the following is most likely?
a. Benign Rolandic epilepsy
b. Childhood absence epilepsy
c. Juvenile myoclonic epilepsy
d. Pyridoxine-dependent epilepsy
e. West syndrome
a. Benign Rolandic epilepsy
Benign focal epilepsy with centrotemporal spikes
(BECTS; Benign Rolandic epilepsy) is the most
frequent focal epilepsy syndrome in childhood.
Age at onset ranges from 3 to 13 years of age,
and peak incidence is usually around age 8 years.
Seizures present usually at night, often shortly
after falling asleep or before waking up. The present as somatosensory aura with perioral paresthesias, a sensation of choking, and jaw or
tongue stiffness. Hemifacial or hemibody motor
seizures, frequently with unilateral clonic, but
also tonic or tonic-clonic, activity are seen in up
to 34% of patients. Motor features involve the
face, lips, tongue, pharynx, and larynx, and this
may be associated with speech arrest. Generalized
tonic-clonic seizures without focal onset have
been described in 54%. The characteristic interictal EEG finding is a distinct high-amplitude,
diphasic spike with prominent aftergoing slow
wave typically in C3/C4 or T3/T4 electrodes.
Spikes are more frequently observed during
sleep, and sleep activation of spikes is a salient feature of BECTS. The condition remits spontaneously in almost all patients around the age of
16 years hence indications for treatment include seizures during daytime, repeated generalized
tonic-clonic seizures, prolonged seizures, and status epilepticus as well as seizure onset before the
age of 4 years. Carbamazepine may control seizures in up to 65% of patients.
- Frequency 8-13 Hz
EEG terminology:
a. Alpha
b. Beta
c. Delta
d. Fast
e. Gamma
f. Lambda
g. Sharp
h. Slow
i. Spike
j. Theta
a. Alpha
- Frequency under 4 Hz
EEG terminology:
a. Alpha
b. Beta
c. Delta
d. Fast
e. Gamma
f. Lambda
g. Sharp
h. Slow
i. Spike
j. Theta
c. Delta
- Frequency 14-40 Hz
EEG terminology:
a. Alpha
b. Beta
c. Delta
d. Fast
e. Gamma
f. Lambda
g. Sharp
h. Slow
i. Spike
j. Theta
b. Beta
- Anterior temporal lobe
EEG electrode position:
a. A1 and A2
b. C3 and C4
c. F3 and F4
d. F7 and F8
e. Fp1 and Fp2
f. O1 and O2
g. P3 and P4
h. P7 and P8 (or T5 and T6)
i. Sp1 and Sp2
j. T7 and T8 (or T3 and T4)
d. F7 and F8
The electrode nomenclature for the original
10-20 electrode system is shown. This original
naming system is still in use in many EEG laboratories. Newer modified version of 10-20
replaces T3 and T4 with T7 and T8, to fit with
the 10-10 system (right).
- Posterior temporal lobe
EEG electrode position:
a. A1 and A2
b. C3 and C4
c. F3 and F4
d. F7 and F8
e. Fp1 and Fp2
f. O1 and O2
g. P3 and P4
h. P7 and P8 (or T5 and T6)
i. Sp1 and Sp2
j. T7 and T8 (or T3 and T4)
j. T7 and T8 (or T3 and T4)
The electrode nomenclature for the original
10-20 electrode system is shown. This original
naming system is still in use in many EEG laboratories. Newer modified version of 10-20
replaces T3 and T4 with T7 and T8, to fit with
the 10-10 system (right).
- A 2-year-old child accidentally hits his
head on the kitchen table when running.
He crys, turns pale and collapses to the floor.
He starts to respond after 30 s but is groggy.
Mimics of epilepsy:
a. Cardiogenic syncope
b. Cough syncope
c. Gastroesophageal reflux
d. Narcolepsy
e. Neurally mediated syncope (vasovagal)
f. Night terrors
g. Non-epileptiform attack disorder
(pseudoseizure)
h. Paroxysmal dyskinesias
i. Paroxysmal vertigo
j. Rages
k. Reflex anoxic seizure
l. Shuddering attack
m. Tic
k. Reflex anoxic seizure
- A 6-month-old baby is seen to exhibit paroxysmal dystonic posturing after feeding
Mimics of epilepsy:
a. Cardiogenic syncope
b. Cough syncope
c. Gastroesophageal reflux
d. Narcolepsy
e. Neurally mediated syncope (vasovagal)
f. Night terrors
g. Non-epileptiform attack disorder
(pseudoseizure)
h. Paroxysmal dyskinesias
i. Paroxysmal vertigo
j. Rages
k. Reflex anoxic seizure
l. Shuddering attack
m. Tic
c. Gastroesophageal reflux
- A 14-year-old boy starts to feel dizzy then loses consciousness during a car journey on a hot day. He slumps in his seat and
his limbs are seen to jerk.
Mimics of epilepsy:
a. Cardiogenic syncope
b. Cough syncope
c. Gastroesophageal reflux
d. Narcolepsy
e. Neurally mediated syncope (vasovagal)
f. Night terrors
g. Non-epileptiform attack disorder
(pseudoseizure)
h. Paroxysmal dyskinesias
i. Paroxysmal vertigo
j. Rages
k. Reflex anoxic seizure
l. Shuddering attack
m. Tic
e. Neurally mediated syncope (vasovagal)
