Childhood Epilepsy Syndrome Etiology Epidemiology

Benign Rolandic Epilepsy Unknown. 7–9 years-old.

Generalized discharges
occur from abnormal
oscillatory rhythm in
thalamocortical circuits.
Childhood Absence Epilepsy 4–6 years-old.
Thought to involve high
density of T-type
calcium channels in the
thalamus.

Healthy young
Juvenile Myoclonic Epilepsy Presumed genetic.
teenager.
 Tuberous sclerosis,
brain malformations,
Infantile Spams
metabolic 3–7 months-old.
(West Syndrome)
abnormalities,
pyridoxine deficiency.

Many causes: genetic

disorders,
neurocutaneous
syndromes,
encephalopathies. 40%
Lennox-Gastaut Syndrome 3–5 years-old.
of patients have an
unknown etiology. 25%
of patients have a
history of infantile
spasms.

Most common seizures

Associated with a fever in childhood. Most
Febrile Seizures
of greater than 100.4 F. children are less than 5-
years-old.

Mutations in SCN1A, a
gene that encodes the
Dravet Syndrome alpha unit of the First year of life.
voltage-gated sodium
channel.
 Clinical Features Diagnosis Management

Simple focal seizure EEG reveals

with motor symptoms centrotemporal spikes
Treatment may not be
that initially involve the in sleep recordings.
necessary.
face and progress to
other areas of the body. Imaging normal.

Automatisms, loss of
consciousness, no EEG reveals 3 Hz spike
Ethosuximide.
post-ictal state, no loss and waves.
of tone.

Interictal EEG reveals 4

to 6 Hz bilateral
polyspike and slow
wave discharges with
Myoclonic jerks (most frontal predominance
frequent in the over a normal
morning), absence background activity. Valproate is first-line.
seizures, generalized
tonic-clonic seizures. Ictal EEG reveals
irregular 3 to 4 Hz
polyspike-waves with
fronto-central
predominance.
 Interictal EEG reveals
hypsarrhythmia.
ACTH, vigabatrin
Symmetric,
Neuroimaging is (especially in tuberous
synchronous spasms
performed to identify sclerosis).
lesions associated with
the disorder.

Optimal therapy may

Multiple seizure types
depend on the
(tonic and atypical
underlying etiology. No
absence), a slow spike- Slow (less than 2.5 Hz)
drug is highly effective,
wave pattern on the spike-wave pattern on
but some drugs are
interictal EEG, mental the interictal EEG that
possibly helpful
retardation, psychotic is generalized and has
(clobazam,
symptoms. the highest amplitude in
cannabidiol), ketogenic
Neurodevelopment is the frontal region.
diet, vagus nerve
normal before the first
stimulation, surgical
seizure.
options.

Simple: generalized
and less than 15
minutes. Simple: lumbar
puncture, blood studies,
Complex: focal, or imaging, and EEG not
greater than 15 necessary.
minutes, or recurrent
with 24 hours.

Febrile, clonic seizure At increased risk for

EEG findings evolve
in the setting of normal premature mortality due
over time, becoming
cognitive and motor to sudden unexpected
progressively more
development, apraxic death in epilepsy and
abnormal.
gait. status epilepticus.
 Prognosis

Resolve by puberty.

Resolve by
adolescence.

Life-long disorder.
 Prognosis is poor.
Mortality Is high and
most patients have
impaired
neurodevelopmental
outcome.

Difficult to manage
medically and have a
poor seizure and
neurologic prognosis.
Mortality is also high.