Type

Simple partial no LOC

Complex partial: LOC but appear
awake
Absences petit mal
Tonic clonic
Tonic
Atonic
Juvenile myoclonic epilepsy

Features
Unsynchronized tonic to clonic
movements with or w/o aura, never
postictal confusion
Lip smacking, GI upset, strange smell
or increased salivation, plus altered
consciousness
Classic stare with complete
unresponsiveness; o aura and no
postictal state
Eyes roll back unresponsive
rhythmic contractions long
postictal state
Asymmetric increase in tone
Sudden loss of tone in postural
muscles leads to drop attack
Myoclonic jerks of head trunk and
extremities complete loss of tone
fall forward

Diagnosis
EEG: spike, sharp waves

Tx
Carbamazepine or phenytoin

EEG: temporal lobe spikes or sharp

waves
MRI to r/o organic brain disease
EEG: spike and wave 3 second
activity

Carbamazepine or phenytoin

Generalized seizure activity

Valproic acid
2nd line: lamotrigine, carbamazepine
or phenytoin
Multiple

Low voltage fast activity, poly spike

and wave patter or elecrodecrement
EEG varied

Ethosuximide,
2nd valproic acid

Treat with valproic acid

Infantile spasm: sudden contraction of trunk and arm, flexing trunk forward and pulling arm in to extension (salaam attack)
o Presents between 3-12 months, ceases psychomotor development at age of seizure onset
o Etiology: PKU, perinatal infections, hypoxic ischemic injury, tuberous sclerosis, microcephaly
o EEG: interrictal finding of hypsarrhymia: chaotic and disorganized reading
o Tx; ACTH and prednisone, vigabatrin
o West syndrome: triad of spasms hypsarrhythima on EEG and developmental delay
Lennox Gastaut syndrome: childhood onset series refractory to treatment:
o Presents with daily multiple seizures, nocturnal tonic seizures, between 2 and 5, me, MR, behavior disorders and delayed psychomotor
development
o Abnormal interrictal by slow spike and wave complexes
Juvenile myoclonic epilepsy: myoclonic jerks in morning, and GTC upon awakening
o EEG: 3-6 Hz spike and wave,
o Tx with valproic acid and lamotrigine
Neonatal seizure
o Etiologies: hypoxic ischemic encephalopathy, intracranial hemorrhage, CNS infection metabolic and inborn errors of metabolism, CNS
malformation
o Myelination not complete at birth rate to have GTC

EEG may show burst suppression, variance

Tx with phenobarbital, fosphenytoin or benzos

Side effects of seizure drugs
o Cytopenias/aplastic anemia: carbamazepine, Ethosuximide, valproic
o Liver dysfunction, carbamazepine, VA, phenytoin, phenobarbital
o Stevens Johnson: phenytoin, pheno, lamotrigine

Benign Rolandic (aka benign childhood epilepsy) Manifest as nocturnal twitching, numbness, or tingling in the face or tongue
Lennox-Gastaut syndrome Onset in early childhood, usually consists of drop attacks from tonic or atonic seizures, which are difficult to control, and generally
involves substantial intellectual impairment
Infantile spasms (West syndrome): Appears in the first year and is associated with cortical dysgenesis and mental impairment
Rasmussen encephalitis Associated with antibodies against glutamate receptors
Reflex epilepsy Seizures that occur in response to particular stimuli
What is the inheritance pattern of benign childhood epilepsy? Autosomal dominant
What is the long-term outlook in Lennox-Gastaut syndrome? Seizures and intellectual impairment continue into adulthood.
To which hormones is West syndrome sometimes responsive? Adrenocorticotropic hormone (ACTH) and corticosteroids
What surgical treatment is effective in severe unilateral Rasmussen encephalitis? Partial hemispherectomy
What is the most common stimulus known to trigger reflex epilepsy? Visual stimuli (especially television and video games)
What two mitochondrial disorders are associated with epilepsy syndromes? MELAS (Mitochondrial myopathy, Encephalopathy, Lactic Acidosis, and Stroke
episodes) MERRF (Myoclonic Epilepsy with Ragged Red Fibers)
Typical Ictal Clinical & EEG Features by Seizure Type
Sz Type

Duration

LC

Clinical Features

PC

Typical EEG

Simple partial

530 secs

Complex partial

13 min

Secondarily
GTC

12 min after start of

Generalization

Primary GTC

12 min

Typical absence
Atypical
absence
Myoclonic
Tonic
Atonic

510 secs
1545 secs or
longer
<12 secs
520 secs
510 secs or longer

+
+

Lennox-Gastaut
syndrome
Benign rolandic
epilepsy
Absence epilepsy
Juvenile
myoclonic
epilepsy

Age of Onset
Childhood
Childhood
Childhood and
adolescence
Adolescence and
young adulthood

Motor; psychic; soma- to sensory;

special sensory; autonomic
Often follows SPS; staring;
unawareness, automatisms
Onset = SPS or CPS; head version;
asymm tonic posture; generalized
clonus
Sudden LOC; ictal cry; tonic phase;
clonic phase
Staring; eyelid fluttering
Staring, clonus; myoclonus; atonic;
confusion
Generalized rapid jerks of limbs
Sustained posture of limbs
Sudden loss of tone

+
+

Focal spikes, rhythmic; may be normal

- spheres

Focally rhythmic then spreads to hemi- spheres &

obscured by rhythmic artifact

Low-voltage rhythmicity then high-amp spikes then

rhythmic artifact
Gen 3 Hz spike-&- wave
Gen <2.5 Hz spike-&-wave

Gen polyspike & wave

Paroxysmal low-amp fast activity
Variable

Types
Tonic, atonic, myoclonic,
GTC, absence
Simple partial involving
mouth and face, GTC
Absence, GTC

Findings
MR

Myoclonic, absence, GTC

Early morning
preponderance of seizures

Phenytoin (Dilantin)
Carbamazepine (Tegretol)
Valproic acid (Depakote)

Site of Action
Na channel
Na channel
Na channel, GABA receptor

Phenobarbital
Ethosuximide (Zarontin)
Gabapentin (Neurontin)
Lamotrigine (Lamictal)
Topiramate (Topamax)
Tiagabine (Gabitril)
Levetiracetam (Keppra)
Oxcarbazepine (Trileptal)
Zonisamide (Zonegran)

GABA receptor
T-type Ca2
Unknown
Na channel glutamate receptor,
Na channel,
GABA reuptake
Unknown
Na channel
Unknown

Simple febrile seizure: brief GTC with complete

rapid recovery
Complex partial seizure, 15 min, focal recurrent
Infantile spasms
West syndrome: hypsarrhythmia, IS, mental
retardation
Benign myoclonus of epilepsy: clinically similar to
IS but resolves in 3 months
Benign myoclonic epilepsy: 4 months to 2 years,
brief myoclonic activity of neck and legs flexion
with arms extension
Benign rolandic epilepsy
Juvenile myoclonic epilepsy
Childhood absence epilepsy
Juvenile absence epilepsy: similar to CAE except
start in adolescence and have more GTC seizures
Lennox Gastaut syndrome

Nocturnal preponderance
of seizures
Hyperventilation as trigger

Treated
Partial
Partial
Partial, generalized,
absence
Partial, generalized
Absence
Partial
Partial, generalized
Partial, generalized
Partial
Partial, generalized
Partial
Partial, generalized

EEG Findings
Slow (1 to 2 Hz) spikeand-wave
Centrotemporal spikes
3-Hz spike-and-wave
4- to 6-Hz polyspikeand-wave

Treatment
Valproic acid,
lamotrigine, felbamate
Carbamazepine;
sometimes no Tx
Ethosuximide, valproic
acid
Valproic acid, lamotrigine

Side Effects
Gingival hyperplasia, coarsening of facial features, ataxia
Hyponatremia, agranulocytosis, diplopia
GI tremor, weight gain, hair loss, hepatotoxicity, thrombocytopenia
Sedation
GI symptoms channel
Sedation, ataxia
Rash, Stevens-Johnson syndrome
Word-finding difficulty, renal stones, GABA activity weight loss
Sedation
Insomnia, anxiety, irritability
Sedation, hyponatremia
Sedation, renal stones, weight l

Not required
Get MRI if focal prolonged
Hypsarrhythmia
Hypsarrhythmia

Rectal valium
ACTH
ACTH

Normal
3 Hz spike/polyspike and wave
Central temporal spikes
4-6 hx generalized polyspike wave with photo
paroxysmal response
3 Hz spike and wave (first second may be 4 hx
then decrease to 2.5 by end)
3 to 3.5 Hz spike and wave
1.5 to 2.5 Hz spike and wave

Valproate

Valproate, lamotrigine
Ethosuximide
Valproate
Valproate, clonazepam, vigabatrin

Landau Kleffner
Mesial temporal sclerosis /temporal lobe epilepsy:
gliotic scarring and atrophy of hippocampal
formation creating seizure focus

Infantile spasms

Lennox Gastaut syndrome

Generalized spike and wave 1.5 to 3 hx range

507 rhythmic sharp theta activity

Epi
Age of onset: 3-18 months,
associated w/ hypoxic
ischemic encephalopathy,
tuberous sclerosis,
intrauterine infections, brain
malformations, inborn errors
of metabolism, herpes
Onset: before 4 years of age,
seizures continue into
adulthood Multiple,
progressive seizure types,
GTCs and Drop attacks

Landau Kleffner

Onset over 7 years of age.

Dev normal but lost language
ability between 3 and 6 years
old

Benign rolandic epilepsy

Onset: 5-16 years

Stop by puberty
Partial seizures during
wakefulness, spread to
GTCs during sleep
Onset: late childhood and
adolescence
AD inheritance
Normal intelligence

Juvenile myoclonic epilepsy

Benzodiazepines, valproate
Phenytoin, phenobarbital, carbamazepine and
valproate

Sx///exam
Clusters of sudden rapid
tonic contractions of trunk
and limbs that gradually relax
over several seconds
jackknife spasms,
psychomotor arrest/dev
regression
Atonic, tonic or atypical
absence,
Have at least 2 kinds on daily
basis
Neuro abnormities
(hemiparesis, sasistc
diplegia)
MR
Progressive decline in
language skills
Seizures usually simple
partial of GTC
Behavioral problems (ADHD,
depression)
Focal onset (involving face)
and may generalize. No
LOC, often occur at night

Diagnosis
Hypsarrhythmia on EEG
(slow disorganized brain
waves with multifocal activity

Treatment
ACTH

Slow spike and wave activity,

atypical spike and wave
pattern primarily in frontal
region on EEG

Valproic acid, Topiramate

and felbemate

EEG shows diffuse or

multifocal spike and wave
discharges most prominent I
anterior/mid temporal regions
Bilateral temporal spike and
sharp wave son EEG
EEG: sharp slow discharges
localized to rolandic (central,
midtemporal or Sylvain)
regions

Valproic

Morning myoclonic jerks,

GTC upon awakening
Precipitated by sleep
deprivation or EtOh

EEG: 3-y Hz spike and wave

discharges that lead to an
impulsive petit mal

VA, lamotrigine,
Levetiracetam

Carbamazepine