Epilepsy: Continuum (Pediatrics) Flashcards
West Syndrome:
Onset and semiology
<2 years old / clusters of neck/truncal flexion with arm abduction often seen in the AM
West Syndrome:
Clinical triad
Psychomotor arrest, hypsarrhythmia and spasms
What is hypsarrhythmia
Seen in West Syndrom. High amplitude slow waves on a background of irregular multifocal spikes.
West Syndrome:
Treatment
ACTH first line. Vigabatrin in tuberous sclerosis.
Dravet Syndrome (severe myoclonic epilepsy of infancy): Onset and Semiology
< 2 years old. Hemiconvulsions with myoclonic and atypical absence
Dravet Syndrome: EEG specific ?
No
Dravet Syndrome: clinical symptoms and tx
regression with ataxia. NEVER sodium channel blockers (scn1a mut). VPA/BZD/LTC are common first line.
Benign epilepsy with centrotemporal spikes (BECTS or benign rolandic):
Onset and Semiology
age 7 with focal seizures that can be autnomic and focal involving mouth/throat. Tonic clonic as well.
Benign epilepsy with centrotemporal spikes (BECTS or benign rolandic):
EEG prognosis and tx
Centrotemporal BIPHASIC spikes/sharps that worsen in sleep. The are usually fine by age 16. Usually no tx but can use carbamazepine.
Lennox Gastaut (LGS: Triad
multiple seizure types, cognitive impairment, and slow spike and wave (1.5-2.5 Hz)
Syndrome that can precede LGS
West syndrome
LGS:
Tx and Prognosis
Can try ACTH, ketogenic diet but very pharmacoresistant with poor outcome.
Childhood Absence epilepsy:
Onset and semiology
ages 4-8 (age >9 is juvenile absence)
Staring spells but 30% can have GTC
Childhood Absence epilepsy:
EEG
3 Hz spike an wave with no post ictal slowing
Bedside test for childhood absence epilepsy
Hyperventilation for 3 minutes
Childhood Absence epilepsy:
Tx and Prognosis
Ethosuximide
70 % remit with 30% developing GTC which merits an added AED.
JME:
Onset and semiology
teenage years Mycolonic jerks (clumsy in AM) with potential for GTC
JME:
EEG
4-6 Hz Generalized spike/polyspike and wave discharge that are photosensitive
JME:
Tx and Prognosis
Lamotrigine or VPA indefinitely . Avoid photic stimuli/alcohol/stress and sleep deprivation
What is epilepsia partialis continua and what is it associated with ?
continuous focal clonic seizures without impairment of consciousness. Associated with Rasmussen’s encephalitis.
Gene for Autosomal dominant partial epilepsy with auditory features
LGI1
Syndrome with language regression and electrical status epileptics in sleep
Landau-Kleffner
