Very small; ~20 infants have been reported.
Age at onset
First week to 7 months of life; mean age 3 months.
Males = females.
Neurological and mental state
Usually normal prior to the onset of seizures.
Unknown. There is no family history. Brain neuropathology in 2 cases showed only severe hippocampal neuronal loss and gliosis.
Nearly continuous multifocal seizures with variable and alternating localizations involving the whole cortex. Initially, seizures are motor (eye and head deviation, clonic jerks of eyelids or limbs, tonic components) with secondarily generalized tonic-clonic seizures and convulsive status epilepticus. Autonomic manifestations (apnea, cyanosis, sweating, hiccups) are prominent. After 1 to 10 months, the seizures become polymorphous, very frequent, or continuous. Epileptic spasms are exceptional.
All tests, including brain imaging, are normal.
Exceptionally normal at onset. Diffuse slow activity is the rule with alternating side emphasis in serial EEGs. Multifocal spikes are prominent.
Focal discharges randomly involve multiple independent sites, moving from one cortical area to another in consecutive seizures. Duration is 1 to 4 min; subclinical discharges of 30 to 60 sec are common.
Devastating and often fatal. Rapid and relentless severe psychomotor regression with major axial hypotonia and quadriplegia.
Focal symptomatic epilepsy.
Conventional AEDs and steroids are ineffective. Treatment with stiripentol# and clonazepam or potassium bromide has been successful in a few otherwise intractable cases.
*Expert opinion, please check FDA-approved indications and prescribing information
#Not approved by the FDA
This page was adapted from:
The educational kit on epilepsies
The epileptic syndromes
By C. P. Panayiotopoulos
Originally published by MEDICINAE
Reviewed and revised June 2008 by Steven C. Schachter, MD
21 Cave Street, Oxford OX4 1BA
First published 2006 and reprinted in 2007