~100 cases have been reported; this may increase with improved awareness of the condition.

Age at onset
3 to 20 months; peak at 5 to 6 months.

Males = females in the non-familial form but more females are reported in the familial cases.

Neurological and mental status

The familial form is most likely autosomal dominant disorder with genetic heterogeneity (chromosomes 19q, 16, or 2).

Clinical manifestations
Diurnal focal seizures of motion arrest, decreased responsiveness, staring, eye and head deviation, simple automatisms, and mild clonic movements. They may or may not progress to generalized convulsions. Alternating from one side to the other side is common. Duration is usually short, from 30 sec to 3 min. They occur in clusters of a maximum of 8 to 10 per day for 1 to 3 days and may recur after 1 to 3 months.

Diagnostic procedures
All relevant tests are normal.

Inter-ictal EEG

Ictal EEG
Focal discharges.

Excellent, with normal development and complete seizure remission.

Differential diagnosis
Difficult in sporadic form that requires long follow-up.

Management options
In the active seizure period, empirical drug treatment is usually effective. This is usually withdrawn after 1 to 3 years.

This section was adapted from:

The educational kit on epilepsies: The epileptic syndromes By C. P. Panayiotopoulos Originally published by MEDICINAE, 21 Cave Street, Oxford OX4 1BA First published 2006 and reprinted in 2007. The Educational Kit on Epilepsies was produced through an unrestricted educational grant from UCB Pharma SA. UCB Pharma SA assumes no responsibility of the views expressed and recommended treatments in these volumes.

Authored By: 
C. P. Panayiotopoulos MD, PhD, FRCP
Authored Date: 
Reviewed By: 
Steven C. Schachter MD
Sunday, June 1, 2008