~2% to 7% of benign childhood focal seizures.

Age at onset
3 to 15 years; mean ~8 years.

Males = females.

Neurological and mental state

Probably genetically determined.

Clinical manifestations
Frequent visual seizures of mainly elementary visual hallucinations, blindness, or both. Ictal elementary visual hallucinations consist of small multicolored circular patterns that often appear in the periphery of a visual field. They develop fast in sec and last for several sec to 1 to 3 min. These may progress and co-exist with other occipital symptoms (sensory illusions of ocular movements and ocular pain, tonic deviation of the eyes, repetitive eye closures). Complex visual hallucinations, visual illusions, and other symptoms from anterior spreading may terminate with hemiconvulsions or generalized convulsions. Ictal blindness usually appears suddenly and usually lasts for 3 to 5 min.

Ictal headache, mainly orbital, occurs in 1/10 of patients.

Consciousness is usually intact. It becomes impaired with seizure progression.

Spreading to temporal lobe involvement is exceptional and may indicate a symptomatic cause.

Post-ictal headache, sometimes indistinguishable from migraine headache, occurs in half of patients.

Predominatly in alert stages.

Diagnostic procedures
All tests other than EEG are normal. High-resolution MRI is mandatory because symptomatic occipital epilepsies may have similar clinico-EEG manifestations.

Inter-ictal EEG
Occipital paroxysms, often with fixation off sensitivity. Others may have only random occipital spikes, some may have occipital spikes in sleep EEG alone, and a few may consistently have normal EEG. Whether occipital photosensitivity is part of this syndrome or not is debated.

Ictal EEG
Occipital discharge of fast rhythms, fast spikes, or both.

Relatively good. Half of patients will remit within 2 to 4 years from onset. The others will continue having visual seizures and infrequent secondarily generalized tonic-clonic seizures, particularly if not appropriately treated with carbamazepine.

Differential diagnosis
Migraine, symptomatic occipital epilepsy, and coeliac disease with visual seizures.

Management options*
Drug treatment, mainly with carbamazepine, is probably mandatory.

*Expert opinion, please check FDA-approved indications and prescribing information

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