Newly recognized syndrome of reflex epilepsy with an age-related onset.

Prevalence
Low (0.4% of all epilepsies).

Age at onset
15 months to 19 years; mean ~12 years.

Sex
Males (60%) slightly predominate.

Etiology
Part of the benign childhood seizure susceptibility syndrome.

Clinical manifestations
Reflex seizures identical to those of spontaneous occipital lobe epilepsy: elementary visual hallucinations, blurring of vision or blindness, alone or in combination. Deviation of the eyes and head, eyelid fluttering, and orbital pain are less common.

Occipital seizures may progress to autonomic symptoms like those of Panayiotopoulos syndrome. Ending with secondarily generalized tonic-clonic seizures (GTCS) is uncommon.

Spontaneous visual or other seizures (myoclonic jerks, absences, and GTCS) may also happen.

Occipital seizures with bizarre ictal symptomatology mimicking hysterical attacks or migraine are well reported.

Post-ictal symptoms include headache, nausea, and vomiting.

Precipitating factors
Flickering lights of minimal photic to combined pattern-photic provocation or prolonged exposure. Photic stimuli are often effective only if combined with other facilitating factors (excitement, fatigue, sleep deprivation).

Animated cartoons on television and video games are common precipitators.

EEG
Intermittent photic stimulation (IPS) elicits (1) photoparoxysmal responses (PPR) of occipital spikes/polyspikes or (2) generalized PPR of spikes/polyspikes with posterior emphasis.

Spontaneous posterior or centrotemporal spikes may occur.

Ictal EEG
Occipital discharge of fast spikes ending spontaneously or spreading to temporal regions or progressing to GTCS.

Visually evoked responses are of abnormally high amplitude.

Differential diagnosis
Migraine (rarely a problem if symptoms are appropriately analyzed), Gastaut-type idiopathic childhood occipital epilepsy (of no prognostic significance other than classification and avoidance of precipitating factors), idiopathic photosensitive generalized epilepsy (probably important in management), and psychogenic seizures (sometimes very difficult to differentiate).

Prognosis
Usually good. Patients may have seizures only during potent IPS or only 1 to 2 seizures in life despite exposure to precipitating factors and no drug treatment. Those with additional spontaneous seizures need medication and avoidance or cautious exposure to insulting stimuli.

Management options*
Avoidance of precipitating factors. Valproate and levetiracetam but also lamotrigine, carbamazepine, and clobazam# are treatment options if AEDs are needed.

*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
21 Cave Street, Oxford OX4 1BA

First published 2006 and reprinted in 2007

Authored by: C. P. Panayiotopoulos MD PhD FRCP on 1/2005
Reviewed by: Steven C. Schachter MD on 6/2008
ADVERTISEMENT
ADVERTISEMENT