The eleciroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G) are analyzed in a study of 33 patients identified and followed between 1990 and 2007 at the Hospital National de Pediatria, Buenos Aires, Argentina. In comparison, over the same 16-year period, 201 children with Panayiotopoulos syndrome (PS) and 418 with benign childhood epilepsy with centrotemporal spikes (BCECTS) were registered. Age at onset of COE-G ranged from 4 to 16 years, with a mean of 8.5 yrs. Febrile seizures occurred in 5 patients (15%), migraine in 3 (9%), and BCECTS in 2 (6.5%). Family history was positive for epilepsy in 21%, febrile seizures (21%), and migraine (12%). Visual hallucinations were the initial seizure manifestation in 27 (82%). Blindness was the presenting symptom in 17 (52%) and the only clinical symptom in 5 patients. Postictal blindness and hemianopsia occurred in 7 patients (21%). Deviation of the eyes and ipsilateral turning of the head followed the visual hallucination in 20 patients (60%), and hemiconvulsions occurred in 45%. Eyelid closure and blinking occurred in 6 (18%). Migraine manifestations were prominent in 16 patients (48%). The duration of seizures was usually brief and <l-2 min. Seizures occurred while awake but sometimes in sleep. Frequency varied from 7 per week to one every 6 months. EEG showed occipital spike-wave paroxysms with eyes closed, disappearing with eyes open. All received antiepileptic treatment with valproic. acid (15), carbamazepine (8), or oxcarbazepine (4). Seizures remitted within 2 to 7 years (mean, 4 yr) after onset in 80%, while EEG abnormalities persisted in 38%. AEDs were discontinued without relapse after 2-4 years in 54%. [1]

COMMENT. Childhood occipital epilepsy (COE) of Gastaut is manifested by brief seizures, mainly visual hallucinations, illusions or amaurosis, followed by hemiclonic seizures while awake, postictal migraine headaches, mean age at onset of 8.9 years, and interictal occipital spike-wave EEG paroxysms that attenuate when eyes are opened. Prevalence is estimated at 0.2-0.9% of epilepsies, and 2-7% of benign childhood focal seizures. Gastaut reported the syndrome in 1982, and it was accepted as an entity by the ILAE in 1989. Despite some recent loss of recognition, the present authors consider the syndrome as a rare but well-defined entity within the group of idiopathic simple partial (focal) epilepsies of childhood. Differential diagnoses include symptomatic occipital epilepsy, migraine with aura, and basilar migraine. Among the idiopathic COEs, the Gastaut type is of late onset and associated with visual symptoms, whereas Panayiotopoulos syndrome is of early onset and characterized by autonomic symptoms (ictal vomiting). The EEG findings alone are not diagnostic of COE. Not all children with occipital spikes develop seizures. Those with COE should be differentiated from patients with occipital spikes occurring both with eyes open and closed, and unassociated with clinical seizures. Occipital spikes also occur in children with myoclonic, absence, and photosensitive epilepsies. [2]