EARLY-ONSET BENIGN OCCIPITAL SEIZURE SUSCEPTIBILITY SYNDROME

Purpose: Childhood epilepsy with occipital paroxysms (CEOP) is charact erised by ictal visual hallucinations and occipital epileptiform activ ity on interictal EEG. A variant has been described with nonvisual sym ptoms including tonic head and eye deviation, vomiting, and episodes o f partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. Methods: This was a multicentre study with pa rticipating investigators submitting details of patients with idiopath ic occipital seizures characterised by ictal head or eye deviation and vomiting. Results: One hundred thirteen patients were recruited. Seiz ures began in early childhood (mean, 4.6 years) and occurred infrequen tly (mean total seizures, 3); 30% of patients had only a single seizur e. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were pre dominantly complex partial in type. Partial status epilepticus occurre d in 44% of patients. Seventy-four percent of patients had occipital i nterictal EEG epileptiform activity, predominantly right sided, with f ixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year. Conclusions: Although the two groups shared ident ical EEG features, the distinct clinical symptoms probably justify sep arate classification. Early-onset benign occipital seizure syndrome (E BOSS) is suggested as an appropriate name for the variant group.