The frequency of different causes of occipital epileptiform discharges (OEDs) and EEG features predictive of epilepsy type and outcome were analyzed in 90 children with OEDs examined at the Floating Hospital for Children, Tufts University School of Medicine, Boston, MA. More than one half (56%) had seizures symptomatic of underlying illness, including cerebral palsy (18), cerebral dysgenesis (11), and genetic disorders (8). One third had idiopathic seizures, including 6 with benign childhood epilepsy with occipital paroxysms, 1 with febrile seizures, and 8 had no seizures. Only 2 had visual symptoms with seizures. Background slowing in the EEG was associated with symptomatic seizures in 87%; normal background EEG activity was present in 87% of those with idiopathic seizures (p<0.001). At follow-up in 72 cases, 62% (28 of 45) of those with normal EEG background had seizure remission compared to 37% (10 of 27) of those with background slowing (p=0.04). Generalized spike-wave discharges correlated with idiopathic epilepsy and a better prognosis. 
COMMENT. Most epilepsies with occipital epileptiform discharges on the EEG are symptomatic and associated with developmental delay or severe behavior problems or both. A normal background rhythm or generalized spike-wave in the EEG correlates with idiopathic seizures and a better prognosis.
Early-onset benign childhood epilepsy with occipital paroxysms was identified by the necessary 5 diagnostic criteria in 19 of 649 children with localization-related epilepsies followed at the Tokyo Women’s Medical College, Japan. An additional 22 exhibited all criteria except occipital EEG foci, and 21 lacked only ictal vomiting. Of a total of 57 patients, 74% were in remission by age 12 years. The remission rate was similar in those with or without ictal vomiting. The clinical spectrum of the syndrome is broad, and the diagnostic criteria require further clarification.