Summary:Purpose: In its recent proposal, the Commission on Classification and Terminology of the International League Against Epilepsy classified childhood epilepsy with occipital paroxysms (CEOP) into two syndromes with different predominant seizure types: early onset (Panayiotopoulos type) with eye deviation and ictal vomiting and late onset (Gastaut type) with initial ictal visual symptoms. We documented the clinical features of a large group of patients with CEOP to confirm whether the classification is justified.Methods: A file review of all patients with partial-onset seizure and interictal occipital spikes referred to our pediatric seizure unit between January 1975 and May 1997 yielded 134 who met the criteria for CEOP. Data were collected with a specially developed protocol and classified according to the two International League Against Epilepsy systems: (a) seizure classification, to test age-specific differences associated with the predominant seizure type, and (b) syndrome classification, to determine whether the clusters of signs and symptoms are sufficiently delineated.Results: Three groups were defined according to the predominant ictal manifestations. Group 1 (visual) consisted of 24 patients (17.9%) with ictal visual symptoms; 19 (14%) of these patients also had overlapping adversive manifestations, either as a separate seizure or as part of the same event (median age at first and last seizure, 7 years 11 months and 10 years). Group 2 (adversive) consisted of 72 patients (53.7%) with tonic eye deviation (median age at first and last seizure, 5 years 2 months and 7 years 2 months). Group 3 (nonvisual, nonadversive) consisted of 38 patients (28.4%) with various seizure spread patterns (median age at first and last seizure, 6 and 7 years 2 months). Two syndromes were identified. The Gastaut type included all 24 patients in the visual group (group 1); seizures were brief and frequent and were diurnal in 83%. The Panayiotopoulos type included all 72 patients in group 2; ictal eye deviation occurred in 100% of the patients and ictal vomiting in 44%; prolonged seizures were observed in 35% and were more frequent in patients who had ictal vomiting than in those who did not (46.8% versus 25%, respectively; p < 0.027). Seizures were infrequent; 24% of patients had a single seizure and 58% had nocturnal seizures. Onset was earlier than for the Gastaut type (p < 0.002). The 38 patients with nonoccipital manifestations did not satisfy the criteria for the complete form of either syndrome.Conclusions: The most common type of CEOP, the Panayiotopoulos type, is characterized by a cluster of signs and symptoms sufficiently delineated to justify their separate classification from Gastaut-type CEOP, despite the absence of ictal vomiting in more than 50% of the patients.