Childhood Epilepsy With Occipital Paroxysms: Clinical Variants in 134 Patients

Abstract

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.

Panayiotopoulos syndrome and diffuse paroxysms as the first EEG manifestation at clinical onset: a study of nine patients

“…Panayiotopoulos et al (2012) stated that generalized paroxysms, as the sole EEG manifestation in children with PS, may occur in 4% of cases. In the most informative studies of patients with PS published, generalized paroxysms were found in 0% to 23.7% of cases (Ferrie et al, 1997;Oguni et al, 1999;Kivity et al, 2000;Verrotti et al, 2000;Lada et al, 2003;Ohtsu et al, 2003;Caraballo et al, 2007;Durá-Travé et al, 2008;Specchio et al, 2010). In the literature, no reports of patients with PS who had only generalized discharges on EEG at onset have been published.…”

“…One year earlier, Panayiotopoulos (1988) had emphasized vomiting as an ictal symptom in epileptic seizures in children. Since then, important studies of children with this syndrome have been published (Caraballo et al, 1997(Caraballo et al, , 2000(Caraballo et al, , 2007Ferrie et al, 1997Ferrie et al, , 2006Oguni et al, 1999;Kivity et al, 2000;Verrotti et al, 2000;Lada et al, 2003;Covanis et al, 2003;Ohtsu et al, 2003;Durá-Travé et al, 2008;Specchio et al, 2010). The authors studied the variants of childhood epilepsies with occipital paroxysms (CEOP) based on a retrospective analysis of patients and confirmed the existence this earlyonset variant.…”

“…Since then, important studies of children with this syndrome have been published (Caraballo et al , , , ; Ferrie et al , , ; Oguni et al , ; Kivity et al , ; Verrotti et al , ; Lada et al , ; Covanis et al , ; Ohtsu et al , ; Durá‐Travé et al , ; Specchio et al , ). The authors studied the variants of childhood epilepsies with occipital paroxysms (CEOP) based on a retrospective analysis of patients and confirmed the existence this early‐onset variant.…”

Epilepsy classification and additional definitions in occipital lobe epilepsy

“…Kivity et al . () pointed out that 28% of their patients showed mixed signs and symptoms of both syndromes. In our study, 28% of the patients with pOLE, 20% of the patients with the typical autonomic symptoms of PS, and 21% of the patients with the typical visual symptoms of OLE‐G could not be classified as either PS or OLE‐G.…”

“…In the literature, studies on childhood OLE have indicated that many patients could not be classified as either PS or OLE-G. Genizi et al (2007) reported that half of their patients with occipital paroxysms could not be segregated into either specific syndrome. Kivity et al (2000) pointed out that 28% of their patients showed mixed signs and symptoms of both syndromes. In our study, 28% of the patients with pOLE, 20% of the patients with the typical autonomic symptoms of PS, and 21% of the patients with the typical visual symptoms of OLE-G could not be classified as either PS or OLE-G. With regard to the classification of OLEs, the most important factor in the literature appears to be the method of study.…”