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Childhood epilepsy with occipital paroxysms: clinical variants in 134 patients
1Pediatric Epilepsy Unit and EEG Laboratory, Schneider Children's Medical Center of Israel, Petah Tikva.
Insights
Childhood epilepsy with occipital paroxysms (CEOP) classification is supported by distinct clinical features. Panayiotopoulos type CEOP, characterized by eye deviation and vomiting, is a well-defined syndrome separate from Gastaut type.
Area of Science:
- Neurology
- Epileptology
- Pediatric Neurology
Background:
- The International League Against Epilepsy proposed classifying childhood epilepsy with occipital paroxysms (CEOP) into Panayiotopoulos and Gastaut types.
- These classifications are based on predominant seizure types, with Panayiotopoulos type associated with eye deviation and vomiting, and Gastaut type with visual symptoms.
Purpose of the Study:
- To evaluate the clinical features of a large cohort of patients with CEOP.
- To determine if the proposed classification into Panayiotopoulos and Gastaut types is clinically justified based on seizure characteristics and symptoms.
Main Methods:
- A retrospective file review of 134 patients diagnosed with CEOP between 1975 and 1997.
- Data were collected using a protocol and classified according to International League Against Epilepsy seizure and syndrome criteria to assess age-specific differences and symptom clusters.
Main Results:
- Three groups emerged: visual symptoms (17.9%), adversive symptoms with eye deviation (53.7%), and nonvisual/nonadversive (28.4%).
- Gastaut type comprised the visual group; Panayiotopoulos type included the adversive group, characterized by 100% eye deviation and 44% ictal vomiting, with earlier onset and more prolonged seizures compared to Gastaut type.
- Patients with nonoccipital manifestations did not fit either complete syndrome classification.
Conclusions:
- The Panayiotopoulos type of CEOP presents a distinct cluster of signs and symptoms, justifying its classification as a separate syndrome from Gastaut-type CEOP.
- This distinction holds even when ictal vomiting is absent in over half of Panayiotopoulos type cases.
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.