AGE-RELATED SECONDARY GENERALIZED EPILEPSY IN CHILDREN WITH MALFORMATIONS OF CORTICAL DEVELOPMENT
Abstract number :
2.120
Submission category :
Year :
2002
Submission ID :
2661
Source :
www.aesnet.org
Presentation date :
12/7/2002 12:00:00 AM
Published date :
Dec 1, 2002, 06:00 AM
Authors :
Maria A. Montenegro, Marilisa M. Guerreiro, Carlos A.M. Guerreiro, Li M. Li, Fernando Cendes. Neurology, University of Campinas, Campinas, SP, Brazil
RATIONALE: The type of epileptic syndrome associated with malformations of cortical development (MCD) can be variable, and the patient[ssquote]s age is one of the most import aspects for its determination. The objective of this study is to evaluate the occurrence of age-related secondary generalized epilepsy (West and Lennox-Gastaut syndromes) in children with MCD.
METHODS: From a group of 100 consecutive patients with the diagnosis of MCD confirmed by high resolution MRI, we selected those with the first epileptic seizure before 6 years old. Firstly, we analyzed the occurrence of secondary generalized epilepsy according to the type of MCD. Secondly, we analyzed if the extension of MCD favored the development of age-related secondary generalized epileptic syndrome. For this second analysis, patients were divided into groups according to the extension of dysplastic lesion: diffuse (lissencephaly, subcortical laminar heterotopia, hemimegalencephaly) or focal (schizencephaly, polymicrogyria, focal cortical dysplasia, focal subcortical heterotopias).
RESULTS: There were 54 women, ages ranging from 5 months to 70 years (mean = 17.5 years). Fifty-four patients presented their first seizure before six years old. Among them, 20 patients (37%) presented a secondary generalized epileptic syndrome: 6/9 had lissencephaly (agyria-pachigyria), 2/2 had subcortical laminar heterotopia, had hemimegalencephaly, 2/19 had focal cortical dysplasia, 4/8 had schizencephaly, had focal heterotopias and 2/8 had polymicrogyria. According to the extension of the dysplastic lesion, the group with diffuse lesion presented secondary generalized epilepsy in 73% (11/15) of patients, as opposed to only 23% (9/39) in the group with focal lesion.
CONCLUSIONS: Our results showed that secondary generalized epilepsy before six years old occur in 37% of patients with MCD, especially when the dysplastic lesion is diffuse. However, it is interesting to note that, in childhood, 23% of the patients with a focal dysplastic lesion may present secondary generalized epilepsy. This information is important because some of these patients with generalized epileptic syndrome due to a focal lesion may be candidates to epilepsy surgery.
[Supported by: FAPESP.]