This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Lopes, C. F. Articles by Guerreiro, M. M. Search for Related Content PubMed PubMed Citation Articles by Lopes, C. F. Articles by Guerreiro, M. M. Social Bookmarking                         What's this?

Epileptic Features of Patients With Unilateral and Bilateral Schizencephaly

Department of Neurology, State University of Campinas

Fernando Cendes, MD, PhD

Department of Neurology, State University of Campinas

Ana Maria S. G. Piovesana, MD, PhD*

Department of Neurology, State University of Campinas

Fábio Torres, MSc

Department of Medical Genetics State University of Campinas (Unicamp), Campinas, SP, Brazil

Iscia Lopes-Cendes, MD, PhD

Department of Medical Genetics State University of Campinas (Unicamp), Campinas, SP, Brazil

Maria Augusta Montenegro, MD, PhD

Department of Neurology, State University of Campinas

Marilisa M. Guerreiro, MD, PhD

Department of Neurology, State University of Campinas, mmg{at}fcm.unicamp.br

The extent of cortical maldevelopment might correlate with the severity of the clinical manifestation, such as cognitive delay or motor dysfunction. The objective of this study was to investigate the clinical features of epilepsy in patients with unilateral and bilateral schizencephaly. We studied 44 consecutive patients with schizencephaly diagnosed by magnetic resonance imaging (MRI). The epileptic features were analyzed in detail: frequency of epilepsy, median age at onset of epilepsy, semiology of seizures, characteristic features of electroencephalographic (EEG) abnormalities, epileptic syndromes, and antiepileptic drug treatment. We also verified the presence of motor disabilities. Data were analyzed according to the presence of unilateral or bilateral clefts and to the presence of open-lip versus closed-lip schizencephaly. We used the chi-square test and Fisher exact test for statistical analysis. Twenty-four patients had a unilateral cleft (group 1) and 20 patients had bilateral clefts (group 2). Ages ranged from 1 to 37 years (mean 10.6 years). Epilepsy was present in 15 (63%) patients in group 1 and in 11 (55%) patients of group 2; a history of status epilepticus occurred in 13% of group 1 and in 27% in group 2; and a history of clusters of seizures occurred in 40% of group 1 and in 45% of group 2. Eight (53%) patients in group 1 and 6 (54%) patients in group 2 were in monotherapy. Ten (67%) patients in group 1 and 7 (64%) patients in group 2 had seizures controlled with antiepileptic drugs. The frequency of EEG abnormalities was similar between groups (75% and 85%, groups 1 and 2, respectively). Statistical analysis showed no difference between the two groups in the variables mentioned above. However, motor disability was significantly more frequent and more severe in group 2. Regarding the type of schizencephaly (open lip versus closed lip), there was no difference in the frequency of patients with epilepsy, and severe motor deficit was more frequently found in bilateral and open-lip schizencephaly. The extent of the cortical maldevelopment in patients with schizencephaly does not correlate with the severity of the clinical and EEG features of epilepsy, unlike the cognitive and motor manifestations. In addition, the type of schizencephaly (open lip versus closed lip) does not correlate with the presence of epilepsy or seizure control, unlike motor deficit. (J Child Neurol 2006;21:757—760; DOI 10.2310/7010.2006.00173).

Journal of Child Neurology, Vol. 21, No. 9, 757-760 (2006)
DOI: 10.1177/08830738060210090501


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?