PMID: 9530947Apr 8, 1998Paper

A clinically recognizable neuronal migration disorder: congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up

Seizure : the Journal of the British Epilepsy Association
B Baykan-KurtA Calişkan

Abstract

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental ...Continue Reading

References

Jan 1, 1989·Annals of Neurology·P S BeckerJ C Troncoso
Nov 1, 1986·Annals of Neurology·N R Graff-RadfordD Tranel

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Citations

Jan 10, 2014·Journal of Clinical Neonatology·Anand K GowdaAshok Kumar
Jun 30, 2009·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Anja De CoeneHelene Verhelst
Mar 16, 2004·Acta Neurologica Scandinavica·J A AhnlideB Geijer

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