Abstracts

RATIONALE: Pseudobulbar palsy, epilepsy, cognitive deficits and polymicrogyria around the Sylvian fissure characterize congenital bilateral perisylvian syndrome (CBPS). Its etiology is unknown, but it has been shown that CBPS may be the result of vascular injury during cortical development, and in some cases it may be familial. METHODS: We evaluated 11 patients with CBPS. Patients and family members were questioned regarding family history of epilepsy, neurological abnormalities and the occurrence of pre-natal events during pregnancy. RESULTS: MRI showed bilateral posterior parietal polymicrogyria (BPPP) in 5 and diffuse perisylvian polymicrogyria in 6 patients. Patients with BPPP had only mild apraxia or speech delay. Patients with diffuse perisylvian polymicrogyria had pseudobulbar palsy and/or motor deficits. Epilepsy was present in 4/6 patients with diffuse perisylvian polymicrogyria and in none of the BPPP. All but one patient with BPPP and 2 of the 6 patients with diffuse perisylvian polymicrogyria had family history of epilepsy or cortical dysgenesis. Four of the 6 patients with diffuse perisylvian polymicrogyria had significant history of pre-natal events as opposed to none of the patients with BPPP. CONCLUSIONS: Our results suggest that BPPP may be a milder form of CBPS and appears to be associated with more important genetic factors and fewer antecedents of environmental factors (pre-natal events) than the diffuse (more severe) form of CBPS. This > research was supported by FAPESP.