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Kuzniecky, R; Andermann, F
"The congenital bilateral perisylvian syndrome: imaging findings in a multicenter study. CBPS Study Group"
AJNR. American journal of neuroradiology 1994 Jan; 15(1):139-144
PURPOSE: To describe the neuroimaging findings and the clinical features in patients with the congenital bilateral perisylvian syndrome. PATIENTS AND METHODS: Evaluation including history, general and neurologic examinations, electroencephalogram, chromosomal studies, and imaging data were reviewed in 31 patients. Pathologic material was available in two patients. RESULTS: All patients had similar neurologic dysfunction, primarily pseudobulbar paresis. Dysarthria and severe restriction of tongue movements were present in all. Motor milestones were delayed in 75% of the patients and language milestones in all. Mild to moderate intellectual deficits were documented in 75% of patients (full-scale IQ = 70). Pyramidal signs were observed in 70%. Seizures were present in 87% and were intractable to medical therapy in half of this group. MR revealed bilateral perisylvian and perirolandic malformations with exposure of the insula. The malformations were symmetrical in 80% of cases. Pathologic correlation revealed four layered polymicrogyria in the affected areas. CONCLUSIONS: The congenital bilateral perisylvian syndrome is a homogeneous clinical-radiologic entity. The underlying abnormality is probably polymicrogyria

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