Unilateral Perisylvian Syndrome with Subcortical Heterotopia: A Case Report

Abstract

Perisylvian syndrome is a neurological disorder in which a specific area of the brain (called the perisylvian area) develops abnormally, with polymicrogyria as the underlying developmental abnormality. A 21-year-old female reported to the department with complaint of two episodes of seizures per day since six months. Magnetic Resonance Imaging (MRI) revealed multiple small gyri with few sulci noted in the right perisylvian cortex and right frontal lobe, suggesting polymicrogyria. Mild volume loss of right frontoparietal lobes was seen. The small subcortical focus of grey matter signal was noted in the right frontal lobe suggesting of heterotopia. The diagnosis of the perisylvian syndrome is mainly based on the clinical and radiologic features which are treated symptomatically and by multidisciplinary rehabilitative measures as there is no cure for this syndrome.

Keywords

• genetic factors
• polymicrogyria
• seizures