Congenital bilateral perisylvian syndrome

Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disease characterized by paralysis of certain facial muscles and epileptic seizures. Signs usually appear at birth or in infancy and include paralysis on both sides of the face, facial spasms, pseudobulbar palsy, difficulty speaking, chewing and swallowing, epilepsy and intellectual disability. The underlying cause is unknown, but it is thought to arise from faulty migration of neuroblasts to the cerebral cortex in the embryo, producing bilateral perisylvian polymicrogyria, an excess of small folds on the brain surface. Diagnosis uses electroencephalography and imaging, and it must be told apart from other neuronal migration disorders. Treatment is mainly anticonvulsant therapy, with special education, speech therapy and physical therapy. Males and females are equally affected.