Congenital bilateral perisylvian syndrome:a case report / 北京大学学报(医学版)

ABSTRACT

Congenital bilateral perisylvian syndrome(CBPS) is rare in literature,especially in China.In this article,we report the clinical and treatment of a patient with CBPS and discuss its mechanism,clinical features and therapy.This patient was a 28-year-old man.His main clinical features were pseudobulbar palsy,cognitive deficits and intractable epilepsy.MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria.The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe.Rhythmal 4 Hz ? waves exist in left frontal and parietal lobe.As the epilepsy was poorly controlled by antiepileptic,section of the corpus callosum was carried out.After callosotomy,there was pronounced seizure reduction and intelligence development improvement.CBPS is characterized by pseudobulbar palsy,cognitive deficits,and bilateral perisylvian abnormalities in imaging studies.If intractable epilepsy is combined,callosotomy may be effective.