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.