Abstract
The authors report a case of congenital portal-systemic encephalopathy (PSE) of which symptom developed from focal neurological sign (left hemiparesis) to diffuse encephalopathy (conscious disturbance). The patient was 70 year-old man who was initially diagnosed as transient ischemic attack with hemiparesis and a short term unconsciousness, and thereafter developed repeated attacks of the same pattern. The brain X-CT showed multiple cerebral infarctions, which was not a direct reason for the attacks. Ictal serum anmonia level was abnormally higher than interictal one. Portal systemic shunt was confirmed by percutaneous portal angiography and was occluded by endovascular surgery using coils. Anmonia level was normalized and the symptoms disappeared. But it began to elevated 3 months later when he was attacked by conscious disturbance again, probably because of recanalization or opening of collateral circulation.
We discussed focusing on focal neurological deficits which rarely occurred in the metabolic encephalopathy, and speculated that it depended upon decrease of tolerance of the brain foci to anmonia and metabolites derived from the portal systemic shunt. This decrease of tolerance was thought to be based on senile and ischemic change of the brain foci.
