An Unusual Cause of Fatal Acute Hyperammonemic Encephalopathy in a Non Cirrhotic Patient – Case Report

Abstract

Hyperammonemia can be secondary to hepatic or non hepatic and idiopathic causes. Among non hepatic or non cirrhotic causes of hyperammonemia, excess of ammonia production or decrease in ammonia excretion predominates to produce the clinical syndrome. Cirrhosis is the commonest cause of hyperammonemia in adults. In children, disorders of urea cycle need to be considered as a suspecting but rare cause. Here we present a middle aged non cirrhotic female patient who presented to us in a state of shock, in whom, severe septicemia with urea-splitting organisms along with sepsis related cardiogenic shock leading to ischemic hepatitis led to severe hyperammonemia eventually leading to brain stem herniation and immediate death.