Management of severe cerebral edema in the metabolic encephalopathy of Reye-Johnson syndrome.

Fifteen critically ill children with the diagnosis of Reye-Johnson syndrome were treated with techniques developed to maintain adequate cerebral perfusion pressure and levels of circulating blood glucose. One child died, three sustained neurological deficit, and nine children (70%) recovered without significant neurological dysfunction. The technique developed during the period these children were treated, the indications for their use, and factors that can interfere with maintaining adequate cerebral perfusion in patients with increased intracranial pressure from metabolic encephalopathy are described. The results suggest that neurological damage in this syndrome results from neuronal injury secondary to inadequate cerebral perfusion and/or hypoglycemia, and that neurological dysfunction like hepatic dysfunction should produce minimal mortality and morbidity if cerebral perfusion and adequate levels of circulating blood glucose are sustained during the period of increased intracranial pressure and liver failure.