The prognosis of Reye's syndrome in Ireland. Can it be improved.

Abstract

Of 48 young children with Reye’s Syndrome seen between 1979/84, 73% made a complete neurological recovery. Fifty-six per cent were in coma and 90% had encephalopathy of sufficient severity for them to be treated with controlled hyperventilation and intracranial pressure monitoring by a multidisciplinary team in the Regional Paediatric Intensive Care Unit. An unfavourable outcome was statistically related to a very short prodrome, presentation in a collapsed state, early seizures and hypoglycaemia. The presence of early seizures alone could predict prognosis in 88% of cases. We postulate that the severest forms of encephalopathy are produced largely by a combination of hypoglycaemia/seizures, which result in serious permanent neurological handicap or demise. In order to improve morbidity and mortality nationally, more vigorous and much earlier therapy is essential. For all but the mildest cases (13% in our series) control of intracranial pressure should be commencedbefore transfer to a regional centre.