Myoclonic encephalopathy in Jamaican children

ABSTRACT

Four children between the ages two and five years presented at the University Hospital of the West Indies between December 1979 and June 1983 and were diagnosed as having acute myoclonic encephalopathy. All had the typical clinical picture of acute onset myoclonus simulating cerebellar ataxia, and opsoclomus. Neuroblastoma was not demonstrated in any of these patients. One had a protracted illness with a relapsing course. Remission was not induced by dexamethasone in two patients. Complete recovery was seen in only one patient. This is the first known report of acute myoclonic encephalopathy in the Caribbean. The condition, although rare, is important to recognize as remisssion may be induced by adrenocoticotrophic hormone or corticosteroids (AU)