Subacute sclerosing panencephalitis in black children--a review of 18 cases

Despite the fact that measles is severe and presents in very young Black children in Natal, South Africa, no case of subacute sclerosing panencephalitis was reported from this region prior to 1982. A retrospective study was therefore made over the six-year period 1982-1987 of 18 patients who presented to the King Edward VIII teaching hospital, Durban, with clinical and laboratory features of subacute sclerosing panencephalitis. The majority of patients (66 per cent) were between 8 and 12 years of age. The mean age of onset was 9.3 years, the youngest patient being four years nine months and the oldest 14 years. The male to female ratio was 1.25:1. A previous history of primary measles infection was obtained in 44.4 per cent of cases; 62.5 per cent occurred before the second birthday. The commonest mode of presentation was personality, intellectual and behaviour disorders (83 per cent) followed by myoclonic seizures (61 per cent) and choreiform movements (28 per cent). Measles antibody was present in the CSF in all cases. The EEG was abnormal in all recorded cases with pathognomonic periodic complexes being found in 56.2 per cent. Confirmation of the diagnosis was provided by brain biopsy in two cases and by necropsy in one case. The findings of this study suggest that subacute sclerosing panencephalitis may not be as uncommon in Black children as has hitherto been thought.