Panencefalitis esclerosante subaguda: una enfermedad que todavía existe

Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare entity with an invariably fatal course that progressively affects the central nervous system. It is caused by persistent infection with the wild-type measles virus. While rare in industrial countries, it is not infrequent in developing countries, where there are still areas of endemic measles infection and immunization is not yet generalized. We describe an eight-year-old Spanish girl who presented rhythmic and symmetric myoclonus. She contracted measles at 13 months and required hospitalization. No cognitive deterioration was found. Neuroimaging and the initial electroencephalogram were normal. Oligoclonal bands and high titers of measles antibodies were found in serum and cerebrospinal fluid. She was treated with oral metisoprinol and intraventricular alpha-interferon (IFN-a) and showed no further progression of her symptoms. The importance of including SSPE in the differential diagnosis of patients consulting for school failure, neurological deterioration or movement disorders is highlighted. Special attention should be paid to the immigrant population from countries where the incidence of SSPE is greater than in Spain.