Fulminant subacute sclerosing panencephalitis: Report of a case and review of literature

Abstract

An 11-year-girl presented with rapidly progressive visual loss, which partially improved following prednisolone therapy. One month later, she developed mental changes and incontinence. She was confused, had bilateral cortical blindness and pyramidal signs. Electroencephalography revealed slowing and T2-weighted magnetic resonance imaging hyperintensity in the frontal and parieto-occipital regions. Cerebrospinal fluid was positive for measles antibody. She died on the third month of illness. Postmortem brain biopsy was positive for measles antigen. She did not have myoclonus or periodic discharges in electroencephalography during the entire course of illness. In children with visual impairment and rapidly deteriorating neurological status, subacute sclerosing panencephalitis should be considered. Cerebrospinal fluid measles antibody may clinch the diagnosis.

Keywords

SSPE - fulminant course - MRI - EEG - retrobulbar neuritis