Acute fulminant SSPE: clinical and EEG features.

We report an autopsy-proven case of SSPE in which the course was rapidly progressive, leading to death in 2 months. The patient was admitted for investigation of visual symptoms. His examination revealed complete right homonymous hemianopia and myoclonic jerks in the right half of the body. EEG showed high voltage periodic slow-wave discharges that were often associated with jerks. Measles antibody was positive in serum and CSF. MRI showed hyperintensity in the white matter of the occipital poles. The patient became completely blind and had jerks in all four limbs. His condition continued to deteriorate and within 3 weeks he reached a state of deep coma. The myoclonic jerks became less marked and gradually completely ceased. The EEG showed diffuse slow activity without periodic complexes. The patient died approximately 2 months from onset of disease. Brain autopsy revealed severe neuronal loss with abundant inclusion bodies in the remaining neuronal and oligodendroglial nuclei, which was compatible with SSPE.