PROLONGED AND ATYPICAL COURSE IN SOME CASES OF SUBACUTE SCLEROSING PANENCEPHALITIS

Subacute sclerosing panencephalitis (SSPE) is a persistent viral infec tion of the central nervous system due to a defective measles virus. B eginning with progressive cognitive malfunctioning, myoclonus and othe r less specific neurologic signs and symptoms, it usually evolves to a vegetative, decorticated state and death. Disease course and characte ristics can be highly variable. Since the widespread measles vaccinati on in the Western countries started in the late seventies and early ei ghties, the incidence of SSPE has dropped significantly. Also adult on set is more frequently seen which changes the field of differential di agnosis. In this article 3 atypical cases of SSPE are described with p eculiarities such as adult onset, pronounced extrapyramidal involvemen t, remission and prolonged disease course. Corn mon features of the di sease are emphasized to enable accurate diagnosis which is based on cl inical features, presence of high titers of serum and cerebrospinal fl uid (CSE) measles antibodies, MRI and EEG.