Subacute sclerosing panencephalitis (SSPE)
Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. CLINICAL PRESENTATION: Patients usually present with behavioral changes, myoclonus, dementia and visual disturbances with pyramidal and extrapyramidal signs. They undergo progressive mental deterioration and involuntary movements. At necropsy, they are often found to have a dominant involvement of grey matter in which neuronal inclusion bodies are abundant. PROGNOSIS : The disease has a gradual progressive course leading to death within 1-3 years DIAGNOSIS: It is based upon the characteristic clinical manifestations, the presence of periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. TREATMENT: Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease. Credits: https://pmj.bmj.com/content/78/916/63 https://images.app.goo.gl/rjx6Dkq6sBTjX3CfA