Subacute Sclerosing Panencephalitis (SSPE) in a 10 ½ Year Old Male Child

Edwin Dias

doi:10.47992/ijhsp.2581.6411.0029

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence, caused by persistent defective measles virus. A 10 ½ yearold male child with h/o having normal milestones till the age of 15 months had an episode of measles for which child was hospitalized. After one year he showed gradual deterioration of already attained milestones but continued regression of milestones noticed, presented to the department at 10½ years with h/o not getting up from the bed. Patients usually have behavioural changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs and can cause death within 1-3 years of presentations. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is being researched. A combination of oral Isoprinosine and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it lifelong. At present effective measles vaccination is the only solution to SSPE.

Highlights

Subacute sclerosing panencephalitis (SSPE) is a disorder of the central nervous system, a slow virus infection caused by defective measles virus
Pathogenesis: SSPE virus is distinguished from the wild type of measles virus in that there appears to be a defect in assembly of the virus within the nervous system, and which is related to an abnormality of matrix of ‘M’ protein of the virus
SSPE is a slow virus infection caused by aberrant measles virus

Summary

Introduction

Subacute sclerosing panencephalitis (SSPE) is a disorder of the central nervous system, a slow virus infection caused by defective measles virus. Greenfield suggested it in 1960 to designate a condition due to a persistent infection by a virus involving both grey matter and white matter [1]. For the first time, described a child with progressive mental deterioration and involuntary movements who, at a necropsy, was found to have a dominant involvement of grey matter in which neuronal inclusion bodies were abundant [2] using the term “subacute inclusion body encephalitis”. In1969 measles virus isolation was done from the brain of a patient with SSPE [6]

Discussion

Conclusion