ABSTRACT

Subacute sclerosing panencephalitis (SSPE) is a serious disorder of the central nervous system. It is a slow virus infection caused by defective measles virus (see Table 7.1). The term 'subacute sclerosing panencephalitis, has been used since 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 In fact, SSPE had originally been described as three different neuropathological entities. In 1933 Dawson, for the first time, described a child with progressive mental deterioration and involuntary movements who, at necropsy, was found to have a dominant involvement of grey matter in which neuronal inclusion bodies were abundant.2 He suggested the term 'subacute inclusion body encephalitis,. Later Pette and Doring (1939) reported a single case of what they called ,nodular panencephalitis,, a disease with equally severe lesions in both grey and white matter.3 Six years later, Van Bogaert drew attention to the presence of dominant demyelination and glial proliferation in the white matter and suggested the term 'subacute sclerosing leukoencephalitis,.4 A viral aetiology was suggested by Dawson, but it was Bouteille et al., in 1965, who on electron microscopy demonstrated the presence of viral structures resembling measles virus in the brain.5 In 1969 measles virus was actually recovered from the brain of a patient with SSPE.6 Since then a lot of progress has been made towards understanding of

this potentially lethal disorder. Various treatment modalities have been tried with little success. In this article all recent information will be reviewed.