NOTES ON THE PATHOGENESIS OF SUBACUTE SCLEROSING PANENCEPHALITIS

In the original description by van Bogaert and De Busscher of subacute sclerosing leukoencephalitis (SSLE), great emphasis was placed upon the involvement of the white matter, a feature that, in addition to the absence of inclusion bodies, differentiated it from subacute inclusion body encephalitis (SIBE) of Dawson. Subsequently, the common features, primarily clinical, electroencephalographic and immunological led to the consolidation of both into the entity known as subacute sclerosing panencephalitis (SSPE). The white matter lesions of SSLE are identical to those that are seen in progressive rubella encephalitis, subacute AIDS encephalomyelitis, tropical spinal paraparesis due to HTLV-1, and visna of Icelandic sheep, but, more importantly, are characterized by the perivascular edema, inflammation and demyelination known in acute, immune-mediated post-infectious and post-vaccinal acute disseminated encephalomyelitis (ADEM). Furthermore, in SSLE and in the other conditions resulting from a persistent viral infection, deposits of immune complexes can be demonstrated in the walls of small cerebral blood vessels. There is therefore strong evidence to suggest that in SSLE as well as in the other persistent viral infections, in addition to the actual invasion by the virus, there is a contemporaneous immune-mediated response to this virus which is responsible for most, perhaps even all of the disseminated, extensive demyelination observed in these conditions. It is also suggested that SSLE and SIBE, sharing a common etiology, may represent two different phenotypic expressions of the same process. In the former, recognition of the early stage of cognitive deficit and deterioration of scholastic performance, coupled with evidence of widespread white matter involvement by magnetic resonance imaging should lead to considering treatment with corticosteroids (or ACTH) as anti-inflammatory agents, or even with immunosuppressive agents. © 1990.