Ophthalmoscopic findings in subacute sclerosing panencephalitis.

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive central nervous system disorder affecting both grey and white matter; it affects children and young adults and, with only occasional exceptions, is fatal. Present interest in the illness stems from the observation that the disease is due to a slow virus infection produced by measles or a measles-like virus (Horta-Barbo-a, Fuccillo, and Sever, I97I). While several antiviral drugs have been tested, no specific therapy has so far been found (Haslam, McQuillen, and Clark, I 969). The incidence of SSPE is about one per million in the United States of America. The male to female ratio is about 4:i and the disease is more common in rural than urban areas. The patient may present with visual problems, behaviour disorders, and memory impairment; then there follows myoclonus and further deterioration including spastic quadriparesis and dementia (Freeman, I 969). The electroencephalogram shows periodic complexes, the gamma globulins are raised in the cerebrospinal fluid, and the measles antibody titres are chronically raised in both blood and cerebrospinal fluid. There is a relatively high incidence of SSPE in the Kentucky-Tennessee area of the United States (Jabbour, Garcia, Lemmi, Ragland, Duenas, and Sever. i969). Because many of these cases present initially with one or more ophthalmological problems, we have reviewed all twenty cases with this condition seen at the Neurology Department of the University of Kentucky Mledical Center with particular reference to the ophthalmoscopic findings. In some cases the ophthalmological examination may disclose findings which readily suggest a more diffuse neurological disease (Table I), but in other cases the findings