Retinal Lesions in Subacute Sclerosing Panencephalitis

Abstract

Two teen-age girls died from a progressive neurological disease diagnosed at autopsy as subacute sclerosing panencephalitis. The presenting symptom in one patient was loss of vision; in the other, akinetic seizures. One patient lost central vision in one eye, then in the other. Ophthalmoscopic examination revealed a macular lesion consisting of edema, pigmentation, and hole formation. The other patient did not have visual complaints during the illness, and retinal lesions were not demonstrated during life, except for some pallor of the optic discs. At autopsy, however, lesions of the retinas were noted. Both patients showed numerous inclusion bodies in the brain and neuronal cells of the retinas. To our knowledge, inclusion bodies previously have not been demonstrated in retinal lesions of patients with this disease.