Background: A primary ocular manifestation of subacute sclerosing panencephalitis is known and can progress to severe visual deterioration. The rare occurrence of the disease makes diagnosis often difficult.
Case report: The lethal clinical course of a patient with subacute sclerosing panencephalitis (SSPE) is presented. The disease manifested itself with severe ophthalmic symptoms preceding clinical and neurological signs and leading to bilateral blindness. The dramatic drop of visual acuity was due to a unilateral and later in the course bilateral pigmentepitheliopathy of the posterior pole. Inflammatory signs of retinal vasculature or inner retinal layers were detected neither clinically nor by fluoresceine angiography. A typical blockage of background fluorescence was demonstrated in fluoresceine and indocyanine angiography. Within two weeks after initial symptoms optic atrophy developed in both eyes.
Conclusion: The primary lesion of retinal pigment epithelium and outer retinal layer were the prominent findings in this case. The presence of an outer retinitis of the posterior pole should alert the physician to the possibility of subacute sclerosing panencephalitis.