Optic atrophy and macular degeneration as initial presentations of subacute sclerosing panencephalitis

PURPOSE: To report a case of subacute sclerosing panencephalitis in which the initial clinical presentations were optic atrophy and macular degeneration. DESIGN: Observational case report. METHODS: A 9-year-old boy with visual loss attributable to optic atrophy and macular degeneration had a diagnostic examination. RESULTS: The visual acuity levels of both eyes were light perception at presentation. Fundus examination revealed bilateral optic atrophy and macular degenerative changes including retinal pigment epithelial atrophy, macular scarring, and epiretinal membrane formation. The antimeasles IgG titers were high both in serum and cerebrospinal fluid samples. Neurologic symptoms of myoclonus, ataxia, motor, and mental disabilities occurred 4 months after the onset of first visual disturbances and 2 weeks after the patient applied to our clinic. CONCLUSION: Children with optic atrophy and macular degenerative lesions should be examined for sclerosing panencephalitis, even if the classical neurologic symptoms of this disease are not present.