Stéphanie Baillif1, Nathalie Tieulie, Viviane Queyrel, Pierre-Loïc Cornut, Pierre Gastaud. 1. *Department of Ophthalmology, Saint-Roch University Hospital, Nice, France †Department of Internal Medicine, l'Archet University Hospital, Nice, France ‡Department of Ophthalmology, Hospices Civils de Lyon, University Hospital, Claude Bernard University, Lyon, France.
Abstract
PURPOSE: To report the clinical, angiographic, and tomographic features of an adult patient with bilateral occlusive vasculitis and necrotizing retinitis preceding the onset of subacute sclerosing panencephalitis by 3 years. METHODS: Observational case report. RESULTS: A 39-year-old white man presented with sudden onset of decreased vision in his left eye. The patient reported a history of right visual loss 3 years ago after an episode of retinal vasculitis of unknown origin. At presentation, left fundus examination showed sensory retinal detachment and retinal hemorrhages in the left macula. Fluorescein angiography revealed occlusive vasculitis. Two weeks later, several circumscribed lesions of necrotizing retinitis appeared in the macula and in the midperiphery after a centrifugal linear pattern from the optic nerve. Spectral-domain optical coherence tomography demonstrated focal areas of moth-eaten disorganization of the ganglion cell and nuclear layers with a spreading from the inner layers to the outer layers. CONCLUSION: This unusual case of adult-onset subacute sclerosing panencephalitis may help improve our knowledge regarding the mechanism of retinal involvement in this disease. Clinical and tomographic findings suggest a continuous transneuronal spread of the measles virus from ganglion cells to the photoreceptor cells.
PURPOSE: To report the clinical, angiographic, and tomographic features of an adult patient with bilateral occlusive vasculitis and necrotizing retinitis preceding the onset of subacute sclerosing panencephalitis by 3 years. METHODS: Observational case report. RESULTS: A 39-year-old white man presented with sudden onset of decreased vision in his left eye. The patient reported a history of right visual loss 3 years ago after an episode of retinal vasculitis of unknown origin. At presentation, left fundus examination showed sensory retinal detachment and retinal hemorrhages in the left macula. Fluorescein angiography revealed occlusive vasculitis. Two weeks later, several circumscribed lesions of necrotizing retinitis appeared in the macula and in the midperiphery after a centrifugal linear pattern from the optic nerve. Spectral-domain optical coherence tomography demonstrated focal areas of moth-eaten disorganization of the ganglion cell and nuclear layers with a spreading from the inner layers to the outer layers. CONCLUSION: This unusual case of adult-onset subacute sclerosing panencephalitis may help improve our knowledge regarding the mechanism of retinal involvement in this disease. Clinical and tomographic findings suggest a continuous transneuronal spread of the measles virus from ganglion cells to the photoreceptor cells.