The early phase in Leber hereditary optic atrophy
E. Nikoskelainen, R. L. Sogg, A. R. Rosenthal, T. R. Friberg and L. J. Dorfman
Clinical, ophthalmoscopic, perimetric, and color vision tests and visual
evoked responses were recorded in symptomatic and asymptomatic eyes in five
members of a family with Leber hereditary optic atrophy. The presymptomatic
eyes showed abnormalities in the retinal nerve fiber layer. Optic nerve
dysfunction was found before central vision failed. The appearance of
degenerating retinal nerve fibers, particularly in the papillomacular
bundle, was documented photographically in the weeks after the onset of
visual symptoms. Treatment with prednisone and hydroxocobalamin did not
reverse or halt serious impairment of vision.
