Kasuistik über OCT-Befunde bei einem Patienten mit Leberscher hereditärer Optikusneuropathie und einem Patienten mit autosomal dominanter Optikusatrophie

Abstract

Hereditary optic neuropathies lead to a decrease in visual acuity corresponding to a reduction of the ganglion cell layer and the nerve fibre layer. The most common neuropathies, Leber hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (ADOA), are mitochondrial disorders. We describe two cases, one with LHON and one with ADOA, and demonstrate the decrease in thickness of the ganglion cell layer using spectral domain OCT (optical coherence tomography). In the case with LHON, the ganglion cell layer and the nerve fibre layer showed a considerable reduction from the initial occurrence of symptoms to the 1? years follow-up examination. In the patient with ADOA, the atrophy of the ganglion cell layer and the nerve fibre layer was clearly visible on OCT corresponding to the poor visual acuity of the patient. Spectral domain OCT instruments allow for high resolution imaging and quantitative analysis of the various retinal layers. Using this technology, it is possible to distinguish between retinal disorders of the outer retinal layers and disorders of the inner retinal layers, like in our cases with LHON and ADOA.