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Dominant hereditary optic atrophy
Review Articles from PubMed
Dominant optic atrophy (DOA) and Leber hereditary optic neuropathy (LHON) are the two most common inherited optic neuropathies encountered in clinical practice. This review provides a summary of recent advances in the understanding of the clinical manifestations, current treatments, ...
Editorials from PubMed
Research Articles from PubMed
: Mitochondrial optic neuropathies such as Leber's Hereditary Optic Neuropathy (LHON) and Dominant Optic Atrophy (DOA) have been shown to produce an optic neuropathy secondary to retinal ganglion cell loss with thinning of the retinal ganglion cell complex (RGCC). : We performed a ...
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.
Patients with long-lasting bilateral optic atrophy showed typical clinical features of autosomal dominant optic atrophy (ADOA). Molecular genetic analysis identified them as atypical cases of Leber's hereditary optic neuropathy (LHON).