Autosomal dominant<unk>  atrophy can present clinically as an isolated bilateral<unk>  neuropathy (non-syndromic form) or rather as a complicated phenotype with extra-ocular signs (syndromic form). Dominant<unk>  atrophy usually affects both eyes roughly symmetrically in a slowly progressive pattern of vision loss beginning in childhood and is hence a contributor to childhood blindness. Vision testing will reveal scotomas (areas of impaired visual acuity) in the central visual fields with peripheral vision sparing and impaired color vision (color blindness). Visual acuity loss varies from mild to severe, typically ranging from 6/6 (in meters, equivalent to 20/20, ft) to 6/60 (20/200, ft) with a median value of 6/36 (roughly equivalent to 20/125 ft), corrected vision. In rare cases, vision loss is more severe. Characteristic changes of the fundus evident on examination is temporal pallor (indicating atrophy) of the<unk>  disc and in its end stage, excavation of the<unk>  disc, as is also seen in Leber hereditary<unk>  neuropathy and normal tension glaucoma. Because the onset of Dominant<unk>  atrophy is insidious, symptoms are often not noticed by the patients in its early stages and are picked up by chance in routine school eye screenings. First signs of<unk> 's typically present between 4–6 years of age, though presentation at as early as 1 year of age has been reported. In some cases, Dominant<unk>  atrophy may remain subclinical until early adulthood. Progression of dominant<unk>  atrophy varies even within the same family. Some have mild cases with visual acuity stabilizing in adolescence, others have slowly but constantly progressing cases, and others still have sudden step-like decreases in visual acuity. Generally, the severity of the condition by adolescence reflects the overall level of visual function to be expected throughout most of the patient’s adult life (Votruba, 1998). Slow decline in acuity is known to occur in late middle age in some families. In complicated cases of autosomal dominant<unk>  atrophy, in addition to bilateral<unk>  neuropathy, several other neurological signs of neurological involvement can be observed: peripheral neuropathy, deafness, cerebellar ataxia, spastic paraparesis, myopathy.
Kjer's optic neuropathy
Leber's hereditary optic neuropathy
Graves' ophthalmopathy
Toxic and nutritional optic neuropathy
Arteritic anterior ischemic optic neuropathy
Infiltrative ophthalmopathy
Sorsby's fundus dystrophy
Posterior ischemic optic neuropathy