Clinically, there is an acute onset of visual loss, first in one eye, and then a few weeks to months later in the other. Onset is usually young adulthood, but age range at onset from 7-75 is reported. The age of onset is slightly higher in females (range 19–55 years: mean 31.3 years) than males (range 15–53 years: mean 24.3). The male-to-female ratio varies between mutations: 3:1 for 3460 G>A, 6:1 for 11778 G>A and 8:1 for 14484 T>C. This typically evolves to very severe optic atrophy and a permanent decrease of visual acuity. Both eyes become affected either simultaneously (25% of cases) or sequentially (75% of cases) with a median inter-eye delay of 8 weeks. Rarely, only one eye is affected. In the acute stage, lasting a few weeks, the affected eye demonstrates an oedematous appearance of the nerve fiber layer, especially in the arcuate bundles and enlarged or telangiectatic and tortuous peripapillary vessels (microangiopathy). The main features are seen on fundus examination, just before or after the onset of visual loss. A pupillary defect may be visible in the acute stage as well. Examination reveals decreased visual acuity, loss of color vision and a cecocentral scotoma on visual field examination.<unk>  with demyelinating lesions or<unk>  Plus<unk>  Plus is a rare variant of the disorder with eye disease together with other conditions. Its symptoms include loss of the brain's ability to control the movement of muscles, tremors, and cardiac arrhythmia. Many cases of<unk>  plus have been compared to multiple sclerosis because of the lack of muscular control and the presence of demyelinating lesions in the CNS. It is therefore a subtype of MS, according to McDonald's definition.
Leber's hereditary optic neuropathy
Kjer's optic neuropathy
Alexander disease
Graves' ophthalmopathy
Bosch-Boonstra-Schaaf optic atrophy syndrome
Sorsby's fundus dystrophy
Neuromyelitis optica spectrum disorder
Akureyri disease