Special Forms of Atrophy of the Optic Nerve

Leber's atrophy. Here there is involvement of both optic nerves without additional neurologic symptoms. In 85% of all cases, men between the ages of 20 and 30 are affected. The disorder is due to mutations in the mitochondrial DNA.

Ophthalmoscopy will reveal optic disk edema as in papillitis followed by primary optic nerve atrophy. Initial retrobulbar optic neuritis is also possible.

Functional symptoms include a large central scotoma with a peripherally limited visual field. This will lead to significant loss of vision within a few months, although the remaining vision will not decrease any further. There is no treatment.

Behr's disease (infantile recessive optic atrophy). This is also a disorder involving both optic nerves. However, in contrast to Leber's atrophy there are additional neurologic symptoms. These may include ataxia and mental retardation. The disease is an inherited autosomal recessive disorder and manifests itself in early childhood.

Ophthalmoscopy will reveal progressive optic atrophy with severe loss of visual acuity but without complete blindness. There is no treatment.

Waxy pallor optic atrophy. This disorder (Fig. 13.16) is associated with tape-toretinal degeneration, such as retinitis pigmentosa.

Ophthalmoscopy will reveal an optic disk with a wax-like pallor that is shallow with a well defined margin. There will be severe thinning of the central retinal vessels. The cause of the wax-like yellow color is not known. There is no treatment.

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