The International Foundation for Optic Nerve Disease
Optic Atrophy
A Good Patient Summary Article on optic atrophy:
Dysfunction of the optic nerve; may be congenital or acquired. If congenital, it is
usually hereditary. The milder form is autosomal dominant and has a gradual onset of
deterioration in childhood but little progression thereafter; the more severe form is
autosomal recessive and is present at birth or within 2 years; this form is accompanied by
nystagmus. Leber's Disease has an unclear mode of inheritance but is suspected of being
X-linked, since it only rarely occurs in women; optic neuropathy occurs more commonly in
20-30 year old males; some vision is retained but there are varying degrees of impairment.
The acquired type of optic atrophy may be due to vascular disturbances (occlusions of the
central retinal vein or artery or arteriosclerotic changes within the optic nerve itself),
may be secondary to degenerative retinal disease (e.g., papilledema or optic neuritis),
may be a result of pressure against the optic nerve, or may be related to metabolic
diseases (e.g., diabetes), trauma, glaucoma, or toxicity (to alcohol, tobacco, or other
poisons). Loss of vision is the only symptom. Pale optic disk and loss of pupillary
reaction are usually proportionate to the visual loss.
