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Early-onset X-linked optic atrophy

ORPHA:98890· ICD-10 H47.2

Definition

Early-onset X-linked optic atrophy is a rare form of hereditary optic atrophy, seen in only 4 families to date, with an onset in early childhood, characterized by progressive loss of visual acuity, significant optic nerve pallor and occasionally additional neurological manifestations, with females being unaffected.

Prevalence
<1 / 1 000 000
Inheritance
X-linked recessive
Age of onset
Childhood