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Spastic ataxia-corneal dystrophy syndrome

ORPHA:2572· ICD-10 G11.8

Definition

A rare, hereditary ataxia disorder characterized by the presence of spastic ataxia in association with bilateral congenital cataract, macular corneal dystrophy (stromal with deposition of mucoid material) and nonaxial myopia. Patients present normal intellectual development. There have been no further descriptions in the literature since 1986.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Childhood