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Ataxia-photosensitivity-short stature syndrome

ORPHA:1184· ICD-10 Q87.8

Definition

A rare, genetic, multiple congenital anomalies/dysmorphic syndrome characterized by cerebellar-like ataxia, photosensitivity (mainly of the face and trunk), short stature and intellectual disability. Additonal features include clinodactyly, single palmar transverse crease, high-arched palate, pseudohypertrophy of the calves and aortic valve lesions. There have been no further descriptions in the literature since 1983.

Prevalence
<1 / 1 000 000
Inheritance
Unknown
Age of onset
Childhood, Infancy