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Mohr-Tranebjaerg syndrome

ORPHA:52368· ICD-10 E88.8

Definition

A rare X-linked syndromic intellectual disability characterized by clinical manifestations commencing with early childhood onset hearing loss, followed by adolescent onset progressive dystonia or ataxia, visual impairment from early adulthood onwards and dementia from the 4th decade onwards.

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