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Mohr-Tranebjaerg syndrome

Disease definition

Mohr-Tranebjaerg syndrome (MTS) is an X-linked recessive neurodegenerative syndrome 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.

ORPHA:52368

  • Synonym(s):
    • DDON syndrome
    • Deafness-dystonia-optic neuronopathy syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: X-linked recessive 
  • Age of onset: Childhood
  • ICD-10: G31.8
  • OMIM: 304700
  • UMLS: C0796074
  • MeSH: C535808
  • GARD: 8331
  • MedDRA: -

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