Orphanet: Spinocerebellar ataxia type 7

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Spinocerebellar ataxia type 7

Disease definition

An autosomal dominant cerebellar ataxia type II that is characterized by progressive ataxia, motor system abnormalities, dysarthria, dysphagia and retinal degeneration leading to progressive blindness.


Classification level: Disorder
  • Synonym(s):
    • Ataxia with pigmentary retinopathy
    • Cerebellar syndrome-pigmentary maculopathy syndrome
    • SCA7
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Childhood, Adolescent, Adult, Elderly
  • ICD-10: G11.8
  • OMIM: 164500
  • UMLS: C0752125
  • MeSH: -
  • GARD: 4955
  • MedDRA: -

Detailed information

Article for general public


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