Orphanet: ADan amyloidosis

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ADan amyloidosis

Disease definition

A rare, neurodegenerative disease characterized by progressive cataracts, hearing loss, cerebellar ataxia, paranoid psychosis and dementia. Neuropathological features are diffuse atrophy of all parts of the brain, chronic diffuse encephalopathy and the presence of extremely thin and almost completely demyelinated cranial nerves.


Classification level: Subtype of disorder
  • Synonym(s):
    • Familial dementia, Danish type
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: -
  • ICD-10: E85.4+  I68.0*
  • OMIM: 117300
  • UMLS: C1861735
  • MeSH: C538209
  • GARD: 9169
  • MedDRA: -

Detailed information

Article for general public

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