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ABri amyloidosis

Disease definition

A rare, neurodegenerative disease characterized by progressive cognitive impairment, spastic tetraparesis, and cerebellar ataxia resulting from amyloid deposits in the brain. Spasticity with increased deep tendon reflexes and tone are early symptoms, muscular rigidity evolves later. Progressive mental deterioration usually starts with apathy and impaired memory with progression to complete disorientation.

ORPHA:97345

Classification level: Subtype of disorder
  • Synonym(s):
    • Familial dementia, British type
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: E85.4+  I68.0*
  • OMIM: 176500
  • UMLS: C1867773
  • MeSH: C538208
  • GARD: 8344
  • MedDRA: -

Detailed information

Article for general public

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