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Benign hereditary chorea

Disease definition

A rare, genetic, movement disorder characterized by early-onset, very slowly progressive choreiform movements that may involve variable parts of the body, typically aggravated by stress or anxiety, in various members of a family. Additional variable manifestations include hypotonia, often resulting in psychomotor delay (including gait disturbances) and dysarthria, as well as myoclonus, dystonia, behavioral symptoms (ADHD, obsessive-compulsive disorder), learning difficulties (particularly in writing) and spasticity with hyperreflexia and/or flexor/extensor plantar reflexes.

ORPHA:1429

Classification level: Disorder
  • Synonym(s):
    • BHC
    • Benign familial chorea
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Childhood
  • ICD-10: G25.5
  • OMIM: 118700  215450
  • UMLS: C0393584  C1859098
  • MeSH: -
  • GARD: 1305
  • MedDRA: -

Detailed information

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