8A01.0

ICD-11 MMS 2026-01

Benign hereditary chorea

Description

Benign hereditary chorea should be considered in people with a relatively stable, nonprogressive chorea, in whom childhood onset and an autosomal dominant family history are present. Benign hereditary chorea can be associated with short stature and developmental delay. Larger deletions of causative gene, TITF-1, can cause a multisystem disorder with congenital hypothyroidism, hypotonia, and pulmonary problems.

Index terms

  • Benign hereditary chorea
  • Benign familial chorea

Exclusions from above levels

  • 6A06 – Stereotyped movement disorder
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8A01.1 – Secondary Chorea
  • 8A01.2 – Hemichorea or hemiballismus
  • 8A01.Y – Other specified choreiform disorders
  • 8A01.Z – Choreiform disorders, unspecified

Foundation entity

Benign hereditary chorea – http://id.who.int/icd/entity/829618737

Part of: 8A01: Choreiform disorders › 08: Diseases of the nervous system