ICD-11 code : 8A01.0;
Preferred Label : Benign hereditary chorea;
ICD-11 definition : 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.;
ICD-11 synonym : Benign familial chorea;
Origin ID : 829618737;
UMLS CUI : C0008489;
Currated CISMeF NLP mapping
ICD-10 Mapping
Semantic type(s)
UMLS correspondences (same concept)
- CHOREA [WHO-ART Included Term]
- Chorea [MedDRA Preferred Term]
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.