hereditary progressive chorea without dementia
MONDO:0021011Mondo
Findings
No curated finding names hereditary progressive chorea without dementia yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Infantile onset · Childhood onset
HPO, annotations 2026-09-02
Features
4 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- ChoreaHPOHP:0002072
- 4 of 4 reported patients
- Motor delayHPOHP:0001270
- 4 of 4 reported patients
- Frequent fallsHPOHP:0002359
- 1 of 4 reported patients
- DementiaHPOHP:0000726
- 0 of 4 reported patients
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- NKX2-1HGNC:11825
- Strong · Genomics England PanelApp · Autosomal dominant · 2021
Where it sits
Other names
3 names
Resolves to: hereditary progressive chorea without dementia
- Also called
- BHCchorea, benign hereditarychorea, hereditary benign