childhood-onset benign chorea with striatal involvement
MONDO:0044332Mondo
Findings
No curated finding names childhood-onset benign chorea with striatal involvement yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Features
5 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
- Very frequent (80% to 99% of cases)
- Abnormal corpus striatum morphologyHPOHP:0010994
- Frequent (30% to 79% of cases)
- Striatal T2 hyperintensityHPOHP:0031206
- Frequent (30% to 79% of cases)
- AnxietyHPOHP:0000739
- Occasional (5% to 29% of cases)
- Parkinsonism with favorable response to dopaminergic medicationHPOHP:0002548
- Occasional (5% to 29% of cases)
Reported absent (2)
- Delayed gross motor developmentHPOHP:0002194
- DementiaHPOHP:0000726
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.
- PDE10AHGNC:8772
- Supportive · Orphanet · Autosomal dominant · 2021