hemidystonia-hemiatrophy syndrome
MONDO:0017649Mondo
Findings
No curated finding names hemidystonia-hemiatrophy syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Hemidystonia-hemiatrophy (HD-HA) is a rare dystonia, usually caused by a static cerebral injury occurring at birth or during infancy, that is characterized by a combination of hemidystonia (HD), involving one half of the body, and hemiatrophy (HA) on the same side as the HD.
Definition from the Mondo Disease Ontology (MONDO:0017649), read 2026-09-29. CC BY 4.0.
Features
15 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.
- Abnormal basal ganglia MRI signal intensityHPOHP:0012751
- Very frequent (80% to 99% of cases)
- Abnormal periventricular white matter morphologyHPOHP:0002518
- Very frequent (80% to 99% of cases)
- DystoniaHPOHP:0001332
- Very frequent (80% to 99% of cases)
- HemiparesisHPOHP:0001269
- Very frequent (80% to 99% of cases)
- Abnormal deliveryHPOHP:0001787
- Frequent (30% to 79% of cases)
- HemiatrophyHPOHP:0100556
- Frequent (30% to 79% of cases)
- Limb dystoniaHPOHP:0002451
- Frequent (30% to 79% of cases)
- Abnormal paranasal sinus morphologyHPOHP:0000245
- Occasional (5% to 29% of cases)
- Abnormal pyramidal signHPOHP:0007256
- Occasional (5% to 29% of cases)
- Advanced pneumatization of cranial sinusesHPOHP:0010540
- Occasional (5% to 29% of cases)
- Babinski signHPOHP:0003487
- Occasional (5% to 29% of cases)
- Dense calvariaHPOHP:0000250
- Occasional (5% to 29% of cases)
Show the remaining 3
- SeizureHPOHP:0001250
- Occasional (5% to 29% of cases)
- Motor delayHPOHP:0001270
- Very rare (1% to 4% of cases)
- Rhizomelic leg shorteningHPOHP:0012106
- Very rare (1% to 4% of cases)
Where it sits
- A kind of
Other names
1 name
Resolves to: hemidystonia-hemiatrophy syndrome
- Also called
- HD-HA syndrome