early-onset cerebellar ataxia with retained tendon reflexes
Findings
No curated finding names early-onset cerebellar ataxia with retained tendon reflexes yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Early onset cerebellar ataxia with retained reflexes (EOCARR) or Harding ataxia is a cerebellar ataxia characterized by the progressive association of a cerebellar and pyramidal syndrome with progressive cerebellar ataxia, brisk tendon reflexes, and sometimes profound sensory loss.
Definition from the Mondo Disease Ontology (MONDO:0008938), read 2026-09-29. CC BY 4.0.
Features
20 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.
- Progressive cerebellar ataxiaHPOHP:0002073
- Very frequent (80% to 99% of cases)
- Abnormal pyramidal signHPOHP:0007256
- Frequent (30% to 79% of cases)
- DysarthriaHPOHP:0001260
- Frequent (30% to 79% of cases)
- DysphagiaHPOHP:0002015
- Frequent (30% to 79% of cases)
- Hyperactive patellar reflexHPOHP:0007083
- Frequent (30% to 79% of cases)
- Jerky ocular pursuit movementsHPOHP:0008003
- Frequent (30% to 79% of cases)
- Lower limb hypertonia
Show the remaining 8
- Cognitive impairmentHPOHP:0100543
- Occasional (5% to 29% of cases)
- Decreased/absent ankle reflexesHPOHP:0200101
- Occasional (5% to 29% of cases)
- Generalized amyotrophyHPOHP:0003700
- Occasional (5% to 29% of cases)
- Impaired visuospatial constructive cognitionHPOHP:0010794
- Occasional (5% to 29% of cases)
- Lower limb muscle weaknessHPOHP:0007340
- Occasional (5% to 29% of cases)
- Lower limb spasticityHPOHP:0002061
- Occasional (5% to 29% of cases)
Where it sits
Other names
3 names
Resolves to: early-onset cerebellar ataxia with retained tendon reflexes
- Also called
- EOCAEOCARRHarding ataxia