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Start free with EleplanChoreoacanthocytosis
ORPHA:2388Disease
Also called ChAc · Chorea-acanthocytosis · Levine-Critchley syndrome
What it is
Chorea-acanthocytosis (ChAc) is a form of neuroacanthocytosis and is characterized clinically by a Huntington disease-like phenotype with progressive neurological symptoms including movement disorders, psychiatric manifestations and cognitive disturbances.
Key facts
- Age of onset
- Adult
- Inheritance
- Autosomal recessive
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- <1 / 1 000 000Neuroacanthocytosis
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Common30–79%
25- Abnormal erythrocyte enzyme activity
- Absent Achilles reflex
- Acanthocytosis
- Atypical behavior
- Caudate atrophy
- Decreased amplitude of sensory action potentials
- Distal amyotrophy
- Distal muscle weakness
- Elevated circulating creatine kinase concentration
- EMG: neuropathic changes
- Falls
- Impaired vibratory sensation
- Laryngeal dystonia
- Lateral ventricle dilatation
- Limb dystonia
- Motor tics
- Muscle fiber atrophy
- Myopathy
- Parkinsonism
- Peripheral axonal neuropathy
- Phonic tics
- Poor motor coordination
- Reduced tendon reflexes
- Seizure
- Square-wave jerks
Sometimes5–29%
52- Abnormal hippocampus morphology
- Abnormality of eye movement
- Abnormal putamen morphology
- Aggressive behavior
- Anxiety
- Apathy
- Arthritis
- Bilateral tonic-clonic seizure
and 44 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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