Short answer · Medically reviewed summary · Last updated: 2026-05-08
Chorea-acanthocytosis (ChAc) is classified under ICD-10 code G23.8 (Other specified degenerative diseases of basal ganglia) and ICD-9 code 333.5 (Other choreas). These codes are used for medical billing and tracking the clinical progression of this rare, progressive neurodegenerative disorder. What is the clinical nature of Chorea-acanthocytosis? Chorea-acanthocytosis is a rare, multisystem disorder characterized by involuntary movements (chorea), oromandibular dystonia, and the presence of spiked red blood cells known as acanthocytes.
ICD10 code of Chorea-acanthocytosis ChAc and ICD9 code
ICD-10 and ICD-9 codes for Chorea-acanthocytosis ChAc, with classification details for clinicians, coders and patients.
Chorea-acanthocytosis (ChAc) is classified under ICD-10 code G23.8 (Other specified degenerative diseases of basal ganglia) and ICD-9 code 333.5 (Other choreas). These codes are used for medical billing and tracking the clinical progression of this rare, progressive neurodegenerative disorder.
What is the clinical nature of Chorea-acanthocytosis?
Chorea-acanthocytosis is a rare, multisystem disorder characterized by involuntary movements (chorea), oromandibular dystonia, and the presence of spiked red blood cells known as acanthocytes. As a specialist, I often see patients present between the ages of 20 and 40. Chorea-acanthocytosis profoundly impacts motor function, cognitive processing, and personality, often requiring a multidisciplinary care team to manage the complex, progressive symptoms.
How is Chorea-acanthocytosis inherited?
Chorea-acanthocytosis is an autosomal recessive condition caused by mutations in the VPS13A gene on chromosome 9q21. Because it is recessive, an individual must inherit two copies of the mutated gene—one from each parent—to manifest the disease. Genetic counseling is essential for families, as siblings of an affected individual have a 25% chance of inheriting the condition.
What are the primary diagnostic markers for Chorea-acanthocytosis?
Diagnosing Chorea-acanthocytosis involves a combination of clinical evaluation and laboratory testing. Key diagnostic features include:
- Peripheral blood smear: Identifying acanthocytes (spiked red blood cells), though these are not present in every blood sample and may require repeated testing.
- Molecular Genetic Testing: Identifying pathogenic variants in the VPS13A gene.
- Neurological Imaging: MRI often shows atrophy of the caudate nucleus.
- Serum Creatine Kinase: Frequently elevated in patients with Chorea-acanthocytosis.
How does the DiseaseMaps community support patients?
Living with Chorea-acanthocytosis can feel isolating due to its rarity. Currently, 6 members of the DiseaseMaps.org community have shared their experiences, providing a space for peer support. Connecting with others facing the same challenges can provide emotional relief and practical insights into managing daily living with this rare neurodegenerative condition.
Next steps
- Consult a movement disorder specialist or neurologist experienced in neuroacanthocytosis syndromes.
- Undergo genetic counseling to understand the inheritance patterns within your family.
- Connect with the 6 members on DiseaseMaps.org to share coping strategies and community resources.
- Monitor for clinical trials through the NIH or specialized rare disease registries.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician.
References
- NIH Genetic and Rare Diseases (GARD) Information Center: Chorea-acanthocytosis.
- Orphanet: Chorea-acanthocytosis (ORPHA:139).
- OMIM (Online Mendelian Inheritance in Man): #200150 Chorea-acanthocytosis.
- GeneReviews: Chorea-Acanthocytosis.
