Disease Directory Neuroacanthocytosis
Neurological

Neuroacanthocytosis

Also known as: Chorea-acanthocytosis, McLeod syndrome, VPS13A deficiency, XK protein deficiency

Prevalence

1-9 per 1,000,000 (Orphanet)

Onset

Adolescent, Adult

Type

Autosomal recessive (chorea-acanthocytosis) or X-linked (McLeod syndrome)

Gene

VPS13A (chorea-acanthocytosis), XK (McLeod syndrome)

About Neuroacanthocytosis

Neuroacanthocytosis syndromes are a group of rare genetic disorders characterized by progressive neurodegeneration with movement disorder and acanthocytes (spiked red blood cells) on peripheral blood smear. The two main forms are chorea-acanthocytosis (VPS13A mutations, autosomal recessive) and McLeod syndrome (XK mutations, X-linked). Features include orofacial dyskinesia, self-mutilation, chorea, parkinsonism, cognitive decline, epilepsy, cardiomyopathy, and elevated serum creatine kinase.

Common Clinical Features

Orofacial dyskinesia Chorea Self-mutilating lip and tongue biting Acanthocytosis on blood smear Elevated serum creatine kinase Cognitive decline Seizures

Clinical Trial Eligibility Tips

What to know before applying to Neuroacanthocytosis trials.

Peripheral blood smear confirming acanthocytes (>3% in context of clinical features) is a required diagnostic marker

Specify neuroacanthocytosis form — VPS13A (chorea-acanthocytosis) versus XK (McLeod syndrome) have distinct genetics and trial eligibility

For McLeod syndrome: Kell antigen blood group phenotyping is a critical safety marker — blood transfusion history must be documented

Creatine kinase, liver enzymes, and cardiac evaluation (ECG, echocardiogram) are required baseline safety and eligibility assessments

Patient Resources

Patient Organization

Advocacy for Neuroacanthocytosis Patients

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Natural History Registry

Neuroacanthocytosis Patient Registry

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Orphanet

European reference resource for rare diseases (ORPHA:2388)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Neuroacanthocytosis trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Neuroacanthocytosis. Updated daily.

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