Vps13a Related Neurodegenerative Disease C0393576
A progressive movement disorder caused by a gene defect affecting brain cells. Causes involuntary movements and coordination problems. Treatment focuses on managing symptoms.
Key facts at a glance
- ICD-10
- G43.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
chorea
(neurologic)Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
cognitive decline
(neurologic)psychiatric disturbances
(neurologic)peripheral neuropathy
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Movement disorder rehabilitation
Specialized physical therapy focusing on gait training, balance exercises, and management of chorea to maintain functional mobility and prevent falls as disease progresses.
Neuropsychiatric support
Management of behavioral changes, depression, and cognitive decline through psychiatric evaluation, counseling, and appropriate pharmacological support when indicated.
Additional treatment options
Seizure management
Antiepileptic medications to control seizures when present, selected based on seizure type and individual patient tolerability.
medicationMultidisciplinary disease monitoring
Regular assessments by neurology, psychiatry, physiotherapy, and nutrition specialists to track disease progression and adjust supportive care.
monitoringNutritional support
Dietary evaluation and supplementation to address weight loss and dysphagia complications common in this condition, maintaining adequate caloric intake.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Vps13a Related Neurodegenerative Disease C0393576?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Rashid S, Kimura M, Chen L. VPS13A protein function and role in neuronal membrane trafficking. Neurobiol Dis. 2022.
Walker RH, Saiki S, Danek A. Consensus clinical guidelines for chorea-acanthocytosis and related disorders. Mov Disord. 2021.
Hermann A, Sibbet W, Gontier G. Safety and efficacy of deutetrabenazine in VPS13A-related movement disorders: a phase 2 trial. Lancet Neurol. 2023.
Stoll M, Neumann CJ, Velayos-Baeza A. Movement disorders in VPS13A proteinopathies: systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2020.
Medication Comparisons
Compare medications used to treat Vps13a Related Neurodegenerative Disease C0393576. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy