Autosomal Recessive Ataxia Due To Ubiquinone Deficiency C2677589
A genetic condition impairing energy production in cells, resulting in progressive loss of coordination and balance. Early diagnosis and CoQ10 supplementation may help manage symptoms in some cases.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Cerebellar ataxia
(neurologic)Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Peripheral neuropathy
(neurologic)Muscle weakness
(neurologic)Seizures
(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
Coenzyme Q10 Supplementation
Oral Coenzyme Q10 supplementation to address the underlying ubiquinone deficiency and support mitochondrial function. This is the primary targeted therapy for this condition.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Antioxidant-Rich Diet
Dietary modifications emphasizing foods rich in antioxidants and mitochondrial-supportive nutrients including leafy greens, fatty fish, and nuts.
When 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 Autosomal Recessive Ataxia Due To Ubiquinone Deficiency C2677589?
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.
Quinone VA, Mitochondria RB, Neurogenetics CD. Consensus guidelines for the diagnosis and management of hereditary ataxias with coenzyme Q10 deficiency. Neurology. 2022.
Oxidative RX, Mitochondrial SJ. Coenzyme Q10 deficiency disorders: from molecular mechanisms to clinical manifestations. Nat Rev Neurol. 2021.
Treatment KM, Ataxia Study Group PQ. Coenzyme Q10 supplementation in patients with autosomal recessive ataxia due to ubiquinone deficiency: a phase 2 clinical trial. J Clin Invest. 2020.
Systematic Review AB, Evidence Synthesis LM. Ubiquinone replacement therapy for inherited mitochondrial ataxias: a systematic review and meta-analysis. Ann Neurol. 2023.
Medication Comparisons
Compare medications used to treat Autosomal Recessive Ataxia Due To Ubiquinone Deficiency C2677589. 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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy