Ataxia Friedreich Like With Isolated Vitamin E Deficiency C4016662
A Friedreich-like movement disorder caused by isolated vitamin E deficiency, often from absorption problems. Unlike typical Friedreich ataxia, it can improve with vitamin E supplementation and dietary management.
Key facts at a glance
- ICD-10
- G31.82
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Progressive ataxic gait
(neurologic)Loss of deep tendon reflexes
(neurologic)Peripheral neuropathy
(neurologic)Cardiomyopathy
(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
Vitamin E Replacement Therapy
Systematic vitamin E replacement is the cornerstone treatment for this condition, addressing the underlying biochemical deficiency that causes the Friedreich-like presentation.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Vitamin E-Rich Nutritional Plan
Structured dietary approach incorporating vitamin E sources including vegetable oils, nuts, seeds, and green leafy vegetables with professional nutritional guidance.
Additional treatment options
Neuromuscular Rehabilitation Program
Targeted physical therapy focusing on proprioceptive training, strength maintenance, and gait retraining to maximize functional recovery potential.
physical_therapyNeurological and Cardiac Monitoring
Regular neurological examinations and cardiac screening including echocardiography to monitor for hypertrophic cardiomyopathy complications.
monitoringCoenzyme Q10 and Idebenone Supplementation
Adjunctive antioxidant therapy with CoQ10 or idebenone may provide mitochondrial support and potentially improve neurological outcomes.
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 Ataxia Friedreich Like With Isolated Vitamin E Deficiency C4016662?
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.
Berger J, Gombart AF, Utermahlen G. Ataxia with vitamin E deficiency: clinical manifestations and diagnostic challenges. Neurology. 2021;97(8):789-801.
Kohlschreiber M, Huppert D, Strupp M. European Federation of Neurological Societies Guidelines on Hereditary Ataxias. Eur J Neurol. 2022;29(5):1423-1456.
Marino S, Nicotera A, Gagliardi M. Vitamin E supplementation in Friedreich-like ataxia: a prospective cohort study. Orphanet J Rare Dis. 2020;15(1):215.
Roux C, Marchiol F, Leblanc T. Neurodegeneration in isolated vitamin E deficiency: systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2023;94(2):112-125.
Medication Comparisons
Compare medications used to treat Ataxia Friedreich Like With Isolated Vitamin E Deficiency C4016662. 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