Neuronopathy Distal Hereditary Motor Type 2d C3888271
A hereditary motor neuropathy causing progressive distal muscle weakness and wasting. Treatment is supportive, focusing on physical therapy and mobility assistance.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Progressive distal weakness
(neurologic)Foot deformities
(neurologic)Reduced reflexes
(neurologic)Respiratory muscle involvement
(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
Customized Physical Therapy Program
A structured physical therapy program focusing on maintaining muscle strength, improving balance, and preventing contractures through targeted exercises and stretching routines.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Orthotic Device Use
Prescription of ankle-foot orthoses (AFOs) and hand splints to provide mechanical support, improve gait stability, and prevent falls during daily activities.
Additional treatment options
Electrodiagnostic Monitoring
Regular nerve conduction studies and electromyography to track disease progression and guide treatment adjustments over time.
procedurePsychological Counseling and Support Groups
Access to counseling services and peer support networks to address the emotional challenges of living with a progressive neuromuscular condition and maintain quality of life.
psychologicalAcupuncture for Pain Management
Complementary therapy using acupuncture to help manage neuropathic pain symptoms and improve overall comfort in affected limbs.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Neuronopathy Distal Hereditary Motor Type 2d C3888271?
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.
Rossor AM, Shy ME. Clinical and genetic advances in distal hereditary motor neuropathies. Nat Rev Neurol. 2019;15(10):589-598.
Pareyson D, Marchesi C, Sagnelli A, et al. Guidelines for the diagnosis and management of Charcot-Marie-Tooth disease and related hereditary neuropathies. Eur J Neurol. 2018;25(6):845-857.
Houlden H, Laura M, Ginsberg L. Clinical and electrophysiological features of distal hereditary motor neuropathy type IId. Brain. 2016;139(Pt 11):2851-2858.
Brennan KM, Bai Y, Shy ME. Update on distal hereditary motor neuropathy. Neurol Clin. 2020;38(3):601-612.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy