Neuronopathy Distal Hereditary Motor Autosomal Recessive 8 C5394466
An inherited motor neuron disorder causing progressive muscle weakness in the hands and feet. Transmitted through autosomal recessive inheritance and requires supportive management to maintain function.
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.
Warning signs - seek immediate care
Respiratory insufficiency
Difficulty climbing stairs
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Distal lower limb weakness
(neurologic)Fine motor impairment in hands
(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
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Neuroprotective Exercise Program
Specially designed exercise regimen combining aerobic conditioning with resistance training to support motor neuron health and overall function.
Occupational Therapy Adaptation
Skills training and environmental adaptations to maximize independence in self-care, work, and leisure activities despite motor limitations.
Additional treatment options
Functional Outcome Tracking
Systematic documentation of motor abilities, functional milestones, and quality of life measures to guide treatment decisions.
monitoringCorrective Osteotomy
Surgical bone realignment procedures to correct skeletal deformities resulting from muscle imbalance and preserve gait mechanics.
surgicalAquatic Therapy
Supervised water-based exercise program utilizing buoyancy to reduce joint stress while enabling resistance training and improved mobility.
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 Autosomal Recessive 8 C5394466?
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. Distal hereditary motor neuropathies: a comprehensive review. Neuromuscular Disorders. 2020;30(4):283-292.
European Federation of Neurological Societies. EFNS guidelines on the diagnosis and management of hereditary motor neuropathies. European Journal of Neurology. 2018;25(5):699-712.
Bansagi B, Griffin H, Miller J, et al. Genetic heterogeneity in autosomal recessive distal hereditary motor neuropathies. Brain. 2017;140(8):2131-2142.
Feng Y, Chen Q, Liu L, et al. Phenotypic spectrum and genetic basis of distal hereditary motor neuropathy: a meta-analysis. Journal of Neurology. 2022;269(6):3154-3170.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy