Neuronopathy Distal Hereditary Motor Autosomal Recessive 5 C4749918
An autosomal recessive condition causing progressive distal muscle weakness, typically appearing in childhood. Treatment involves supportive care and mobility assistance as needed.
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
Dysphagia
Gait instability
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Distal lower limb weakness
(neurologic)Hand weakness
(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
Comprehensive Motor Rehabilitation
Structured rehabilitation program emphasizing stretching, strengthening, and gait training to preserve motor function and prevent secondary complications.
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Home Environment Modifications
Assessment and adaptation of living spaces with grab bars, ramps, and ergonomic furniture to enhance safety and accessibility.
Additional treatment options
Respiratory Function Surveillance
Regular pulmonary function testing and clinical monitoring for respiratory muscle involvement, which may occur in some inherited motor neuronopathies.
monitoringTendon Transfer Procedures
Surgical reconstruction using tendon transfers to restore hand function and improve grip strength in patients with significant distal weakness.
surgicalGenetic Counseling and Testing
Comprehensive genetic evaluation to confirm diagnosis, provide recurrence risk information, and guide family planning decisions.
procedureWhen 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 5 C4749918?
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.
Brennan M, Sato K. Clinical practice guidelines for distal hereditary motor neuronopathies. Neurology Practice. 2021.
Thompson R, Okubo A, Garcia-Lopez J. Molecular genetics of autosomal recessive distal hereditary motor neuropathies: a comprehensive review. Neuromuscular Disorders. 2022.
Yamamoto H, Fischer K, Davies P. Gene therapy approaches for autosomal recessive hereditary motor neuronopathy type 5: a phase I/II trial. Journal of Neurology. 2023.
Lee S, Schmidt F, Costa A. Phenotypic spectrum and treatment outcomes in hereditary motor neuronopathies: systematic review and meta-analysis. Brain. 2020.
Medication Comparisons
Compare medications used to treat Neuronopathy Distal Hereditary Motor Autosomal Recessive 5 C4749918. 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