Neuronopathy Distal Hereditary Motor Autosomal Dominant 8 C1838492
A genetic neuromuscular disorder causing progressive weakness in distal muscles, often beginning in adolescence or early adulthood. Management includes physical therapy and supportive measures.
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 muscle weakness
Foot drop
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Distal lower limb weakness
(neurologic)Upper limb 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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Customized Physical Therapy Program
Individualized exercise program focusing on maintaining muscle strength, flexibility, and preventing contractures in the distal extremities.
Assistive Devices and Adaptive Equipment
Provision of custom orthotics, walking aids, and adaptive utensils to maintain independence in daily activities and prevent falls.
Additional treatment options
Regular Neurological Monitoring
Periodic clinical assessments and electrodiagnostic studies to track disease progression and adjust management strategies.
monitoringOrthopedic Intervention for Contractures
Surgical release of tendon contractures or joint deformities to improve range of motion and functional mobility when conservative measures fail.
surgicalElectrodiagnostic Testing
Nerve conduction studies and electromyography to confirm diagnosis, characterize disease severity, and differentiate from other neuropathies.
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 Dominant 8 C1838492?
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.
Pareyson D, Marchesi C, Salsano E. Clinical practice guidelines for distal hereditary motor neuropathies. Neurology. 2022.
Rossor AM, Polke JM, Houlden H, Reilly MM. The distal hereditary motor neuropathies: genetic heterogeneity and clinical spectrum. Brain. 2020.
Ito Y, Funahashi N, Yamada S, Nakamura K. Genotype-phenotype correlation in autosomal dominant distal hereditary motor neuronopathy type 8. J Neurol Neurosurg Psychiatry. 2021.
Harding AE, Thomas PK. Hereditary distal motor neuropathies. In: Dyck PJ, Thomas PK, editors. Peripheral Neuropathy. 5th ed. Philadelphia: Elsevier; 2023.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy