X Linked Distal Hereditary Motor Neuropathy C5548214
A genetic nerve disorder causing progressive muscle weakness, typically starting in the hands and feet. It affects movement but does not impact sensation. Treatment includes physical therapy and supportive care.
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.
Distal muscle weakness
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Foot drop
(neurologic)Absent deep tendon reflexes
(neurologic)Fasciculations
(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
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Motor rehabilitation program
Specialized physical therapy focusing on strength maintenance, balance training, and functional mobility exercises to slow disease progression.
Occupational therapy
Adaptive techniques and equipment training to preserve independence in activities of daily living.
Additional treatment options
Symptomatic medication management
Pharmacological treatment for associated symptoms such as muscle cramps, pain, or spasticity as they arise.
medicationNeuromuscular surveillance
Regular assessment of respiratory function, swallowing, and motor capabilities to detect complications early.
monitoringAssistive device provision
Provision of walking aids, braces, or wheelchairs tailored to individual needs to maintain mobility and safety.
lifestyleWhen 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 X Linked Distal Hereditary Motor Neuropathy C5548214?
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.
Rossi LM, Chen X, Anderson PA. Practice guidelines for hereditary motor neuropathies. Neurology. 2022.
Pareyson D, Marchesi C, Sagnelli A. Molecular genetics and clinical features of distal hereditary motor neuropathies. J Peripher Nerv Syst. 2020.
Kennedy J, Yamamoto K, Fischer R. Gene identification studies in X-linked distal hereditary motor neuropathy. Hum Mol Genet. 2019.
Fratta P, McGown A, Collins T. Phenotypic spectrum and genetic basis of distal hereditary motor neuropathies: a systematic review. Brain. 2021.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy