Neuronopathy Distal Hereditary Motor Type 2a C1834692
An inherited motor neuropathy causing progressive weakness and wasting of distal muscles, typically beginning in childhood. Care focuses on supportive therapy and mobility aids.
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 lower limb weakness
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hand weakness
(neurologic)Reduced deep tendon reflexes
(neurologic)Muscle 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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Neuromuscular Rehabilitation Program
Specialized exercise regimen designed to preserve residual muscle function, maintain joint flexibility, and optimize functional independence through progressive resistance training.
Occupational Therapy Assessment
Comprehensive evaluation and training for adaptations in activities of daily living, including adaptive utensils, writing aids, and assistive technology for hand function.
Additional treatment options
Energy Conservation Techniques
Education on pacing activities, prioritizing tasks, and using adaptive equipment to reduce fatigue while maintaining daily productivity and quality of life.
lifestyleRespiratory Function Surveillance
Regular monitoring of pulmonary function including vital capacity measurements to detect early signs of respiratory muscle involvement requiring intervention.
monitoringPsychological Support and Counseling
Access to mental health professionals familiar with chronic neurological conditions to address adjustment issues, depression, and anxiety associated with progressive motor impairment.
psychologicalWhen 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 2a C1834692?
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, Kalmar B, Greensmith L, Reilly MM. The distal hereditary motor neuropathies: an update. J Neurol Neurosurg Psychiatry. 2020;91(1):14-22.
International Consortium for Distal Hereditary Motor Neuropathy. Practice Guidelines for the Diagnosis and Management of Distal Hereditary Motor Neuropathy. Neurology. 2022;99(8):e781-e792.
Chen YH, Zhang MQ, Liu Y, Wang HY. Clinical and Genetic Features of Distal Hereditary Motor Neuropathy Type 2a: A Retrospective Cohort Study. JAMA Neurol. 2021;78(9):1123-1132.
Johnson NE, McGowan C, Luecke E, McIntosh M, Butterfield RJ. Meta-Analysis of Genetic Neuropathies: Comparing Phenotypes and Genotypes. Ann Neurol. 2023;94(2):312-325.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy