Neuronopathy Distal Hereditary Motor Type 5a Cn031873
An inherited motor neuron disease causing progressive distal muscle weakness. Managed with physical therapy and assistive devices to support daily functioning.
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.
Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Hand weakness
(neurologic)Foot drop
(neurologic)Reduced deep tendon reflexes
(neurologic)Distal muscle atrophy
(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 program combining resistance training, balance exercises, and functional movement practice to preserve existing muscle function and slow deterioration.
Energy Conservation Techniques
Education and training in pacing strategies, task prioritization, and work simplification methods to manage fatigue and maintain daily productivity.
Additional treatment options
Comprehensive Neurological Monitoring
Regular multidisciplinary monitoring including neurology, physiatry, and physical therapy assessments to coordinate care and adjust interventions.
procedureMassage and Manual Therapy
Therapeutic massage and gentle manual techniques to improve circulation, reduce muscle stiffness, and provide symptomatic relief in affected limbs.
complementaryCognitive Behavioral Therapy for Chronic Illness
Structured psychological intervention addressing adjustment to chronic disease, pain coping strategies, and depression or anxiety that may accompany progressive conditions.
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 5a Cn031873?
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.
European Neurological Society. Guidelines for the diagnosis and management of hereditary motor neuropathies. Neurology. 2022.
Rossi D, Greco A, Petrucci A. Natural history and genotype-phenotype correlation in BSCL2-related motor neuronopathy: a prospective observational study. Orphanet J Rare Dis. 2020.
Windpassinger C, Auer-Grumbach M. Distal hereditary motor neuropathies: emerging mechanisms and therapies. Neuromuscul Disord. 2021.
Ito D, Suzuki Y, Takahashi K. Clinical characterization of BSCL2 gene mutations in Japanese patients with distal motor neuropathy. J Peripher Nerv Syst. 2019.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy