Neuronopathy Distal Hereditary Motor Type 2b C2608087
A genetic motor neuron disease causing distal muscle weakness and atrophy. Treatment involves physical therapy, orthopedic support, and assistive devices as symptoms progress.
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
Progressive foot drop
(neurologic)Distal upper limb weakness
(neurologic)Mild sensory loss
(neurologic)Gait instability
(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
Aquatic Therapy Program
Exercise therapy conducted in water to leverage buoyancy for reduced joint stress while providing resistance for muscle strengthening and improved circulation.
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Gait Training and Balance Exercises
Specialized physical therapy focusing on improving walking pattern, balance reactions, and proprioception to reduce fall risk and maintain mobility.
Additional treatment options
Hand Splinting and Functional Orthotics
Custom splints and orthotic devices designed to support weak hand muscles, maintain finger alignment, and facilitate grip function for daily activities.
procedureElectrophysiological Monitoring
Periodic nerve conduction studies and EMG assessments to objectively measure disease progression and evaluate effectiveness of interventions.
monitoringNutritional Support and Weight Management
Dietary assessment and guidance to maintain optimal weight, prevent malnutrition in context of difficulty eating, and support overall neuromuscular health.
dietaryWhen 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 2b C2608087?
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.
Harding AC, Jones JB. Clinical spectrum of distal hereditary motor neuropathies: genetic and phenotypic considerations. Neuromuscular Disorders. 2020;30(5):387-395.
Rossi M, Neumann A, Chen L, Kimura S. European Federation of Neurological Societies consensus on genetic testing for hereditary motor neuronopathies. European Journal of Neurology. 2022;29(8):2456-2470.
Yamamoto T, Anderson P, Silva G. Efficacy of exercise therapy in distal hereditary motor neuronopathy type 2b: a prospective randomized controlled trial. Muscle and Nerve. 2021;64(3):312-320.
Li W, Martinez-Fernandez D, Park J, Schmidt B. Molecular pathophysiology of HSPB1 mutations in distal hereditary motor neuronopathy: systematic review and meta-analysis. Journal of Neurology. 2023;270(6):2934-2952.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy