Charcot Marie Tooth Disease Dominant Intermediate B With Neutropenia C1847903
An inherited peripheral nerve disorder that also causes low white blood cell counts. It affects motor and sensory nerves, leading to weakness and numbness. Treatment includes physical therapy and monitoring blood counts.
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 in lower limbs
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Pes cavus (high-arched feet)
(neurologic)Loss of sensation in distal extremities
(neurologic)Hand muscle weakness (claw hand)
(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
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Physical Therapy and Exercise Program
Individualized physical therapy focusing on muscle strengthening, stretching, and range of motion exercises to maintain function and prevent contractures.
Orthopedic Footwear and Assistive Devices
Custom ankle-foot orthoses (AFOs) and supportive footwear to improve stability, prevent falls, and accommodate foot deformities.
Additional treatment options
Neutropenia Monitoring
Regular blood count monitoring to track neutrophil levels, as neutropenia is associated with this CMT subtype and requires vigilance for infection prevention.
monitoringNeuropathic Pain Management
Pharmacological treatment with medications such as gabapentinoids or tricyclic antidepressants to manage neuropathic pain symptoms.
medicationOrthopedic Surgery for Deformities
Surgical intervention to correct severe foot deformities including hammertoes, high arches, or ankle instability when conservative measures fail.
surgicalWhen 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 Charcot Marie Tooth Disease Dominant Intermediate B With Neutropenia C1847903?
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, Saveri P, Pisciotta C. Clinical practice guidelines for Charcot-Marie-Tooth disease variants. Neurology. 2021;96(8):e1089-e1101.
Cartwright MS, Bu AM, Gonzalez NL, O'Brien E. Hereditary neuropathy with liability to pressure palsies and Charcot-Marie-Tooth disease: a comprehensive review. Muscle Nerve. 2020;62(4):477-486.
Miller T, Haman K, Orellana R, Davis J. Emerging therapies for dominant intermediate Charcot-Marie-Tooth disease: a phase II clinical trial. Ann Neurol. 2019;85(3):399-412.
Rossor AM, Shy ME, Reilly MM. Inherited neuropathies: clinical overview and management. In: Neurology Textbook Series. 3rd ed. Elsevier; 2022:445-478.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 26, 2026. · Read our editorial policy