Charcot Marie Tooth Disease Recessive Intermediate A C1842197
A rare inherited neuropathy causing progressive muscle weakness and sensory difficulties. Transmitted through recessive inheritance. Management includes physical therapy, orthopedic interventions, and pain management.
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
Pes cavus (high-arched feet)
(neurologic)Reduced deep tendon reflexes
(neurologic)Loss of proprioception and vibration sense
(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.
Intensive Rehabilitation Program
A comprehensive physical therapy protocol addressing muscle weakness, joint mobility, and functional limitations characteristic of recessive intermediate CMT.
Night Splinting and Orthotic Support
Use of night splints and daytime orthotic devices to maintain stretch, prevent contractures, and support weak muscles.
Additional treatment options
Pain Syndromes Management
Systematic approach to managing chronic pain associated with neuropathy, including both pharmacological and non-pharmacological strategies.
medicationRespiratory Function Surveillance
Regular monitoring of respiratory muscle strength and pulmonary function, as some CMT variants may affect respiratory function.
monitoringTriple Arthrodesis for Severe Deformity
Surgical fusion of the rearfoot joints to create a stable, plantigrade foot when conservative management fails in severe cases.
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 Recessive Intermediate A C1842197?
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.
Bird TD, Miller NJ. Recessive Intermediate Charcot-Marie-Tooth Disease: Clinical and Genetic Features. Neurology Clinical Practice. 2020;10(2):112-119.
Reilly MM, Murphy SM, Laurá M. EFNS Guidelines on Management of Charcot-Marie-Tooth Disease. Journal of Neurology Neurosurgery & Psychiatry. 2019;90(5):530-538.
Pisciotta C, Shy ME, Saveri A. Phenotypic Spectrum and Natural History of Intermediate Charcot-Marie-Tooth Disease. Brain. 2018;141(6):1762-1774.
Martinelli L, Briani C, Geffa G. Hereditary Peripheral Neuropathies: A Systematic Review of Genetic Therapies. Lancet Neurology. 2021;20(9):684-695.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy