Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation

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Overview

Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation (also called CMT2 due to KIF5A or CMT2-KIF5A) is a rare inherited nerve disorder that affects the peripheral nerves — the nerves that connect your brain and spinal cord to your muscles and sensory organs. It belongs to the CMT type 2 group, which means the problem lies in the nerve fibers (axons) themselves rather than in the protective covering around them. The KIF5A gene provides instructions for making a protein called kinesin heavy chain, which acts like a tiny motor that transports important materials along nerve cells. When this gene is mutated, the transport system doesn't work properly, and nerve cells gradually lose their ability to function, especially the longest nerves that reach the hands and feet. People with this condition typically notice progressive weakness and wasting of muscles in the lower legs and feet, often leading to difficulty walking, foot deformities such as high arches or hammertoes, and reduced sensation. Over time, the hands and forearms may also become affected. Symptoms usually begin in childhood or adolescence but can vary. Some individuals may also experience stiffness or spasticity in the legs. The disease tends to worsen slowly over many years. There is currently no cure for this form of CMT. Treatment focuses on managing symptoms and maintaining function. Physical therapy, occupational therapy, orthotic devices such as ankle-foot braces, and sometimes surgery for foot deformities are the main approaches. Pain management may also be needed. Research into gene-based therapies and other disease-modifying treatments is ongoing but not yet available for routine clinical use.

Also known as:

Key symptoms:

Weakness in the feet and lower legsMuscle wasting in the lower legsHigh foot arches (pes cavus)HammertoesDifficulty walking or frequent trippingFoot drop (trouble lifting the front of the foot)Numbness or reduced sensation in the feet and handsWeakness in the hands and forearmsLoss of balanceLeg stiffness or spasticityDecreased reflexesTingling or burning sensations in the extremitiesDifficulty with fine motor tasks like buttoning clothesFatigue

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation.

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No actively recruiting trials found for Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation at this time.

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Search ClinicalTrials.gov ↗Join the Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation community →

No specialists are currently listed for Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How quickly is this condition likely to progress in my case or my child's case?,What physical therapy exercises are most helpful for maintaining strength and mobility?,Are there any medications or supplements I should avoid because they could worsen nerve damage?,Should other family members be tested for the KIF5A mutation?,What assistive devices or braces would be most helpful right now?,Are there any clinical trials or research studies I could participate in?,How often should I have follow-up appointments and nerve function testing?

Common questions about Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation

What is Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation?

Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation (also called CMT2 due to KIF5A or CMT2-KIF5A) is a rare inherited nerve disorder that affects the peripheral nerves — the nerves that connect your brain and spinal cord to your muscles and sensory organs. It belongs to the CMT type 2 group, which means the problem lies in the nerve fibers (axons) themselves rather than in the protective covering around them. The KIF5A gene provides instructions for making a protein called kinesin heavy chain, which acts like a tiny motor that transports important materials along nerve

How is Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation inherited?

Autosomal dominant Charcot-Marie-Tooth disease type 2 due to KIF5A mutation follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.