Charcot Marie Tooth disease (CMT) is an inherited progressive disorder associated with damage to the peripheral nerves, which transmit movement and sensory signals between the brain and the rest of the body.

The initial and most common symptom of the disease is a weakening of the muscles. The uneven weakening of muscles results in joints being pulled on unequally, which can eventually shift bones into abnormal positions and result in a condition called scoliosis.

What is scoliosis?

Scoliosis is when the spine develops an abnormal sideways curvature. The severity of the curvature can vary from one patient to another and may become progressively worse if not monitored and treated. This often results in the patient leaning to one side, with the shoulder, hip, or ribs sticking out on one side.

Scoliosis can cause back pain in some patients and can make it more difficult to find clothes that fit well. Severe scoliosis can restrict the chest, reducing the space available to breathe and impairing lung and heart function.

Scoliosis develops in about one-third of CMT patients and is usually diagnosed around age 13. It is more common in CMT type 4.

How is scoliosis treated?

Scoliosis should be monitored closely for progression through physical examinations and X-rays to visualize the curvature of the spine. The severity of scoliosis can help determine the type of treatment needed.

Braces

Early or less severe scoliosis may be treated using a specialized back brace, although this is often not successful for scoliosis caused by CMT. A study published in the Journal of Bone and Joint Surgery reported that brace treatment worked in only 3 out of 16 CMT patients with scoliosis.

Braces are generally only used when the spine is still developing to encourage it to do so correctly. However, even though this can limit or stop the progression of scoliosis, it cannot reverse the abnormal curve that has already developed.

Surgery

If scoliosis progresses, surgery may be required to fix the abnormal curvature of the spine, especially if it prevents movement or causes pain.

Surgery could include a spinal fusion. This takes advantage of the body’s normal bone healing process to permanently join two or more vertebrae together. The procedure involves putting bone, or a synthetic bone-like material, in place between two vertebrae using metal screws or rods. Over time, the bone healing process fuses the structure into a single solid bone.

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Charcot-Marie-Tooth News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.