Spinal muscle atrophy (SMA) is a heritable disease characterized by the loss of specialized nerve cells called motor neurons. Such loss leads to progressive muscle weakness, including in areas around the spine, causing scoliosis in many patients.
What is scoliosis?
Scoliosis is an abnormal and progressive curvature of the spine caused by weakness in muscles of the back that support the spine’s position. It generally refers to a sideways curve of the spine; when viewed from behind, the spine forms an “S” shape. However, patients may hear other terms used to describe the spine’s shape. For example, lordosis refers to an excessive inward bend of the spine, and kyphosis refers to an outward curve, sometimes called hunchback.
Scoliosis is the most common symptom that SMA patients may encounter, and its severity is linked to SMA type. Patients with type 1 or type 2 SMA are likely to develop scoliosis, as are about 50 percent of patients with type 3, especially those who cannot walk.
Scoliosis can cause breathing difficulties, because the lungs have less room to expand and allow for deep breathing. The condition also reduces patients’ mobility, which causes joints to stiffen or become displaced, especially those placed under stress by the bending spine, such as the hip joints. Patients who are unable to stand straight often must support their weight with their arms, further limiting their mobility.
Scoliosis usually develops from an early age in people with SMA, and is often diagnosed around ages 4 to 6. It can become progressively worse even after patients have reached adulthood and finished growing.
Scoliosis is diagnosed by a visual examination and X-rays of the spine.
Scoliosis can be difficult to treat in young children because they are still growing. It is important that treatments do not limit growth.
Chiropractic treatments, muscle stimulation, and dietary supplements have been shown to be ineffective in treating scoliosis. Physical therapy, or physiotherapy, may help in the early stages of scoliosis to slow its progression by strengthening key muscles and relaxing the joints.
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