Rigid spine muscular dystrophy (RSMD) is a type of congenital muscular dystrophy characterized by weakness of the muscles of the torso and neck, which can affect the spine and cause breathing difficulties. Like all congenital dystrophies, the muscle weakness that marks RSMD is either present at birth or develops within the first few months of life.
There is currently no cure for RSMD, and management options focus on the relief of symptoms and improving quality of life.
Physiotherapy
Physiotherapy and occupational therapy can help improve muscle strength and reduce muscle loss. They may also help to preserve flexibility and range of motion.
Adaptive devices
Muscle weakness in the back often leads to scoliosis (abnormal sideways curvature of the spine). Specific braces can help support the spine.
Surgery
RSMD patients experience a limited flexion of the spine (bending forward) and scoliosis. Surgical correction can be used to straighten the spine.
Respiratory care
Because RSMD also affects respiratory muscles, patients may need help with breathing, especially at night. Non-invasive ventilation, such as a BiPAP device, facilitates breathing by providing higher pressure during inhalation.
Last updated: August 26, 2019
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