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Spinal Muscular Atrophy

The spinal muscular atrophies (SMAs) are a group of inherited, degenerative motor neuron diseases that cause progressive weakness and atrophy of skeletal muscles. There are several types of SMA; classification is determined by the age of onset and severity of symptoms.

SMA is characterized by extensive loss of large motor neurons. Hypotonia of the extremities is a common initial finding. Progressive respiratory muscle weakness and bulbar dysfunction are also common and can lead to scoliosis, dysphagia, and feeding problems. Immobility and an ineffective cough can lead to the retention of pulmonary secretions and consequent respiratory infection.

Airway clearance indications that may be present in spinal muscular atrophy include:

• Abnormalities in oral-motor function and coordination resulting in swallowing difficulties, leading to choking and aspiration.
• Inadequate control of respiratory muscles due to denervation or hypotonia, impairing the normal cough reflex and interfering with the ability to clear aspirated material and pulmonary secretions from the airways.
• Retained secretions provide a cultural medium for bacterial pathogens. Consequent infections produce still more mucus, initiating a vicious cycle of mucus obstruction, recurrent respiratory infection, and progressive pulmonary compromise.
• Static or diminished lung volumes and vital capacities can occur as the result of restrictive lung disease. Where respiratory muscles are weak and the spine and thorax deformed, the ability to take a big breath, to generate expiratory force, and to cough effectively can be affected.

Aggressive bronchial hygiene, including daily Airway Clearance Therapy, is an essential component in the management of patients with pulmonary complications from spinal muscular atrophy. Conscientious attention to daily secretion clearance may prevent or minimize the pulmonary complications associated with this disorder.

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Sources

Brown RH. The motor neuron diseases. In: Fauci, Braunwald, eds. Harrison's Principles of Internal Medicine. New York: McGraw Hill; 2368-2371. National Institute of Neurological Disorders and Stroke. Spinal muscular atrophy. Available at: http://www.ninds.nih.gov/patients/disorder/sma/spinal . Accessed March, 2000.

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