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The authors studied the evolution of spinal and respiratory involvements in 88 patients with prolonged spinal muscular atrophy. Patients were divided into 3 groups according to their ambulatory capabilities: never acquired, lost or retained. Evaluation at the time of the first examination gives an idea of the spontaneous evolution. It shows the high incidence (91%) and severity of scoliosis, and the variable severity of the respiratory impairment from one group to another. The follow-up period ranged from 6 to 9 years. Ten patients died. At the end of the follow-up period a new spinal and respiratory evaluation was performed. Results suggest the efficacy of early treatment of spinal impairment and emphasize the need for assisted ventilation as soon as the disease is diagnosed.
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