Corticosteroid Treatment Impact on Spinal Deformity in Duchenne Muscular Dystrophy

Figure 1

Duchenne muscular dystrophy: natural history of a progressive disease with a progressive scoliosis. A boy at 10 (a), 12 (b), and 16 years of age ((c), (d)). This boy, born in the mid-sixties, presented at 5 years of age with a 2-year story of progressive difficulty in climbing stairs and frequent falls. He was never able to run. CK was markedly elevated (more than 100 times normal). Muscle biopsy showed marked necrosis and proliferation of connective tissue. Loss of ambulation occurred at the age of 8 years and 6 months. At 10 years ((a), (e)), he had a minimal spinal curvature of 10°. He was immediately fitted with a spinal brace, which he wore during daytime. In spite of this treatment, the curvature progressed to 58° at age 12 ((b), (f)). At age 14 years and 6 months, the scoliosis measured 91°. At age 16, he was no more able to sit unsupported because of the severe collapsing spinal “C” curve of 116° with marked pelvic tilt. At age 17 years, he had pneumonia and needed ventilatory support and was subsequently left with a permanent tracheostomy. He died at age 19 years. Family history was positive for an X-linked muscular dystrophy. The mother showed big calf and persistent mild elevation of CK. A maternal uncle had a progressive muscular dystrophy and died at 15 years.

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