Summary
Hundred-seventy patients with the 4 commonest degenerative neuro-muscular disorders (limbgirdle and Duchenne muscular dystrophy, Kugelberg-Welander's spinal muscular atrophy and peroneal muscular atrophy) were screened for scoliosis, which was found in 56%. Of 76 patients in the early stages of their respective discorders (stages 1–6 of Gardner-Medwin and Walton), scoliosis was found in 72%. The incidence of scoliosis was not related to the duration or degree of clinical weakness. Morphologically, scoliosis in these disorders was not found to differ from idiopathic scoliosis.
Neither side nor site of the scoliosis were related to the distribution of muscle weakness as determined by manual testing.
Résumé
L'examen systématique de 170 sujets atteints d'une des quatre maladies dégénératives neuromusculaires les plus fréquentes (la forme infantile de la myopathie primitive progressive de Duchenne, la myopathie atrophique progressive, l'atrophie musculaire progressive de Kugelberg-Welander et l'amyotrophie péronière de Charcot-Marie-Tooth) a mis en évidence une scoliose dans 56% des cas. Des 76 patients au stade initial d'une de ces maladies (stades 1 à 6 de Gardner-Medwin et Walton) 72% présentaient une scoliose.
Il n'y avait pas de corrélation entre la fréquence de celle-ci et la durée ou le degré de la myopathie. Du point de vue morphologique, la scoliose associée à ces maladies ne différait pas de la scoliose idiopathique. Enfin, ni le côté ni le niveau de la scoliose n'étaient en rapport avec la distribution de l'atrophie musculaire, évaluée par l'examen clinique.
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