Scoliosis in spinal muscular atrophy: Natural history and management

L. Merlini, C. Granata, S. Bonfiglioli, M. L. Marini, S. Cervellati, R. Savini

Research output: Contribution to journalArticle

Abstract

This is a report on the natural history of 109 patients with infantile spinal muscular atrophy (SMA). All 18 children with the severe form died, as did five of the 52 with the intermediate form. Functional ability was more or less stable in the 47 living patients with the intermediate form, but 16 of the 39 with mild SMA lost the ability to walk. Ventilatory function was severely impaired in those with intermediate SMA: orthopaedic treatment for scoliosis in this group did not prevent the curves from worsening with age. Scoliosis was also severe in patients with mild SMA who had stopped walking: surgical treatment in six cases resulted in stable correction of the spine, with functional and cosmetic improvement and without impairment of respiratory function.

Original languageEnglish
Pages (from-to)501-508
Number of pages8
JournalDevelopmental Medicine and Child Neurology
Volume31
Issue number4
Publication statusPublished - 1989

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Neuroscience(all)

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    Merlini, L., Granata, C., Bonfiglioli, S., Marini, M. L., Cervellati, S., & Savini, R. (1989). Scoliosis in spinal muscular atrophy: Natural history and management. Developmental Medicine and Child Neurology, 31(4), 501-508.