University of Hertfordshire

  • Rachel Salazar
  • Jacqueline Montes
  • Sally Dunaway Young
  • Michael P. McDermott
  • William Martens
  • Amy Pasternak
  • Janet Quigley
  • Elizabeth Mirek
  • Allan M. Glanzman
  • Matt Civitello
  • Richard Gee
  • Tina Duong
  • Elena S. Mazzone
  • Marion Main
  • Anna Mayhew
  • Robert Muni Lofra
  • Giorgia Coratti
  • Lavinia Fanelli
  • Roberto De Sanctis
  • Nicola Forcina
  • Claudia Chiriboga
  • Basil T. Darras
  • Gihan I. Tennekoon
  • Mariacristina Scoto
  • John W. Day
  • Richard Finkel
  • Francesco Muntoni
  • Eugenio Mercuri
  • Darryl C. De Vivo
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Original languageEnglish
Number of pages7
Pages (from-to)209-215
JournalPediatric Physical Therapy
Journal publication date1 Jul 2018
Volume30
Issue3
DOIs
Publication statusPublished - 1 Jul 2018

Abstract

Purpose: To quantitatively describe passive lower extremity range of motion in participants with spinal muscular atrophy (SMA) types 2 and 3, and to establish preliminary thresholds to identify individuals at risk for performing poorly on disease-specific motor function outcome measures. Methods: Eighty participants with SMA types 2 and 3, enrolled in an international multicenter natural history study, were evaluated with lower extremity range of motion testing and the Hammersmith Functional Motor Scale-Expanded. Results: A hip extension joint angle of -7.5° or less for SMA type 2 and 0° or less for SMA type 3 identified diminished motor ability with good sensitivity. For knee extension, a joint angle of -9.0° or less for SMA type 2 or 0° or less for SMA type 3 was similarly sensitive. Conclusions: Minimal hip and knee joint contractures were associated with diminished motor ability. Clinical trial designs should consider the effect of contractures on motor function.

ID: 15289433