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Lookup NU author(s): Dr Anna Mayhew, Robert Muni Lofra
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© 2018 Academy of Pediatric Physical Therapy of the American Physical Therapy Association. 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.
Author(s): Salazar R, Montes J, Young SD, McDermott MP, Martens W, Pasternak A, Quigley J, Mirek E, Glanzman AM, Civitello M, Gee R, Duong T, Mazzone ES, Main M, Mayhew A, Ramsey D, Muni Lofra R, Coratti G, Fanelli L, De Sanctis R, Forcina N, Chiriboga C, Darras BT, Tennekoon GI, Scoto M, Day JW, Finkel R, Muntoni F, Mercuri E, De Vivo DC
Publication type: Article
Publication status: Published
Journal: Pediatric Physical Therapy
Year: 2018
Volume: 30
Issue: 3
Pages: 209-215
Online publication date: 01/07/2018
Acceptance date: 02/04/2018
ISSN (print): 0898-5669
ISSN (electronic): 1538-005X
Publisher: Lippincott Williams and Wilkins
URL: https://doi.org/10.1097/PEP.0000000000000515
DOI: 10.1097/PEP.0000000000000515
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