Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function.

Eugenio Maria Mercuri, Elena Stacy Mazzone, Giorgia Coratti, 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, Robert Muni Lofra, Lavinia Fanelli, Roberto De Sanctis, Nicola ForcinaClaudia Chiriboga, Basil T. Darras, Gihan I. Tennekoon, Mariacristina Scoto, John W. Day, Richard Finkel, Francesco Muntoni, Eugenio Mercuri, Darryl C. De Vivo

Risultato della ricerca: Contributo in rivistaArticolo in rivista

7 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)209-215
Numero di pagine7
RivistaPediatric Physical Therapy
DOI
Stato di pubblicazionePubblicato - 2018

Keywords

  • spinal muscular atrophy

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