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.
Salazar, R., Montes, J., Dunaway Young, S., Mcdermott, M., Martens, W., Pasternak, A., Quigley, J., Mirek, E., Glanzman, A., Civitello, M., Gee, R., Duong, T., Mazzone, E. S., Main, M., Mayhew, A., Ramsey, D., Muni Lofra, R., Coratti, G., Fanelli, L., De Sanctis, R., Forcina, N., Chiriboga, C., Darras, B., Tennekoon, G., Scoto, M., Day, J., Finkel, R., Muntoni, F., Mercuri, E. M., De Vivo, D., Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function., <<PEDIATRIC PHYSICAL THERAPY>>, 2018; (30(3)): 209-215. [doi:10.1097/PEP.0000000000000515] [http://hdl.handle.net/10807/125536]
Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function.
Mazzone, Elena Stacy;Coratti, Giorgia;Mercuri, Eugenio Maria;
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.
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