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

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; Danielle Ramsey; 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

doi:10.1097/PEP.0000000000000515

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

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, Danielle Ramsey, Robert Muni Lofra, Giorgia Coratti, Lavinia Fanelli, Roberto De SanctisNicola Forcina, Claudia Chiriboga, Basil T. Darras, Gihan I. Tennekoon, Mariacristina Scoto, John W. Day, Richard Finkel, Francesco Muntoni, Eugenio Mercuri, Darryl C. De Vivo

Research output: Contribution to journal › Article › peer-review

9 Citations (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.

Original language	English
Pages (from-to)	209-215
Number of pages	7
Journal	Pediatric Physical Therapy
Volume	30
Issue number	3
DOIs	https://doi.org/10.1097/PEP.0000000000000515
Publication status	Published - 1 Jul 2018

Keywords

contractures
motor function
outcome measures
range of motion
spinal muscular atrophy

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Salazar, R., Montes, J., Young, S. D., McDermott, M. P., Martens, W., Pasternak, A., Quigley, J., Mirek, E., Glanzman, A. M., Civitello, M., Gee, R., Duong, T., Mazzone, E. S., Main, M., Mayhew, A., Ramsey, D., Muni Lofra, R., Coratti, G., Fanelli, L., ... De Vivo, D. C. (2018). Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function. Pediatric Physical Therapy, 30(3), 209-215. https://doi.org/10.1097/PEP.0000000000000515

@article{54e473d3c1d74acba4e2c276ab195f9b,

title = "Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function",

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.",

keywords = "contractures, motor function, outcome measures, range of motion, spinal muscular atrophy",

author = "Rachel Salazar and Jacqueline Montes and Young, {Sally Dunaway} and McDermott, {Michael P.} and William Martens and Amy Pasternak and Janet Quigley and Elizabeth Mirek and Glanzman, {Allan M.} and Matt Civitello and Richard Gee and Tina Duong and Mazzone, {Elena S.} and Marion Main and Anna Mayhew and Danielle Ramsey and {Muni Lofra}, Robert and Giorgia Coratti and Lavinia Fanelli and {De Sanctis}, Roberto and Nicola Forcina and Claudia Chiriboga and Darras, {Basil T.} and Tennekoon, {Gihan I.} and Mariacristina Scoto and Day, {John W.} and Richard Finkel and Francesco Muntoni and Eugenio Mercuri and {De Vivo}, {Darryl C.}",

year = "2018",

month = jul,

day = "1",

doi = "10.1097/PEP.0000000000000515",

language = "English",

volume = "30",

pages = "209--215",

journal = "Pediatric Physical Therapy",

issn = "0898-5669",

publisher = "Lippincott Williams and Wilkins",

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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 2018, 'Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy: Implications for Motor Function', Pediatric Physical Therapy, vol. 30, no. 3, pp. 209-215. https://doi.org/10.1097/PEP.0000000000000515

TY - JOUR

T1 - Quantitative Evaluation of Lower Extremity Joint Contractures in Spinal Muscular Atrophy

T2 - Implications for Motor Function

AU - Salazar, Rachel

AU - Montes, Jacqueline

AU - Young, Sally Dunaway

AU - McDermott, Michael P.

AU - Martens, William

AU - Pasternak, Amy

AU - Quigley, Janet

AU - Mirek, Elizabeth

AU - Glanzman, Allan M.

AU - Civitello, Matt

AU - Gee, Richard

AU - Duong, Tina

AU - Mazzone, Elena S.

AU - Main, Marion

AU - Mayhew, Anna

AU - Ramsey, Danielle

AU - Muni Lofra, Robert

AU - Coratti, Giorgia

AU - Fanelli, Lavinia

AU - De Sanctis, Roberto

AU - Forcina, Nicola

AU - Chiriboga, Claudia

AU - Darras, Basil T.

AU - Tennekoon, Gihan I.

AU - Scoto, Mariacristina

AU - Day, John W.

AU - Finkel, Richard

AU - Muntoni, Francesco

AU - Mercuri, Eugenio

AU - De Vivo, Darryl C.

PY - 2018/7/1

Y1 - 2018/7/1

N2 - 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.

AB - 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.

KW - contractures

KW - motor function

KW - outcome measures

KW - range of motion

KW - spinal muscular atrophy

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U2 - 10.1097/PEP.0000000000000515

DO - 10.1097/PEP.0000000000000515

M3 - Article

AN - SCOPUS:85049336269

SN - 0898-5669

VL - 30

SP - 209

EP - 215

JO - Pediatric Physical Therapy

JF - Pediatric Physical Therapy

IS - 3

ER -

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

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