Compound muscle action potential and motor function in children with spinal muscular atrophy

Aga Lewelt; Kristin J. Krosschell; Charles Scott; Ai Sakonju; John T. Kissel; Thomas O. Crawford; Gyula Acsadi; Guy D'Anjou; Bakri Elsheikh; Sandra P. Reyna; Mary K. Schroth; Jo Anne Maczulski; Gregory J. Stoddard; Elie Elovic; Kathryn J. Swoboda

doi:10.1002/mus.21838

Compound muscle action potential and motor function in children with spinal muscular atrophy

Aga Lewelt, Kristin J. Krosschell, Charles Scott, Ai Sakonju, John T. Kissel, Thomas O. Crawford, Gyula Acsadi, Guy D'Anjou, Bakri Elsheikh, Sandra P. Reyna, Mary K. Schroth, Jo Anne Maczulski, Gregory J. Stoddard, Elie Elovic, Kathryn J. Swoboda

School of Medicine

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

65 Scopus citations

Abstract

Reliable outcome measures that reflect the underlying disease process and correlate with motor function in children with SMA are needed for clinical trials. Maximum ulnar compound muscle action potential (CMAP) data were collected at two visits over a 4-6-week period in children with SMA types II and III, 2-17 years of age, at four academic centers. Primary functional outcome measures included the Modified Hammersmith Functional Motor Scale (MHFMS) and MHFMS-Extend. CMAP negative peak amplitude and area showed excellent discrimination between the ambulatory and non-ambulatory SMA cohorts (ROC = 0.88). CMAP had excellent test-retest reliability (ICC = 0.96-0.97, n = 64) and moderate to strong correlation with the MHFMS and MHFMS-Extend (r = 0.61-0.73, n = 68, P < 0.001). Maximum ulnar CMAP amplitude and area is a feasible, valid, and reliable outcome measure for use in pediatric multicenter clinical trials in SMA. CMAP correlates well with motor function and has potential value as a relevant surrogate for disease status.

Original language	English (US)
Pages (from-to)	703-708
Number of pages	6
Journal	Muscle and Nerve
Volume	42
Issue number	5
DOIs	https://doi.org/10.1002/mus.21838
State	Published - Nov 2010

Keywords

Compound muscle action potential
Motor function
Outcome measures
Pediatrics
Spinal muscular atrophy

ASJC Scopus subject areas

Physiology
Clinical Neurology
Cellular and Molecular Neuroscience
Physiology (medical)

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10.1002/mus.21838

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Lewelt, A., Krosschell, K. J., Scott, C., Sakonju, A., Kissel, J. T., Crawford, T. O., Acsadi, G., D'Anjou, G., Elsheikh, B., Reyna, S. P., Schroth, M. K., Maczulski, J. A., Stoddard, G. J., Elovic, E., & Swoboda, K. J. (2010). Compound muscle action potential and motor function in children with spinal muscular atrophy. Muscle and Nerve, 42(5), 703-708. https://doi.org/10.1002/mus.21838

Lewelt, A, Krosschell, KJ, Scott, C, Sakonju, A, Kissel, JT, Crawford, TO, Acsadi, G, D'Anjou, G, Elsheikh, B, Reyna, SP, Schroth, MK, Maczulski, JA, Stoddard, GJ, Elovic, E & Swoboda, KJ 2010, 'Compound muscle action potential and motor function in children with spinal muscular atrophy', Muscle and Nerve, vol. 42, no. 5, pp. 703-708. https://doi.org/10.1002/mus.21838

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abstract = "Reliable outcome measures that reflect the underlying disease process and correlate with motor function in children with SMA are needed for clinical trials. Maximum ulnar compound muscle action potential (CMAP) data were collected at two visits over a 4-6-week period in children with SMA types II and III, 2-17 years of age, at four academic centers. Primary functional outcome measures included the Modified Hammersmith Functional Motor Scale (MHFMS) and MHFMS-Extend. CMAP negative peak amplitude and area showed excellent discrimination between the ambulatory and non-ambulatory SMA cohorts (ROC = 0.88). CMAP had excellent test-retest reliability (ICC = 0.96-0.97, n = 64) and moderate to strong correlation with the MHFMS and MHFMS-Extend (r = 0.61-0.73, n = 68, P < 0.001). Maximum ulnar CMAP amplitude and area is a feasible, valid, and reliable outcome measure for use in pediatric multicenter clinical trials in SMA. CMAP correlates well with motor function and has potential value as a relevant surrogate for disease status.",

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Compound muscle action potential and motor function in children with spinal muscular atrophy

Abstract

Keywords

ASJC Scopus subject areas

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