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Compound Muscle Action Potential and Motor Function in Children with Spinal Muscular Atrophy

Overview
Journal Muscle Nerve
Date 2010 Aug 26
PMID 20737553
Citations 45
Authors
Aga Lewelt
Kristin J Krosschell
Charles Scott
Ai Sakonju
John T Kissel
Thomas O Crawford
Gyula Acsadi
Guy DAnjou
Bakri Elsheikh
Sandra P Reyna
Mary K Schroth
Jo Anne Maczulski
Gregory J Stoddard
Elie Elovic
Kathryn J Swoboda
Affiliations
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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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