Document Detail


Compound muscle action potential and motor function in children with spinal muscular atrophy.
MedLine Citation:
PMID:  20737553     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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
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Publication Detail:
Type:  Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Muscle & nerve     Volume:  42     ISSN:  1097-4598     ISO Abbreviation:  Muscle Nerve     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-10-26     Completed Date:  2010-11-22     Revised Date:  2014-03-19    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  United States    
Other Details:
Languages:  eng     Pagination:  703-8     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Action Potentials / physiology*
Adolescent
Carnitine / therapeutic use
Child
Child, Preschool
Electromyography
Female
GABA Agents / therapeutic use
Humans
Male
Movement / physiology
Muscle, Skeletal / physiopathology*
ROC Curve
Reproducibility of Results
Spinal Muscular Atrophies of Childhood / drug therapy,  physiopathology*
Treatment Outcome
Ulnar Nerve / physiopathology
Valproic Acid / therapeutic use
Grant Support
ID/Acronym/Agency:
R01 HD054599/HD/NICHD NIH HHS; R01 HD054599-01/HD/NICHD NIH HHS; R01 HD054599-05/HD/NICHD NIH HHS; R01-HD054599/HD/NICHD NIH HHS; UL1 RR025005/RR/NCRR NIH HHS; UL1 RR025755/RR/NCRR NIH HHS; UL1 RR025764-01/RR/NCRR NIH HHS; UL1RR025005/RR/NCRR NIH HHS; UL1RR025755/RR/NCRR NIH HHS; UL1RR025764/RR/NCRR NIH HHS
Chemical
Reg. No./Substance:
0/GABA Agents; 541-15-1/Carnitine; 614OI1Z5WI/Valproic Acid
Comments/Corrections

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