| Compound muscle action potential and motor function in children with spinal muscular atrophy. | |
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MedLine Citation:
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PMID: 20737553 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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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:
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Type: Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Muscle & nerve Volume: 42 ISSN: 1097-4598 ISO Abbreviation: Muscle Nerve Publication Date: 2010 Nov |
Date Detail:
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Created Date: 2010-10-26 Completed Date: 2010-11-22 Revised Date: 2013-01-28 |
Medline Journal Info:
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Nlm Unique ID: 7803146 Medline TA: Muscle Nerve Country: United States |
Other Details:
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Languages: eng Pagination: 703-8 Citation Subset: IM |
Affiliation:
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Division of Physical Medicine and Rehabilitation, University of Utah School of Medicine, Salt Lake City, Utah 84132, USA. aga@genetics.utah.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Action Potentials
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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:
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R01 HD054599-01/HD/NICHD NIH HHS; R01 HD054599-05/HD/NICHD NIH HHS; R01-HD054599/HD/NICHD 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:
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0/GABA Agents; 541-15-1/Carnitine; 99-66-1/Valproic Acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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