COMPOUND MUSCLE ACTION POTENTIAL AND MOTOR FUNCTION IN CHILDREN WITH SPINAL MUSCULAR ATROPHY

被引：83

作者：

Lewelt, Aga ^{[1
]}

Krosschell, Kristin J. ^{[2
]}

Scott, Charles ^{[3
]}

Sakonju, Ai ^{[4
]}

Kissel, John T. ^{[5
]}

Crawford, Thomas O. ^{[6
]}

Acsadi, Gyula ^{[7
]}

D'anjou, Guy ^{[8
]}

Elsheikh, Bakri ^{[5
]}

Reyna, Sandra P. ^{[4
]}

Schroth, Mary K. ^{[9
]}

Maczulski, Jo Anne ^{[10
]}

Stoddard, Gregory J. ^{[11
]}

Elovic, Elie ^{[1
]}

Swoboda, Kathryn J. ^{[4
]}

机构：

[1] Univ Utah, Sch Med, Div Phys Med & Rehabil, Salt Lake City, UT 84132 USA

[2] Northwestern Univ, Feinberg Sch Med, Dept Phys Therapy & Human Movement Sci, Chicago, IL 60611 USA

[3] CBS Squared Inc, Ft Washington, PA USA

[4] Univ Utah, Sch Med, Dept Neurol, Salt Lake City, UT 84132 USA

[5] Ohio State Univ, Med Ctr, Dept Neurol, Columbus, OH 43210 USA

[6] Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA

[7] Wayne State Univ, Sch Med, Dept Neurol, Detroit, MI 48201 USA

[8] Hop St Justine, Div Pediat Neurol, Montreal, PQ H3T 1C5, Canada

[9] Univ Wisconsin, Sch Med, Dept Pediat, Madison, WI USA

[10] Pediat Occupat Therapy Serv, Chicago, IL USA

[11] Univ Utah, Sch Med, Dept Internal Med, Salt Lake City, UT 84132 USA

来源：

MUSCLE & NERVE | 2010年 / 42卷 / 05期

基金：

美国国家卫生研究院;

关键词：

compound muscle action potential; motor function; outcome measures; pediatrics; spinal muscular atrophy; NATURAL-HISTORY; SMA;

D O I：

10.1002/mus.21838

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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. Muscle Nerve 42: 703-708, 2010

引用

页码：703 / 708

页数：6

共 13 条

[1] Spinal muscular atrophy: Survival pattern and functional status [J].

Chung, BHY ;

Wong, VCN ;

Ip, P .

PEDIATRICS, 2004, 114 (05) :E548-E553

[2] Concerns about the design of clinical trials for spinal muscular atrophy [J].

Crawford, TO .

NEUROMUSCULAR DISORDERS, 2004, 14 (8-9) :456-460

[3]

Emery A E, 1991, Neuromuscul Disord, V1, P19, DOI 10.1016/0960-8966(91)90039-U

[4] Differences in SMN1 allele frequencies among ethnic groups within North America [J].

Hendrickson, B. C. ;

Donohoe, C. ;

Akmaev, V. R. ;

Sugarman, E. A. ;

Labrousse, P. ;

Boguslavskiy, L. ;

Flynn, K. ;

Rohlfs, E. M. ;

Walker, A. ;

Allitto, B. ;

Sears, C. ;

Scholl, T. .

JOURNAL OF MEDICAL GENETICS, 2009, 46 (09) :641-644

[5]

Hosmer W., 2000, Applied Logistic Regression, VSecond

[6] A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy [J].

Krosschell, Kristin J. ;

Maczulski, Jo Anne ;

Crawford, Thomas O. ;

Scott, Charles ;

Swoboda, Kathryn J. .

NEUROMUSCULAR DISORDERS, 2006, 16 (07) :417-426

[7]

McDowell I., 1996, MEASURING HLTH GUIDE

[8] Function changes in spinal muscular atrophy II and III [J].

Russman, BS ;

Buncher, CR ;

White, M ;

Samaha, FJ ;

Iannaccone, ST .

NEUROLOGY, 1996, 47 (04) :973-976

[9] Strength training by children and adolescents [J].

Small, Eric W. ;

McCambridge, Teri M. ;

Benjamin, Holly J. ;

Bernhardt, David T. ;

Brenner, Joel S. ;

Cappetta, Charles T. ;

Congeni, Joseph A. ;

Gregory, Andrew John Maxwell ;

Griesemer, Bernard A. ;

Reed, Frederick E. ;

Rice, Stephen G. .

PEDIATRICS, 2008, 121 (04) :835-840

[10] Phase II Open Label Study of Valproic Acid in Spinal Muscular Atrophy [J].

Swoboda, Kathryn J. ;

Scott, Charles B. ;

Reyna, Sandra P. ;

Prior, Thomas W. ;

LaSalle, Bernard ;

Sorenson, Susan L. ;

Wood, Janine ;

Acsadi, Gyula ;

Crawford, Thomas O. ;

Kissel, John T. ;

Krosschell, Kristin J. ;

D'Anjou, Guy ;

Bromberg, Mark B. ;

Schroth, Mary K. ;

Chan, Gary M. ;

Elsheikh, Bakri ;

Simard, Louise R. .

PLOS ONE, 2009, 4 (05)

← 1 2 →