Motor unit number estimation in infants and children with spinal muscular atrophy

被引:65
作者
Bromberg, MB [1 ]
Swoboda, KJ [1 ]
机构
[1] Dept Neurol, Salt Lake City, UT 84132 USA
关键词
motor neuron disease; motor unit number estimation (MUNE); spinal muscular atrophy (SMA);
D O I
10.1002/mus.10050
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Spinal muscular atrophy (SMA) is a disease of lower motor neurons. Motor unit number estimation (MUNE) is an electrophysiologic method to estimate the number of motor neurons innervating a muscle group. We applied the multiple point stimulation technique to the ulnar nerve-hypothenar muscle group to study lower motor neuron loss in 14 SMA subjects, including those presymptomatic, and varying from newborn through 45 years of age. Preliminary data support the value of MUNE to help understand the time course of motor neuron loss in SMA. (C) 2002 Wiley Periodicals, Inc.
引用
收藏
页码:445 / 447
页数:3
相关论文
共 15 条
[1]   MOTOR UNIT ESTIMATION - REPRODUCIBILITY OF THE SPIKE-TRIGGERED AVERAGING TECHNIQUE IN NORMAL AND ALS SUBJECTS [J].
BROMBERG, MB .
MUSCLE & NERVE, 1993, 16 (05) :466-471
[2]   SOME ELECTRICAL-PROPERTIES OF MOTOR UNITS AND THEIR EFFECTS ON METHODS OF ESTIMATING MOTOR UNIT NUMBERS [J].
BROWN, WF ;
MILNERBROWN, HS .
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1976, 39 (03) :249-257
[3]   ELECTROMYOGRAPHY AND MUSCLE BIOPSY IN INFANTILE SPINAL MUSCULAR ATROPHY [J].
BUCHTHAL, F ;
OLSEN, PZ .
BRAIN, 1970, 93 :15-&
[4]   From enigmatic to problematic: The new molecular genetics of childhood spinal muscular atrophy [J].
Crawford, TO .
NEUROLOGY, 1996, 46 (02) :335-340
[5]   THE ESTIMATED NUMBERS AND RELATIVE SIZES OF THENAR MOTOR UNITS AS SELECTED BY MULTIPLE POINT STIMULATION IN YOUNG AND OLDER ADULTS [J].
DOHERTY, TJ ;
BROWN, WF .
MUSCLE & NERVE, 1993, 16 (04) :355-366
[6]  
FEINSTEIN B., 1955, ACTA ANAT, V23, P127
[7]   ELECTROMYOGRAPHIC FINDINGS IN DIFFERENT FORMS OF INFANTILE AND JUVENILE PROXIMAL SPINAL MUSCULAR-ATROPHY [J].
HAUSMANOWAPETRUSEWICZ, I ;
KARWANSKA, A .
MUSCLE & NERVE, 1986, 9 (01) :37-46
[8]   Prospective analysis of strength in spinal muscular atrophy [J].
Iannaccone, ST ;
Russman, BS ;
Browne, RH ;
Buncher, CR ;
White, M ;
Samaha, FJ .
JOURNAL OF CHILD NEUROLOGY, 2000, 15 (02) :97-101
[9]   IDENTIFICATION AND CHARACTERIZATION OF A SPINAL MUSCULAR ATROPHY-DETERMINING GENE [J].
LEFEBVRE, S ;
BURGLEN, L ;
REBOULLET, S ;
CLERMONT, O ;
BURLET, P ;
VIOLLET, L ;
BENICHOU, B ;
CRUAUD, C ;
MILLASSEAU, P ;
ZEVIANI, M ;
LEPASLIER, D ;
FREZAL, J ;
COHEN, D ;
WEISSENBACH, J ;
MUNNICH, A ;
MELKI, J .
CELL, 1995, 80 (01) :155-165
[10]  
McComas A.J., 1987, CLIN ELECTROMYOGRAPH, P431