ELECTRICAL IMPEDANCE MYOGRAPHY IN INDIVIDUALS WITH COLLAGEN 6 AND LAMININ α-2 CONGENITAL MUSCULAR DYSTROPHY: A CROSS-SECTIONAL AND 2-YEAR ANALYSIS

被引：11

作者：

Nichols, Carmel ^{[1
,2
]}

Jain, Minal S. ^{[1
]}

Meilleur, Katherine G. ^{[3
]}

Wu, Tianxia ^{[4
]}

Collins, James ^{[5
]}

Waite, Melissa R. ^{[1
]}

Dastgir, Jahannaz ^{[6
]}

Salman, Anam ^{[7
,8
]}

Donkervoort, Sandra ^{[9
]}

Duong, Tina ^{[10
]}

Keller, Katherine ^{[11
]}

Leach, Meganne E. ^{[9
]}

Lott, Donovan J. ^{[12
]}

Mcguire, Michelle N. ^{[13
]}

Nelson, Leslie ^{[14
]}

Rutkowski, Anne ^{[15
]}

Vuillerot, Carole ^{[16
,17
]}

Bonnemann, Carsten G. ^{[9
]}

Lehky, Tanya J. ^{[7
]}

机构：

[1] NIH, Mark O Hatfield Clin Res Ctr, Bldg 10, Bethesda, MD 20892 USA

[2] Univ Chicago, Sch Med, Chicago, IL 60637 USA

[3] NINR, Tissue Injury Branch, NIH, Bethesda, MD 20892 USA

[4] NINDS, Clin Trials Unit, NIH, Bldg 36,Rm 4D04, Bethesda, MD 20892 USA

[5] Mercy Clin Pediat Neurol, Springfield, MO USA

[6] Columbia Univ, New York, NY USA

[7] NINDS, EMG Sect, NIH, Bldg 10,Room 7-5680, Bethesda, MD 20892 USA

[8] Mercy Hosp, Baltimore, MD USA

[9] NINDS, Neuromuscular & Neurogenet Disorders Childhood Se, NIH, Bldg 36,Rm 4D04, Bethesda, MD 20892 USA

[10] Stanford Childrens Hlth, Stanford, CA USA

[11] Rady Childrens Hosp, San Diego, CA USA

[12] Univ Florida, Gainesville, FL USA

[13] Cincinnati Childrens Hosp, Cincinnati, OH USA

[14] Univ Texas Southwestern Med Ctr Dallas, Dallas, TX 75390 USA

[15] Kaiser Permanente SCPMG Cure CMD, Los Angeles, CA USA

[16] Hosp Civils Lyon, Hop Femme Mere Enfant, Bron, France

[17] Univ Lyon, Lyon, France

来源：

MUSCLE & NERVE | 2018年 / 57卷 / 01期

关键词：

COL6 related dystrophies; congenital muscular dystrophy; electrical impedance myography; LAMA2 related dystrophies; neuromuscular disorders; WHOLE VASTUS LATERALIS; DIFFERENT FIBER TYPES; MYOFIBER HYPERTROPHY; MYONUCLEAR ADDITION; SKELETAL-MUSCLE; MOTOR UNITS; OLDER MEN; NUMBER; YOUNG; MECHANISMS;

D O I：

10.1002/mus.25629

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Introduction: Electrical impedance myography (EIM) is a noninvasive electrophysiological technique that characterizes muscle properties through bioimpedance. We compared EIM measurements to function, strength, and disease severity in a population with congenital muscular dystrophy (CMD). Methods: Forty-one patients with CMD, either collagen 6 related disorders (COL6-RD; n=21) or laminin a-2-related disorders (LAMA2-RD; n=20), and 21 healthy pediatric controls underwent 2 yearly EIM exams. In the CMD cohorts, EIM was compared with functional and strength measurements. Results: Both CMD cohorts exhibited change over time and had correlation with disease severity. The 50-kHZ phase correlated well with function and strength in the COL6-RD cohort but not in the LAMA2-RD cohort. Discussion: EIM is a potentially useful measure in clinical studies with CMD because of its sensitivity to change over a 1-year period and correlation with disease severity. For COL6-RD, there were also functional and strength correlations.

引用

页码：54 / 60

页数：7

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