Endomicroscopy and electromyography of neuromuscular junctions in situ.
Endomicroscopy and electromyography of neuromuscular junctions in situ.
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DOI：
10.1002/acn3.124
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发表时间：
2014-11
影响因子：
5.3
通讯作者：
Ribchester, Richard R.
中科院分区：
文献类型：
--
作者：
Brown, Rosalind;Dissanayake, Kosala N.;Skehel, Paul A.;Ribchester, Richard R.
肌电图（EMG）常规用于诊断神经肌肉功能障碍的范围广泛的周围神经病，肌病和神经肌肉退行性疾病，包括运动神经元疾病，如肌萎缩性侧索硬化症（ALS）。明确的神经学诊断也可以通过运动点活检病理神经肌肉神经支配的分析来指出。在这项研究中，我们的目的是通过结合活体成像和体内神经肌肉连接（NMJs）缓慢变性的电生理分析来预防运动点活检。我们将传统的针肌电图与光纤共聚焦内窥镜（CEM）相结合，使用一个集成的手持式1.5 mm直径探针。我们以麻醉后双纯合thy1.2YFP16: WldS小鼠后肢的各种axo切除肌肉作为实验平台，这些肌肉在运动神经元中共同表达wallerandevethslow （WldS）蛋白和黄色荧光蛋白（YFP）。我们还测试了外源性生命染色剂，包括Alexa488-α-bungarotoxin；苯乙烯基吡啶染料4-二-2- asp；a型肉毒毒素重链GFP偶联物（GFP- hcbont / a）。我们的研究表明，综合肌电/脑电探针可以有效地纵向评估在7天内发生的功能和形态学变化，这些变化发生在肛门切开术诱导的缓慢神经肌肉突触变性过程中。肌电图振幅下降与运动神经末梢明显变性并行。神经末梢和运动终板选择性染色时，肌电图/CEM安全有效。基于动物模型的实时成像，我们的研究结果构成了概念验证，即肌电图/CEM结合可能是神经系统诊断的微创前驱或运动点活检的替代方法，也可用于监测潜在治疗药物的局部给药。
Electromyography (EMG) is used routinely to diagnose neuromuscular dysfunction in a wide range of peripheral neuropathies, myopathies, and neuromuscular degenerative diseases including motor neuron diseases such as amyotrophic lateral sclerosis (ALS). Definitive neurological diagnosis may also be indicated by the analysis of pathological neuromuscular innervation in motor-point biopsies. Our objective in this study was to preempt motor-point biopsy by combining live imaging with electrophysiological analysis of slow degeneration of neuromuscular junctions (NMJs) in vivo. We combined conventional needle electromyography with fiber-optic confocal endomicroscopy (CEM), using an integrated hand-held, 1.5-mm-diameter probe. We utilized as a test bed, various axotomized muscles in the hind limbs of anaesthetized, double-homozygous thy1.2YFP16: WldS mice, which coexpress the Wallerian-degeneration Slow (WldS) protein and yellow fluorescent protein (YFP) in motor neurons. We also tested exogenous vital stains, including Alexa488-α-bungarotoxin; the styryl pyridinium dye 4-Di-2-Asp; and a GFP conjugate of botulinum toxin Type A heavy chain (GFP-HcBoNT/A). We show that an integrated EMG/CEM probe is effective in longitudinal evaluation of functional and morphological changes that take place over a 7-day period during axotomy-induced, slow neuromuscular synaptic degeneration. EMG amplitude declined in parallel with overt degeneration of motor nerve terminals. EMG/CEM was safe and effective when nerve terminals and motor endplates were selectively stained with vital dyes. Our findings constitute proof-of-concept, based on live imaging in an animal model, that combining EMG/CEM may be useful as a minimally invasive precursor or alternative to motor-point biopsy in neurological diagnosis and for monitoring local administration of potential therapeutics.
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影响因子：
6.7
作者：
Couesnon A;Molgó J;Connan C;Popoff MR
通讯作者：
Popoff MR
影响因子：
3.7
作者：
Ben Ammar A;Soltanzadeh P;Bauché S;Richard P;Goillot E;Herbst R;Gaudon K;Huzé C;Schaeffer L;Yamanashi Y;Higuchi O;Taly A;Koenig J;Leroy JP;Hentati F;Najmabadi H;Kahrizi K;Ilkhani M;Fardeau M;Eymard B;Hantaï D
通讯作者：
Hantaï D
影响因子：
38.1
作者：
Boulis, Nicholas M.;Federici, Thais;Feldman, Eva L.
通讯作者：
Feldman, Eva L.
影响因子：
4.2
作者：
Chen S
通讯作者：
Chen S
影响因子：
2.5
作者：
Dawkins, R;Keller, SL;Sewell, WF
通讯作者：
Sewell, WF