关键词: Amyotrophic lateral sclerosis CMAP Chronic inflammatory demyelinating polyneuropathy Conduction slowing Neuropathy

来  源:   DOI:10.1016/j.heliyon.2023.e18400   PDF(Pubmed)

Abstract:
UNASSIGNED: Since motor nerve conduction slowing can occur due to loss of large axons, we investigate the conduction slowing profile in amyotrophic lateral sclerosis (ALS) and identify the limits beyond which the diagnosis of exclusive axonal loss is unlikely.
UNASSIGNED: First, using linear regression analysis, we established the range of motor conduction slowing in 76 chronic inflammatory demyelinating polyneuropathy (CIDP) patients. Demyelinating range confidence intervals were defined by assessing conduction velocity (CV), distal latency (DML), and F-wave latency (F) in relation to distal compound muscle action potential (CMAP) amplitude of median, ulnar, fibular, and tibial nerves. Results were subsequently validated in 38 additional CIDP patients. Then, the newly established demyelination confidence intervals were used to investigate the profile of conduction slowing in 95 ALS patients.
UNASSIGNED: CV slowing, prolonged DML, and abnormal F were observed in 22.2%, 19.6%, and 47.1% of the studied nerves respectively in ALS patients. When slowing occurred, it affected more than one segment of the motor nerve, suggesting that CMAP amplitude dependent conduction slowing caused by an exclusive loss of large axons is the main mechanism of slowing. No ALS patient had more than 2 nerves with CV slowing in the confidence interval defined by the regression equations or the American Academy of Neurology (AAN) research criteria for CIDP diagnosis.
UNASSIGNED: The presence of more than two motor nerves with CV slowing in the demyelinating range defined by the regression analysis or AAN criteria in ALS patients suggests the contribution of acquired demyelination or other additional mechanisms exist in the electrodiagnostic profile of ALS.
摘要:
由于大轴突的丢失会导致运动神经传导减慢,我们研究了肌萎缩侧索硬化(ALS)的传导减慢情况,并确定了超过该限度就不可能诊断为唯一轴突丢失.
首先,使用线性回归分析,我们在76例慢性炎症性脱髓鞘性多发性神经病(CIDP)患者中建立了运动传导减慢的范围.通过评估传导速度(CV)定义脱髓鞘范围置信区间,远端潜伏期(DML),和F波潜伏期(F)与远端复合肌肉动作电位(CMAP)幅度的关系,尺骨,腓骨,和胫神经.随后在38名额外的CIDP患者中验证了结果。然后,新建立的脱髓鞘置信区间用于调查95例ALS患者的传导减慢情况.
CV变慢,延长DML,异常F占22.2%,19.6%,ALS患者分别占研究神经的47.1%。当减速发生时,它影响了运动神经的一个以上部分,表明由大轴突的专有损失引起的CMAP振幅依赖性传导减慢是减慢的主要机制。在回归方程或美国神经病学学会(AAN)诊断CIDP的研究标准定义的置信区间内,没有ALS患者的CV减慢超过2条神经。
在ALS患者中存在超过两条运动神经,其CV在回归分析或AAN标准定义的脱髓鞘范围内减慢,这表明获得性脱髓鞘或其他额外机制存在于ALS的电诊断谱中。
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