Park Donghwi, Park Jin-Sung
Department of Rehabilitation Medicine, Daegu Fatima Hospital, Daegu, South Korea.
Department of Neurology, Kyungpook National University School of Medicine, 680 Gukchaebosang-ro, Jung-gu, Daegu, 700-842, South Korea.
Neurol Sci. 2017 May;38(5):775-781. doi: 10.1007/s10072-017-2842-8. Epub 2017 Feb 10.
Amyotrophic lateral sclerosis (ALS) has a peculiar involvement pattern; clinically it is known as split hand syndrome and electrophysiologically shows abnormalities in the abductor pollicis brevis (APB)/abductor digiti minimi (ADM) ratio. The aim of this study was to find a significant electrophysiological parameter in upper limb onset ALS patients with normal APB/ADM ratio when compared to cervical spondylotic amyotrophy (CSA) and healthy controls. We retrospectively reviewed the electrophysiological results of 47 upper limb onset ALS and 42 CSA cases; 20 healthy individuals were included as controls. We included ALS and CSA patients with normal ADM/APB ratio (≥0.6, and ≤1.7), and the parameters of electrophysiological study were compared. The electrophysiological parameters of statistical significance among ALS, CSA and normal controls were: amplitude of median and ulnar nerves, the terminal latency of median nerve, F-wave latency of median and ulnar nerves, terminal latency ratio of ulnar/median nerves, and F-wave latency ratio of ulnar/median nerves (p < 0.05). Among these parameters, the terminal latency ratio of ulnar/median nerve and terminal latency of median nerve in ALS were significantly different with both of CSA and normal control (p < 0.006). The abnormality in the terminal latency of the median nerve can be partly explained by the distal motor axonal dysfunction due to sodium and potassium channel abnormalities. The hypothesis of distal axonopathy is known to play an important role in the pathogenesis of ALS causing a significant prolongation of the terminal latency in the median nerve and the ulnar/median nerve ratio.
肌萎缩侧索硬化症（ALS）有独特的受累模式；临床上称为裂手综合征，电生理显示拇短展肌（APB）/小指展肌（ADM）比值异常。本研究的目的是在与颈椎病性肌萎缩（CSA）和健康对照相比时，找到上肢起病且APB/ADM比值正常的ALS患者的一个显著电生理参数。我们回顾性分析了47例上肢起病的ALS患者和42例CSA患者的电生理结果；纳入20名健康个体作为对照。我们纳入了ADM/APB比值正常（≥0.6且≤1.7）的ALS和CSA患者，并比较了电生理研究参数。ALS、CSA和正常对照之间具有统计学意义的电生理参数为：正中神经和尺神经的波幅、正中神经的终末潜伏期、正中神经和尺神经的F波潜伏期、尺神经/正中神经的终末潜伏期比值以及尺神经/正中神经的F波潜伏期比值（p<0.05）。在这些参数中，ALS患者的尺神经/正中神经终末潜伏期比值和正中神经终末潜伏期与CSA和正常对照均有显著差异（p<0.006）。正中神经终末潜伏期异常可部分归因于钠钾通道异常导致的远端运动轴突功能障碍。已知远端轴索性神经病假说在ALS发病机制中起重要作用，导致正中神经终末潜伏期和尺神经/正中神经比值显著延长。
