Kim Jee-Eun, Hong Yoon-Ho, Lee Joon-Hyung, Ahn Suk-Won, Kim Sung-Min, Park Kyung-Seok, Sung Jung-Joon, Lee Kwang-Woo, Seong Seung-Yong
Department of Neurology, Seoul Medical Center, Seoul, Republic of Korea.
Muscle Nerve. 2015 Mar;51(3):333-7. doi: 10.1002/mus.24323. Epub 2015 Jan 29.
Split hand is considered to be a specific feature of amyotrophic lateral sclerosis (ALS).
We evaluated the pattern difference of intrinsic hand muscles of upper limb-onset ALS (UL-ALS), upper limb-onset progressive muscular atrophy (UL-PMA), brachial amyotrophic diplegia (BAD), and Hirayama disease (HD) by measuring objective electrophysiological markers.
The abductor digiti minimi (ADM)/abductor pollicis brevis (APB) compound muscle action potential (CMAP) amplitude ratio was significantly higher in UL-ALS than other variants, but a considerable proportion of UL-ALS cases had an amplitude ratio in the range of other variants. Absent APB CMAP and abnormally high ADM/APB CMAP amplitude ratio (≥4) occurred only with UL-ALS. Conversely, an absent ADM CMAP was identified only in UL-PMA and BAD.
The absolute ADM/APB CMAP amplitude ratio was not specific for ALS; however, several findings from simple electrophysiological measurements may help predict prognosis in patients with motor neuron diseases and may be early diagnostic markers for ALS.
裂手被认为是肌萎缩侧索硬化症（ALS）的一种特定特征。
我们通过测量客观电生理指标，评估了上肢起病的ALS（UL-ALS）、上肢起病的进行性肌肉萎缩（UL-PMA）、臂丛性肌萎缩性双侧瘫（BAD）和平山病（HD）患者手部固有肌的模式差异。
UL-ALS患者的小指展肌（ADM）/拇短展肌（APB）复合肌肉动作电位（CMAP）波幅比显著高于其他类型，但相当一部分UL-ALS患者的波幅比处于其他类型的范围内。仅在UL-ALS患者中出现APB CMAP缺失和异常高的ADM/APB CMAP波幅比（≥4）。相反，仅在UL-PMA和BAD患者中发现ADM CMAP缺失。
ADM/APB CMAP的绝对波幅比对ALS并不具有特异性；然而，简单电生理测量的一些结果可能有助于预测运动神经元疾病患者的预后，并且可能是ALS的早期诊断标志物。
