Hu Fangfang, Jin Jiaoting, Qu Qiumin, Dang Jingxia
Department of Neurology, the First Affiliated Hospital, Medical College, Xi'an Jiaotong University, Xi'an, China.
J Clin Neurophysiol. 2016 Feb;33(1):60-5. doi: 10.1097/WNP.0000000000000226.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive loss of motor neurons, but it is increasingly recognized to be a more disseminated disease. The literature on the involvement of the sympathetic skin response (SSR) in ALS is few.
We reviewed the literature with specific emphasis on SSR in ALS and investigated SSR in 120 patients with sporadic ALS and in 130 age-matched healthy subjects to determine the effects of SSR in ALS patients. The SSR was conducted in all ALS patients and healthy subjects.
We found prolonged mean SSR latency in ALS patients, and the mean SSR amplitude is reduced compared with that of control subjects, especially in their low extremities (P < 0.05). The disease duration seems to have little impact on the SSR latency and amplitude (P > 0.05). Yet, whichever arm or leg involved, the overall difference in lower extremity SSR measurements between the two groups is significant (P < 0.05). And there is no correlation between initial manifestation of autonomic nervous impairment and amplitude and latency of SSR (P > 0.05).
The SSR impairment occurs mainly in lower extremities, which is earlier than clinical manifestation of autonomic nervous impairment in ALS. This may be a subclinical manifestation of ALS. We hypothesize that these results are caused by damage to the unmyelinated postganglionic fibers in ALS patients.
肌萎缩侧索硬化症(ALS)是一种神经退行性疾病,其特征为运动神经元进行性丧失,但人们越来越认识到它是一种更为广泛的疾病。关于交感神经皮肤反应(SSR)在ALS中受累情况的文献较少。
我们回顾了文献,特别关注ALS中的SSR,并对120例散发性ALS患者和130例年龄匹配的健康受试者进行了SSR研究,以确定SSR在ALS患者中的影响。对所有ALS患者和健康受试者都进行了SSR检测。
我们发现ALS患者的平均SSR潜伏期延长,与对照组相比,平均SSR波幅降低,尤其是在下肢(P < 0.05)。病程似乎对SSR潜伏期和波幅影响不大(P > 0.05)。然而,无论累及哪一侧上肢或下肢,两组之间下肢SSR测量的总体差异均有统计学意义(P < 0.05)。自主神经功能障碍的初始表现与SSR波幅和潜伏期之间无相关性(P > 0.05)。
SSR损害主要发生在下肢,早于ALS患者自主神经功能障碍临床表现,这可能是ALS的一种亚临床表型。我们推测这些结果是由ALS患者无髓节后纤维受损所致。