重复神经刺激在肌萎缩侧索硬化和重症肌无力患者的比较研究

发布时间：2019-07-09 08:18

【摘要】：目的：1.收集湖北地区肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)患者信息,建立ALS数据库。2.比较ALS患者和重症肌无力(myasthenia gravis, MG)患者重复神经刺激(repetitive nerve stimulation, RN S)的阳性率差异和递减幅度特点。3.比较近、远端神经RNS阳性率差异。4.探索ALS和MG患者RNS递减机制的异同。5.探索ALS患者中临床指标(年龄、性别、起病部位、病程、ALSFRS-r评分、病情进展速度)对RNS结果的影响。6.探索ALS患者中电生理指标(正中神经、尺神经波幅和ADM/APB比值)与RNS结果的相关性。7.寻找预测RNS预后的相关指标。方法：1.收集2013年12月至2015年8月期间就诊于武汉大学人民医院,临床确诊或拟诊的ALS患者以及临床诊断的MG患者。2.记录ALS和MG患者的基本信息、病程,ALS患者评估改良ALS功能评分(revised ALS functional rating scale, ALSFRS-r)(总分48分),并计算病情进展速度,即(48-就诊时ALSFRS-r评分)÷病程(月数)。3.ALS患者进行常规神经传导和针电极肌电图(electromyography, EMG)检查,以及副、面、尺、正中神经低频RNS测定,记录连续10次刺激的反应,递减反应以第5个运动波的波幅较第1个降低≥8%为标准,≥1条神经显示递减反应判定为RNS阳性。4.分析ALS患者临床指标、神经电生理指标与RNS的关系。5.MG进行副、面、尺、正中神经低频RNS检测,并与ALS患者进行比较。结果：1.ALS患者68例、MG患者52例,RNS递减阳性者分别为37例(54.4%)、34例(65.4%),两组总的阳性率无差异(χ2=0.993,P=0.319)。2.尺、副神经RNS阳性率在ALS患者分别为4.4%、51.5%(2=6.972,P=0.000),尺、正中、面、副神经RNS阳性率在MG患者分别为15.4%、38.7%、56.0%、65.4%(χ2=30.152,P=0.000)3.尺神经RNS递减幅度(%)在ALS和MG患者分别为15.5±7.5、30.4±8.4(t=2.699,P=0.024)；副神经分别为18.2±7.1、27.3±10.7(t=17.442,P=0.000)。4.ALS患者RNS阳性和阴性亚组的ALSFRS-评分分别为38.0±6.1、39.7±5.0(t=0.256,P=0.609),病程(月)分别为10.9±8.8、13.0±10.1(t=0.929,P=0.339),病情进展速度分别为1.49±1.01、1.01±0.90(t=2.11,P=0.039)；正中神经神经波幅(mV)分别为2.8±2.4、4.6±2.7(t=-2.993,P=0.004),尺神经波幅(mV)分别为5.0±3.4、7.0±3.0(t=-2.496,P=0.016),ADM/APB比值分别为4.6±6.3、3.2±5.6(t=0.982,P=0.329)。5.ALS患者男性亚组RNS阳性率53%,女性亚组55%(χ2=0.024,P=0.878)；中青年组RNS阳性率57.5%,老年组50%(χ2=0.373,P=0.541)；球部起病亚组RNS阳性率25%,肢体起病亚组66.7%(χ2=9.881,P=0.002)。结论：1.ALS患者中RNS阳性率较高,高达50%以上,和MG患者无明显差异,但递减幅度没有MG患者高,提示ALS患者神经肌肉接头功能障碍比较常见,但是其机制可能与MG患者不同。2.低频RNS递减幅度大于20%不应该作为ALS排除诊断标准。3.在ALS患者中正中神经、尺神经运动波幅低,轴索损害越严重,终板也越容易出现传递功能障碍,更容易出现低频RNS递减反应。4.RNS阳性常提示ALS病情快速进展,是疾病活跃的表现,可用于预后判断和随访观察。5. ADM/APB比值诊断ALS时特异性较高,但在随访中并无优势。6.ALS患者的RNS结果和患者年龄、性别、ALSFRS-r、病程无明显相关性。
文内图片：

图片说明：图２邋ＡＬＳ患者尺神经和副神经ＲＮＳ阳性率比较（拉＝邋６．９７２，户＝０．０００）逡逑Ｆｉｇ．邋２邋Ｃｏｍｐａｒｉｓｏｎ邋ｏｆ邋化ｅ邋ｐｏｓｉｔｉｖｅ邋ｒａ化邋ｏｆ邋ＲＮＳ邋ｉｎ邋ＡＬＳ邋ｐａｔｉｅｎｔｓ邋（NB２邋＝邋６．９７２，b6＝０．０００）逡逑
[Abstract]:Objective:1. The information of amyotrophic lateral sclerosis (ALS) in the region of Hubei was collected and the ALS database was established. The positive rate and decreasing amplitude of repeat nerve stimulation (RN S) in patients with ALS and myasthenia gravis (MG) were compared. There was a difference in the positive rate of the distal nerve in the proximal and distal nerves. To explore the similarities and differences of the descending mechanism of RNS in ALS and MG patients. The effect of clinical indicators (age, sex, onset, course, ALSFRS-r score, disease progression rate) on the RNS results was investigated in ALS patients. The correlation between the electrical physiological index (median nerve, the amplitude of the ulnar nerve and the ADM/ APB ratio) and the results of the RNS was explored in ALS patients. Find relevant indicators for predicting the prognosis of the RNS. Method:1. From December 2013 to August 2015, the patients who visited the People's Hospital of Wuhan University, the patients with ALS diagnosed or to be diagnosed, and the MG patients with clinical diagnosis were collected. The basic information, course, ALS patient assessment for ALS and MG patients were recorded, the ALS functional rating scale (ALSFRS-r) was improved (total score of 48), and the disease progression rate was calculated. That is, (48-visit ALSFRS-r score) and course of disease (number of months).3. ALS patients undergo routine neuroconduction and needle electrode electromyography (EMG) examination, as well as secondary, surface, ruler, median nerve low frequency RNS measurement, record the response of continuous 10 times of stimulation, The decreasing response was based on the first decrease of the amplitude of the 5th motion wave and the first decrease in the first one. 5. The relationship between the clinical index, the neurophysiological index and the RNS in ALS patients was analyzed. Results:1.68 patients with ALS,52 patients with MG,37 (54.4%),34 (65.4%), and 2 (2 = 0.993, P = 0.319). The positive rate of RNS positive rate in the patients with ALS was 4.4%, 51.5% (2 = 6.972, P = 0.000), and the positive rate of the median, median, and secondary nerve was 15.4%, 38.7%, 56.0%, 65.4% (Sup2 = 30.152, P = 0.000)3, respectively. The descending amplitude of the ulnar nerve (%) was 15.5, 7.5, 30.4 and 8.4 (t = 2.699, P = 0.024) in ALS and MG respectively; the secondary nerves were 18.2, 7.1, 27.3, 10.7 (t = 17.442, P = 0.000), respectively. The ALSFRS-scores in the positive and negative subgroups of the RNS in the ALS patients were 38.0, 6.1, 39.7 and 5.0 (t = 0.256, P = 0.609), respectively, and the course of the disease (months) was 10.9, 8.8, 13.0 and 10.1 (t = 0.929, P = 0.339), respectively. The rate of disease progression was 1.49, 1.01, 1.01 and 0.90 (t = 2.11, P = 0.039). The median nerve wave amplitude (mV) was 2.8, 2.4, 4.6, 2.7 (t =-2.993, P = 0.004), and the amplitude of the ulnar nerve (mV) was 5.0, 3.4, 7.0, 3.0 (t =-2.496, P = 0.016), and the ratio of ADM/ APB was 4.6, 6.3, 3.2 and 5.6 (t = 0.982, P = 0.329), respectively. The positive rate of RNS in the middle and middle-aged group was 57.5%,50% in the old group (Sup2 = 0.373, P = 0.541), and the positive rate of the RNS was 25% and 66.7% in the limb-onset subgroup (Sup2 = 9.881, P = 0.002). Conclusion:1. The positive rate of RNS in patients with ALS is high, as high as 50%, and there is no significant difference in MG patients, but the decrease is not high in MG patients, suggesting that the dysfunction of neuromuscular junction in ALS patients is more common, but the mechanism may be different from that of MG patients. The low-frequency RNS decrements greater than 20% should not be used as the ALS exclusion diagnostic criteria. In ALS, the lower the median nerve, the lower the amplitude of the ulnar nerve, the more serious the damage of the axonal, the more easily the lower-frequency RNS is more likely to occur, and the lower the low-frequency RNS.4. The RNS-positive often suggests that the rapid progression of ALS is an active manifestation of the disease. Can be used for prognosis judgment and follow-up observation. The rate of ADM/ APB was higher in the diagnosis of ALS, but there was no advantage in the follow-up.6. The results of the RNS and the age, sex, ALSFRS-r, and course of the patients were not significantly correlated.
【学位授予单位】：武汉大学
【学位级别】：博士
【学位授予年份】：2016
【分类号】：R744.8;R746.1

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