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目的探讨MND患者ALSFRS与运动传导改变的相关性及其对预后评估的价值。方法2007年8月~2008年7月间符合Escorial诊断标准不同确定性水平的MND患者40例,健康志愿者102名为对照组。对所有患者均进行ALSFRS,并分别测量尺神经(腕部)-小指展肌以及胫神经(踝部)-距母展肌的复合肌肉动作电位(CMAP)波幅和末端运动潜伏期(DML),分析ALSFRS与运动传导参数的关系。结果(1)肯定型ALS20例,拟诊型ALS10例,可能型ALS4例,进行性脊肌萎缩6例;(2)与对照组比较,患者组小指展肌和距母展肌CMAP波幅(mV)减低,尺神经DML延长(P<0.01);(3)ALSFRS与小指展肌和距母展肌CMAP波幅以及尺神经DML均呈显著相关(r分别为0.653,0.446和-0.592;P分别为0.000、0.004和0.000);ALSFRS<30分的9例(100%)患者CMAP波幅均异常减低,31例ALSFRS≥30分的患者有17例(54.8%)的CMAP波幅异常减低(χ2=6.25,P=0.012);(4)随访的8例患者中6例ALSFRS在30分以下;与首次就诊相比,随访的8例患者其ALSFRS以及小指展肌CMAP波幅均减低(P<0.05)。随访者的ALS-FRS与小指展肌及距母展肌CMAP波幅均呈正相关(r分别为0.836和0.822;P分别为0.01和0.012),与DML无相关(P>0.05)。结论MND患者可出现CMAP波幅减低及DML延长;ALSFRS与CMAP波幅显著相关,二者同时减低提示预后差,可作为客观反映MND患者严重程度的可靠指标。
Objective To investigate the correlation between ALSFRS and motor conduction in patients with MND and its value in prognosis evaluation. Methods From August 2007 to July 2008, 40 patients with MND with different definite levels of Escorial diagnostic criteria and 102 healthy volunteers as control group. ALSFRS was performed in all patients and the composite muscle action potential (CMAP) amplitude and terminal locomotor latency (DML) of ulnar nerve (wrist) - little finger abductor muscle and tibial nerve (ankle) - maternal muscle were measured, ALSFRS and exercise conduction parameters. Results (1) 20 cases of positive ALS, 10 cases of suspected ALS, 4 cases of possible ALS and 6 cases of progressive spinal muscle atrophy. (2) Compared with the control group, (P <0.01). (3) There was a significant correlation between ALSFRS amplitude and CMAP amplitude and dural nerve DML (r = 0.653,0.446 and -0.592, P = 0.000, 0.004 and 0.000). The amplitude of CMAP in all 9 patients (100%) with ALSFRS <30 was abnormally decreased. The abnormal amplitude of CMAP in 17 patients (54.8%) was decreased in 31 patients with ALSFRS≥30 (χ2 = 6.25, P = 0.012). (4) Among the 8 follow-up patients, 6 patients had ALSFRS less than 30 points. Compared with the first visit, the amplitude of ALSFRS and amplitude of CMAP of the abductorum showed a decrease in all 8 patients (P <0.05). There was a positive correlation between the ALS-FRS of the responders and the amplitude of the CMAP amplitude of the little finger and the maternity muscle (r = 0.836 and 0.822, respectively; P = 0.01 and 0.012, respectively), but no correlation with DML (P> 0.05). Conclusions The decrease of CMAP amplitude and prolongation of DML may occur in patients with MND. The amplitude of ALSFRS is significantly correlated with the amplitude of CMAP. The simultaneous decrease of both may indicate the poor prognosis, which can be used as a reliable indicator to objectively reflect the severity of MND.