ALS功能评分和CMAP波幅对MND患者预后评估的研究

Prognostic significance of ALSFRS and compound muscle action potentials in patients with motor neuron disease

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）-[足母]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[足母]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[足母]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（Х^2=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ALSFRS与小指展肌及[足母]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 explore the correlation between ALSFRS and motor nerve conduction studies and their prognostic significance. Methods Forty MND patients with different levels of diagnostic certainty were enrolled, according to revised criteria of E1 Escoria; 102 healthy volunteers with age and gendermatched were served as controls. In all patients, ALSFRS was performed; and compound muscle action potential （CMAP） amplitude and distal motor latencies （DML） were measured using surface electrodes for stimula ting ulnar （at wrist）, tibial （at ankle） nerve and recording on abductor digiti minimi （ADM） and adductor hallucis （AH）, respectively; and correlation between ALSFRS and motor conduction was analyzed. Results （1） There were 20 patients diagnosed as definite, 10 probably, 4 possible ALS and 6 progressive spinal muscular atrophy. （2） Compared with the controls, the CMAP amplitude （mV） of both ADM [（10. 18 ± 1.99） v. s. （6. 80±4.06）; t =5.037, P=0.000] andAH [（11.73±2.77） v.s. （9. 86±5.39）; t =2.088, P =0.042] decreased significantly in the patient group; and DML of ulnar nerve prolonged significantly （t = -6. 426,P = 0. 000）. （3） ALSFRS correlated significantly with CMAP amplitude of ADM （r= 0. 653, P = 0. 000）, AH （r = 0. 446, P = 0. 004） and DML of ulnar nerve （r = - 0. 529, P = 0. 000）, respectively; and those with ALSFIRS 〈30 all had decreased CMAP amplitude in 9 （9/9, 100%） cases, whereas those with ALSFRS ≥30 had decreased CMAP amplitude in 17 （ 17/31,54. 8%） cases （Х^2 = 6. 25, P = 0. 012）. （4） In 8 followed-up patients, the ALSFRS were 28.75 ± 4. 10, among which there were 6 cases with ALSFRS 〈30; compared with the first investigation , both the ALSFRS （ t = - 6. 164 , P = 0. 000 ） and CMAP amplitude of ADM （ t = -5. 073,P = 0. 001） decreased significantly in the followed-up patients. The ALSFRS correlated positively with CMAP amplitude （mV） of both ADM/-（7. 05± 2. 88） ;r = 0. 836, P = 0. 010] and AH[（7. 58 ± 3. 23） ; r =0. 822,P = 0. 012], respectively, and there was no significant correlation of ALSFRS with DML of ulnar nerve （P〉0. 05）. Conclusions Decreased amplitude of CMAP and delayed DML may be present in MND patients; ALSFRS correlated significantly with CMAP amplitude, decrease of both could predict poor prognosis, which could be served as objective and reliable indication.