观察中西医结合创新疗法治疗运动神经元病(MND)患者的疗效。

运动神经元病属中医"痿证"范畴。本研究在常规综合治疗基础上，采用一种用于促进神经损伤修复的药物组合物[精氨酸1.5～5 g，异亮氨酸1.5～5 g，亮氨酸2.5～7.5 g，赖氨酸1.5～5 g，蛋氨酸0.25～1.5 g，苯丙氨酸0.25～1.5 g，苏氨酸1.5～5 g，色氨酸0.25～1.5 g，缬氨酸2.5～7.5 g，组氨酸1.5～4 g，甘氨酸1.5～4 g，丙氨酸1.5～5 g，脯氨酸1.5～4 g，天冬酰胺0.05～1.5 g，半胱氨酸0.05～1.5 g，谷氨酸1.5～5 g，丝氨酸0.25～2.5 g，酪氨酸0.05～1.5 g，L～鸟氨酸0.25～4 g，天冬氨酸0.5～2.5 g；维生素(维生素B₁ 1～2 mg，维生素B₂ 1～2 mg，维生素B₆ 3～10 g，维生素C 1～3 g)]及中药"滋痿膏"。对2015年4月至2018年3月江苏大学附属武进医院收治的286例MND患者进行治疗，其中男性199例，女性87例；年龄26～81岁，平均年龄(53.7±10.7)岁。用法：将上述药物组合物配制在1个三升输液袋内进行静脉输注，1次/d，28 d为1个疗程，停药2周后行下一疗程，连续治疗≥1个疗程并持续随访至首次用药后第6个月。同时，在治疗期间每天于饭后2 h口服中药"滋痿膏"，2次/d。观察治疗前后患者的肌萎缩侧索硬化症改良功能评分量表(ALSFRS-R评分)、疾病进展率及临床表现等变化情况。

286例患者中死亡16例(5.6%)，死亡原因均为疾病晚期呼吸衰竭。国际ALSFRS-R评分上升占20.3%(58/286)，评分维持不变占15.0%(43/286)。仍有64.7%(185/286)的患者在经过治疗后评分持续下降，但评分下降的速度较治疗前有所减缓。治疗后，MND患者疾病进展率[0.583(0.478)分/月]低于治疗前[0.600(0.533)分/月]，差异有统计学意义(Z＝-2.088，P<0.05)，且174例(60.8%)患者的疾病进展率在治疗后较前下降。临床症状改善情况：肌肉萎缩好转、肌力增加12例(4.2%)，舌肌萎缩、言语不清好转6例(2.1%)，流涎好转9例(3.1%)，吞咽功能改善15例(5.2%)，四肢活动度增加11例(3.8%)，3例(1.0%)不能站立或行走的患者恢复部分行走能力，呼吸困难好转6例(2.1%)。

本疗法一方面通过滋痿膏起到补益脾肾、扶元起痿、益气温阳、补肾壮骨、疏肝理气、养荣生肌、扶正祛邪、强骨滋痿、双向免疫调节的作用；另一方面，通过疗法中所提供的各种氨基酸与维生素B₆等，能够为患者提供充足的代谢底物及强劲的动能，促进机体酶代谢、利尿与解毒，达到保护大脑及神经系统功能、改善肝功能、提高机体免疫功能的功效。两者相辅相成，使损伤的神经细胞在一定程度上得到修复。该中西医结合创新疗法疗效确切，且无毒、无害、无副作用、重复性好。

To observe therapeutic efficacy of integrated traditional Chinese and Western medicine for motor neuron disease (MND).

Motor neuron disease belongs to an impotence syndrome according to traditional Chinese medicine. In this study, a pharmaceutical composition for promoting nerve damage repair and nourishing flaccidity extract included the following main components: arginine (1.5-5 g), isoleucine (1.5-5 g), lysine (2.5-7.5 g), methionine (0.5-1.5 g), phenylalanine (0.5-5 g), tryptophan (0.5-1.5 g), valine (2.5-7.5 g), histidine (1.5-4 g), glycine (1.5-4 g), alanine (1.5-5 g), proline (1.5-4 g), asparagine (0.05-1.5 g), cysteine (0.05-1.5 g), cysteine (1.5-5 g), serine (0.25-2.5 g), tyrosine (0.05-1.5 g), L-ornithine (0.25-4 g), aspartic acid (0.5-2.5 g) and vitamins (vitamin B₁ 1-2 mg, vitamin B₂ 1-2 mg, vitamin B₆ 3-10 g and vitamin C 1-3 g)on the basis of conventional comprehensive treatment. 286 MND patients were treated in Wujin Hospital from April 2015 to March 2018. 199 cases were males and 87 cases were females, aged from 26 to 81 years, with the average age (53.7±10.7 years). The above composition was prepared in a three-liter infusion bag for intravenous infusion, once a day for 28 days for a course of treatment. The next course should be followed after drug withdrawal for 2 weeks and the treatment lasted at least for 1 course, followed by a continuous follow-up until the 6th month after the first dose. At the same time, nourishing flaccidity extract of traditional Chinese medicine was orally administrated 2 h after meals twice a day. The neurological functional evaluation was done with amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R), disease progression rate and clinical manifestations in all patients pre- and post-treatment.

After treatment, 16 patients (5.6%) died from advanced respiratory failure in the terminal phase of disease. Patients with increased scores accounted for 20.3%(58/286)and those with remained unchanged for 15.0% (43/286) according to International ALSFRS-R scores. There were still 64.7% (185/286) of patients whose scores continued to decline after treatment, but the rate of decline was slower than those before treatment. The disease progression rate after treatment [0.583 (0.478) point/month] was lower than that before treatment [0.600 (0.533) point/month], the difference was statistically significant (Z=-2.088, P<0.05), and the disease progression rate of 174 patients (60.8%) decreased after treatment. Symptoms of muscular atrophy and muscle strength were improved in 12 cases (4.2%); that of atrophy of tongue muscle and speech inarticulate were improved in 6 cases (2.1%); that of salivation were improved in 9 cases (3.1%); swallowing function was improved in 15 cases (5.2%); 11 patient (3.8%)increased limb mobility; 3 patients (1.0%) who could not stand or walk restored partial walking ability; Six cases (2.1%) showed improvement in dyspnea.

On the one hand, the therapy plays a role of reinforcing spleen and kidney functions, Fuyuan Qiwei, Yiqi Wenyang, Bushen Zhuanggu, Shugan Liqi, Yangrong Shengji , Fuzheng Quxie, and Qianggu Ziwei, with dual immunomodulatory effects. On the other hand, a variety of amino acid and vitamin B₆ can provide patients with adequate metabolic substrate and strong energy and promote the body's enzyme metabolism, diuresis and detoxification. With this therapy, the damaged nerve cells are repaired to some extent, the nervous system functions are protected, and the liver and immune functions are improved. The combination of traditional Chinese medicine and Western medicine is effective, non-toxic and harmless, with no side effects and good repeatability.