复元生肌颗粒改善肌萎缩侧索硬化症患者中医证侯临床疗效观察

目的 研究复元生肌颗粒治疗肌萎缩侧索硬化症(ALS)的临床疗效及其安全性.方法 对125例ALS患者进行随机、力如太对照研究.结果 复元生肌颗粒在改善痿病中医证侯量表的总分以及改善气短乏力、心烦、口渴咽干症状方面优于力如太(P<0.05).两组治疗前后血、尿常规、肝肾功能均无显著变化(P>0.05).两组均未发现药物相关性恶心、呕吐、胃纳减退、腹胀、腹泻等胃肠道症状和皮肤过敏.结论 复元生肌颗粒能延缓ALS患者的痿病中医证侯积分.     

复元生肌颗粒对肌萎缩侧索硬化症患者短期预后的疗效

目的 观察复元生肌颗粒治疗肌萎缩侧索硬化症(ALS)的临床疗效.方法 58例ALS患者分为治疗组和对照组,对照组予利鲁唑治疗,治疗组在利鲁唑的基础上加用复元生肌颗粒.分别于治疗前及治疗后2、4、6个月进行改良Norris评分评定,治疗前及治疗后6个月对ALS患者进行肺功能检查,并随访患者18个月.结果 两组患者在治疗第6个月时,改良N.rris评分变化值组间差异有统计学意义(P＜0.05),但用力肺活量(FvC)变化值的组间差异无统计学意义(P＞0.05).生存分析显示,在随访第18个月时治疗组与对照组累积生存率分别为80.0％和71.4％,差异无统计学意义(P＞0.05).结论 复元生肌颗粒在延缓ALS患者延髓及肢体功能进展方面可能有一定作用,但在改善患者肺功能、延长生存时间方面有待于进一步证实.     

复元生肌饮治疗肌萎缩侧索硬化症伴抑郁障碍的临床疗效

目的 探讨复元生肌饮治疗肌萎缩侧索硬化症伴抑郁障碍的临床疗效,并与西药利鲁唑(riluzole)进行对比.方法 选择肌萎缩侧索硬化症患者35例,分为中药组25例,西药组10例.分别服用复元生肌饮及利鲁唑,疗程为3个月.观察治疗前后两组患者的临床症状、ALS神经功能量表积分、中医症候评分、汉密尔顿抑郁量表评分,并对中药组患者的疗效与抑郁量表评分间作相关性分析.结果 临床观察及相关分析表明肌萎缩侧索硬化症患者疗效与抑郁量表评分间存在负相关性(P<0.05),复元生肌饮能改善ALS患者的临床症状,降低中医症候临床积分(P<0.05).结论 肌萎缩侧索硬化症患者抑郁障碍程度越严重,药物治疗效果越差,复元生肌饮是可以改善ALS患者临床症状的有效中药制剂.     

肌萎缩侧索硬化症治疗的研究进展

肌萎缩侧索硬化症 (ALS)的发病机制尚未完全明确 ,近期研究发现可能与神经营养因子的缺失和异常、兴奋性氨基酸毒性作用、氧化应激反应以及钙细胞毒作用等有关。本病目前尚缺乏有效的治疗方法。神经营养因子、兴奋性氨基酸拮抗剂、抗氧化剂、钙通道阻滞剂等可尝试用于ALS的治疗。     

肌萎缩侧索硬化症的治疗进展

自身骨髓衍化造血干细胞、KNS-760704和碳酸锂均能延迟肌萎缩侧索硬化症(ALS)患者的病情进展.在ALS的鼠实验模型中,锂盐+丙戊酸或利鲁唑+去氧苯丁酸钠治疗后也能延迟疾病的发生和发展.     

肌萎缩侧索硬化症药物治疗的研究进展

肌萎缩侧索硬化症(ALS)是一种运动神经元的进行性变性疾病。其发病机制未明,目前缺乏特效治疗。我们对近年来药物:抗兴奋性氨基酸毒性、神经营养因子、抗凋亡、抗炎性类药物等治疗ALS的研究进展进行了综述。     

肌萎缩侧索硬化症的干细胞治疗研究进展

干细胞替代治疗可能是一种能彻底治愈肌萎缩侧索硬化症的方法。体外培养的胚胎干细胞和神经干细胞能分化为具有电生理活性并能支配肌管的运动神经元。在运动神经元缺失的成年大鼠脊髓内,移植的神经干细胞能分化为运动神经元,并能支配骨骼肌及部分恢复实验动物的运动功能。另一些研究表明,干细胞可通过释放神经营养因子及重建运动神经元微环境等机制,保护濒死的运动神经元,从而延缓疾病进展。在目前看来,这种干细胞保护治疗更具实际意义。     

精神分裂症与肌萎缩性侧索硬化症的关系

何××(208145),男,46岁,未婚,工人,因近二月来行为紊乱,乱花钱,否认母亲是亲生而于87年3月16日第七次入院。总病程12年。患者系工人,原来工作一般。75年(29岁)时组织发现他劳动纪律变得松懈,常有迟到早退,即使人在车间也不能坚守岗位,以致无法完成生产任务。领导多次对他督促帮助,并不见效。至76年春,家人发现他对邻居一少女有单相思,扬言要结婚,乱花钱;后又经常到海员俱乐部及涉外宾馆等地找外国人要求帮助出国,为此于76年6月底首次收住精     

细胞移植治疗肌萎缩侧索硬化症进展

肌萎缩侧索硬化症是一种致死性神经退行性疾病,治疗相当棘手,近年来,研究的焦点集中在细胞替代疗法。本文描述了人类神经元样细胞、干细胞和分泌神经营养因子的转基因细胞移植治疗肌萎缩侧索硬化症的疗效和移植方法,认为改善神经功能的机制可能与细胞替代、重建神经环路和旁分泌神经营养作用等有关。     

转基因大鼠模型与肌萎缩侧索硬化症

随着转基因技术及基因定位技术的发展,对大鼠基因进行操作的方法也得到相应的改善,从而提高了制作有关人类遗传性疾病转基因动物模型的能力。这使我们更便于研究这些疾病的发病机制及筛选可能的治疗药物。本文介绍了转基因大鼠在肌萎缩侧索硬化症病因研究中的应用。     

肌萎缩侧索硬化的治疗进展

本文就肌萎缩侧索硬化的治疗进展,包括抗兴奋性氨基酸毒性治疗、免疫治疗、基因治疗及神经保护治疗的现状和进展作介绍。     

Treatment of Amyotrophic Lateral Sclerosis with Thyrotropin-Releasing Hormone (TRH)

Abstract: Despite the efforts of many workers, the cause and therapy has not been clarified. We carried out the therapeutic trial of thyrotropin releasing hormon (TRH) for amyotrophic lateral sclerosis (ALS) from January, 1979 to January, 1983. There were 16 subjects. The patients were given a low dose (0.5-2 mg) of TRH intravenously or intramusculary.
Mild to moderate improvement was found in 9 (56%) of 16 patients.
TRH has been reported to have the activating effects on the pyramidal tract, brainstem motor nuclei, and motoneuron in the spinal cord as a neurotransmitter or neuromodulator. We thought such action of TRH to be useful to the therapy of ALS.     

Psychiatric Aspects of Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is a progressive disorder characterized by degeneration of motor neurons. Given the severe nature of ALS, many believed that patients would suffer from a high level of depression and a low quality of life. However, research into the psychological health of patients with ALS has shown that this is not the case. This article reviews the state of current knowledge as it pertains to the psychological health of ALS patients in four broad areas: quality of life, personality characteristics, emotional reactions, and end-of-life choices.     

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Progressive weakness remains the clinical hallmark of amyotrophic lateral sclerosis (ALS). Accordingly, a variety of tools has been developed to capture this disease feature, including questionnaires, such as the ALS-functional rating scale, strength testing, pulmonary function tests, electrophysiologic measures, including motor unit number estimation, and imaging techniques. Despite this plethora of approaches, there is little agreement as to what measures to use in a given clinical trial or in the clinic during routine patient care. Part of the reason for this uncertainty is that ALS is a remarkably protean disease. Some individuals progress rapidly, others slowly; some patients have considerable upper motor neuron dysfunction, whereas others have little; and there is considerable variation in the sequence of body regions affected, in some the disease beginning in the bulbar musculature and in others in one arm or one leg. Here, I present a variety of basic and more complex clinical measures for potential use in therapeutic trials with the aim of offering a balanced and practical set of recommendations, as well as considerations for future studies.

Electronic supplementary material

The online version of this article (doi:10.1007/s13311-014-0331-9) contains supplementary material, which is available to authorized users.Key Words: Amyotrophic lateral sclerosis, functional measures, biomarkers, electrophysiology     

肌萎缩侧索硬化症的临床特点与起病部位的关系研究

目的:探索肌萎缩侧索硬化症(ALS)发病的危险因素与起病部位的关系,为该病的早期诊断提供新思路.方法:选取复旦大学附属华山医院神经内科2000至2010年出院诊断为ALS患者57例,回顾性分析患者的临床资料及相关危险因素.根据不同的起病部位分为延髓起病组、颈段起病组、腰段起病组.通过单因素/多因素回归方法分析比较各组间...