运动神经元病中神经电生理研究现状和进展

运动神经元病(motor neuron disorder,MND)是一组以上下运动神经元同时或分别受累为主要表现的神经系统变性病,目前尚无特效治疗方法,其中以肌萎缩侧索硬化(amyotrophic lateralsclerosis,ALS)最为常见,目前有关MND的电生理研究主要针对ALS。另外三型包括进行性肌萎缩(progressive muscular atrophy,PMA)、原发性     

运动神经元病的谷氨酸中毒学说

肌萎缩侧索硬化 (AmyotrophicLateralSclerosis,ALS)是选择性侵犯上下运动神经元的神经系统变性疾病 ,病因不明 ,目前尚无有效治疗。主要的发病机制假说之一为谷氨酸中毒学说。目前已经证实肌萎缩侧索硬化存在谷氨酸代谢、摄取异常 ,认为其选择性运动神经元损伤可能是通过Ca2 + -AMPA/Kanait受体介导的兴奋性中毒发生的 ,此外运动神经元保护机制异常可能也发挥了一定作用     

神经干细胞治疗运动神经元病的研究进展

运动神经元病（motor neuron disease，MND）包括肌萎缩侧索硬化（Amyotrophic lateral sclerosis，ALS）、原发性侧索硬化和进行性脊肌萎缩等，是运动神经元选择性变性导致进行性肌无力和死亡，目前尚无有效治疗方法。临床最常见的MND类型是ALS。对运动神经元损伤的细胞机制已经作了很多研究，但既然运动神经元已经变性死亡，一种可能的疗法就是用细胞移植的方法恢复其功能，其中包括基于人类神经于细胞的细胞替代治疗策略。     

肌萎缩侧索硬化26例临床分析

<正>肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是成年运动神经元病中最常见的形式,其发病机制不清楚,诊断仍处于临床水平,无特效治疗,存活期为3~5年,预后不良。本文对我科近几年收治的肌萎缩侧索硬化病例的临床特征进行分析如下:     

肌萎缩侧索硬化的药物治疗进展

肌萎缩侧索硬化(ALS)是一种运动神经元进行性变性疾病,目前缺乏特效治疗。现就ALS的抗兴奋性氨基酸毒性治疗、抗凋亡、神经保护、抗氧化、免疫调节等的现状和进展进行综述。     

肌萎缩侧索硬化患者呼吸功能不全的认识及对策进展

肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是上、下运动神经元均受损害的神经变性疾病,大多数ALS患者最终死于呼吸衰竭或肺部感染。本文对近年国内外ALS患者呼吸功能主要评估方法以及治疗呼吸功能障碍的相应对策(营养支持、气道管理、无创通气、机械通气、膈肌起搏和功能锻炼)进行综述,以便临床更好把握ALS患者呼吸功能情况,及时采取有效措施延缓呼吸功能恶化的进展。     

ALSFRS-R量表与AALS量表在评估肌萎缩侧索硬化不同治疗中的价值比较

目的 比较修订版肌萎缩侧索硬化功能评分量表(ALSFRS-R)和Appel肌萎缩侧索硬化评定量表(AALS)在肌萎缩侧索硬化(ALS)临床治疗中的应用价值差异。方法 回顾性分析94例ALS患者，依据不同治疗方案分为：利鲁唑组(31例)、依达拉奉组(32例)和对照组(未进行正规治疗的患者，31例)。收集患者的人口统计学及临床功能量表评分等资料。比较3组间临床资料，分析在不同药物治疗中两种量表的评分差异以及相关性。结果 采用标准化评分比较ALSFRS-R和AALS，结果提示基线两种量表评分呈显著负相关。ALS伦敦临床分期3期、4期患者中两种量表评分均呈显著负相关。利鲁唑组和依达拉奉组中两种量表评分反映的疾病严重程度在部分时间点上差异有显著性；对照组差异无显著性。3组的各时间点ALSFRS-R评分均较低。结论 ALSFRS-R与AALS在临床功能评估上差异有显著性，ALSFRS-R反映的疾病严重程度较轻。     

早期误诊为重症肌无力的肌萎缩侧索硬化1例报告并文献复习

肌萎缩侧索硬化( amyotrophic lateral sclerosis,ALS)是一种快速进展的神经退行性疾病,同时影响上、下运动神经元,并导致进行性肌肉萎缩和无力.ALS是一种高度异质性的疾病实体,上下运动神经元受累的程度在ALS中可变,其中进行性肌萎缩(progressive muscular atrophy...     

逆向轴突运输障碍与肌萎缩侧索硬化

<正>肌萎缩侧索硬化(ALS)是运动神经元变性疾病,特异性损害运动神经细胞。典型临床表现包括肌肉无力、强直以及萎缩,自然病程约2~5年。大部分患者为散发病例,10%的患者属于家族遗传性。迄今为止还没有治疗肌萎缩侧索硬化的有效方法。ALS具体发病机制尚不明确。神经营养因子等经逆向轴突运输由神经末梢输送到神经元胞体,维持神经细胞的存活及功能。在ALS患者及动物模型中都发现了逆向轴     

原发性侧索硬化2例报道及相关文献复习

目的 探讨原发性侧索硬化(PLS)的临床诊断、与经典的肌萎缩侧索硬化的关系、神经电生理特点、影像学特点及鉴别诊断.方法 分析我科收治的2例PLS患者的临床资料,并复习相关文献.结果 PLS是隐匿起病,进展缓慢的仅累及上运动神经元的神经退行性疾病,很多初诊为PLS的患者经过长期随访最后发展为肌萎缩侧索硬化,PLS的影像学表现多样,神经电生理检查容易早期发现下运动神经元损伤.结论 对于疑诊PLS的患者,明确是否具有局限性下运动神经元损伤症状十分重要,对患者进行时间的纵断随访对疾病最后的诊断和预后评估意义重大.     

Mental illness in England,in Scotland and in Scots living in England

Summary Beginning from the observation that Scots living in England have much higher rates of mental hospital admission than do the English, several hypotheses are proposed to account for this. Much of the excess in rates of mental illness is accounted for by those diagnosed as having alcohol-related disorders and behaviour and personality problems. The results of an examination of offical statistics in the two countries enabled some explanations to be offered. It was found that rates of admissions to mental hospitals are higher in Scotland than in England but not as high as those found among Scots migrants who have a much higher rate of readmission to hospitals than either of the other groups. In fact, if first admissions only are considered the rates of admission in Scotland are not only higher than rates for English natives but also higher than for Scottish migrants. It seems that Scots living in England are somewhat less likely to become mental patients than Scots in Scotland but that once they do achieve this status they are very much more likely to be readmitted on subsequent occasions. It was concluded that there might be two fairly distinct groups of migrants from Scotland to England who have different backgrounds, different reasons for migrating and different psychological characteristics. On the one hand there are stable, economically motivated migrants who move south for definite employment related reasons and who show few psychological symptoms. While on the other hand there is a group of migrants who perhaps have psychological problems and who move more in hope than expectation without definite prospects and who account for the high rates of mental hospital admission found in Scottish migrants.     

Prostacyclin release in the coronary circulation during sustained stimulation in in vitro and in in vivo experimental systems

Prostacyclin release from rat isolated perfused hearts and from dog coronary circulation was studied by measuring immunoreactive 6-keto-PGF1 alpha (6-keto-PGF1a) in heart perfusate and in plasma obtained from the great cardiac vein respectively. Continuous infusion of arachidonic acid at constant concentration in isolated perfused hearts induced an increased prostacyclin release. This release showed a rapid peak within 10 min and a subsequent decrease. Low-flow ischemia induced an increased perfusate concentration of 6-keto-PGF1a but, considering the decreased flow, prostacyclin release was actually reduced. During the whole period of ischemia (60 min) prostacyclin release was constant. In open-chest anesthetized dogs 6-keto-PGF1a concentration in the great cardiac vein was increased after ligation of the left anterior descending coronary artery. A prolonged period of coronary occlusion (4.5 hours) resulted in a progressive rise of prostacyclin release. 6-keto-PGF1a determinations in the femoral vein and in the aorta did not show relevant variations during the observation period.     

Neuroimaging in epilepsy in tropics

Epilepsy is a major public health problem in many tropical countries. Also, some of the tropical diseases are major contributors to the higher prevalence of epilepsy in these countries. The etiologic factors responsible for epilepsy in these countries are quite different from those in the developed world. This article discusses the etiologic factors and neuroimaging of epilepsy in light of the conditions in these tropical countries.     

癫痫与抑郁关系的研究进展

抑郁是癫痫患者中常见的精神障碍,严重地影响了患者的生活质量。传统的观点认为癫痫患者因为存在着诸多社会学问题易出现抑郁倾向,癫痫和抑郁是单向的联系,但大量的研究已经证明癫痫和抑郁之间存在双向的联系,一种异常状态的存在可能易转化为另一种异常状态的发展。癫痫和抑郁存在着共同的发病机制。本文主要就癫痫和抑郁的双向联系以及抗抑郁药物在癫痫患者中的应用进行阐述。