肌萎缩侧索硬化研究进展

<正>肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种主要累及大脑皮质、脑干和脊髓运动神经元的慢性致死性神经系统变性疾病,临床表现为骨骼肌无力和萎缩,进行性加重。其病因、发病机制均不明确,迄今为止还未发现特效治疗方法,患者平均生存期仅3～5 y。其中5%～10%为家族性ALS(fA LS),90%～95%为散发性ALS(sA LS)。本文综述ALS在临床表现及相关生物标记物等方面的发展历程,重点介绍ALS神经电生理及神经影像等技术的应用,利于临床     

肌萎缩侧索硬化发病机制的研究进展

肌萎缩侧索硬化(ALS)发现至今已有一百余年, 但其发病机制尚未阐明, 曾提出过多种学说. 近年来在基因遗传学和分子生物学方面的研究取得了长足的进步, 现已确定该病是一种基因病. 下面从基因遗传学、自由基、兴奋毒性、神经微丝等方面综述了与ALS发病机制有关的研究进展, 试图为其治疗提供依据.     

肌萎缩侧索硬化的研究进展

<正>肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)属于严重致死性神经系统变性疾病,目前还未有明确的发病机制,主要是由于运动神经病变导致,ALS在该类疾病中发病最为严重且发病率最高。1临床表现ALS大多数为获得性,少数为家族性。起病隐匿,发病年龄多在30～60岁之间。男性多于女性,5%的患者以躯干肌或呼吸肌无力起病~([1])。发病初期多表现为一侧或两侧手指灵活度下降、无力,慢慢手部小肌肉开始出现萎缩,蚓状肌、大小鱼际肌及骨间肌萎缩程度较重,从手部肌肉开始蔓     

天然抗氧化剂治疗肌萎缩侧索硬化的研究进展

肌萎缩侧索硬化是一种威胁生命的神经退行性疾病,导致运动神经元进行性变性,最终 导致死亡。在各种细胞培养和动物模型中已经证明,天然抗氧化剂能够通过减轻氧化应激和损伤来保 护神经元细胞。现总结天然抗氧化剂作用机制及在肌萎缩侧索硬化治疗相关研究中的意义。     

肌萎缩侧索硬化免疫学研究进展

肌萎缩侧索硬化免疫学研究进展李晓光郭玉璞肌萎缩侧索硬化（ＡＬＳ）是一种神经系统变性病，至今病因及发病机理尚不清楚。有许多证据说明本病的发病可能是多源性的或异质性的。已提出的病因涉及遗传因素、环境因素、病毒感染及免疫因素等。过去数十年临床及病理研究缺乏...     

肌萎缩侧索硬化实验模型及发病机制研究进展

肌萎缩侧索硬化（ALS）病因及发病机制的研究一直是神经科较棘手的问题。本文介绍最近对ALS研究较多的几种模型:免疫介导的动物模型、体外细胞培养模型及转基因动物模型,并介绍各自的优、缺点,适应研究的方向及由此而阐明的发病机制。为今后研究ALS的发病机制及治疗方案奠定基础。     

肌萎缩侧索硬化分裂手现象研究进展

<正>肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种以皮质脊髓束、皮质脑干束和脊髓运动神经元变性为特征的进行性神经退行性疾病~([1])。常表现为肌肉无力和萎缩,尤其是手部小肌肉。在ALS患者中,常优先累及手内肌的大鱼际肌肌群包括拇短展肌(Abductor pollicis brevis,APB)和第一骨间肌(first dorsal interosseous muscle,FDI),而包括小指展肌(Abductor digiti minimi,ADM)在内的小鱼际肌群则相对豁免,这一独特的手内肌分裂萎缩模式称为"分裂手"。近年     

肌萎缩侧索硬化的免疫学研究进展

肌萎缩侧索硬化（AmyotrophicLateralSclerosis,ALS）由Charot于1869年首先报道以来,至令其病因和发病机制尚不清楚。已提出的病因有遗传因素、环境因素、病毒感染及无疾因素等。近年来随着免疫学及分子生物学的迅速发展,ALS免疫学资料越来越多。现就本病的免疫学研究情况作一综述。     

肌萎缩侧索硬化的病毒病因学研究进展

运动神经元病(motor neuron disease,MND)是一组由于上、下运动神经元丢失导致延髓部、四肢、胸部肌肉逐渐无力和萎缩的进展性神经系统退行性疾病.MND多于45～60岁发病,发病率为1～2/100 000,患病率为4～6/100 000,生存期为1～5年~([1]).     

环境因素与肌萎缩侧索硬化相关性的研究进展

中枢 5- 羟色胺功能活性与情感障碍疾病密切相关,而响度依赖性听觉诱发电位可反映中枢 5- 羟色胺功能的活性,为情感障碍疾病的诊断和疗效预测提供新的方法和依据。现将响度依赖性听觉诱发电位及其在抑郁症及双相情感障碍中的应用进行综述。     

The relationship between amyotrophic lateral sclerosis and frontotemporal dementia

Despite the traditional view of amyotrophic lateral sclerosis (ALS) as an isolated motor neuron disorder, recent evidence suggests that ALS is, in fact, a multisystem disorder with a varying presentation and with widespread extramotor neuropathologic involvement. Support for a concept of ALS as a multisystem disorder has some basis in historical clinical reports that have highlighted the existence of a frank dementia in at least a small percentage of ALS patients. More recent evidence of extramotor involvement in ALS, derived from neurocognitive, neuropathologic, genetic, proteomic, and neuroradiologic perspectives, provides further support for these early observations and has drawn considerable attention to a possible association between ALS and frontotemporal dementia (FTD). Literature from these diverse clinical and basic scientific disciplines, when integrated, demonstrates commonalities between ALS and FTD and suggests that these disorders not only affect the same general neuroanatomic substrate, but also may represent two points on the same neuropathologic continuum. This review discusses this putative association between ALS and FTD and provides possible directions for future research in this area.     

The relationship between amyotrophic lateral sclerosis and frontotemporal dementia

Despite the traditional view of amyotrophic lateral sclerosis (ALS) as an isolated motor neuron disorder, recent evidence suggests that ALS is, in fact, a multisystem disorder with a varying presentation and with widespread extramotor neuropathologic involvement. Support for a concept of ALS as a multisystem disorder has some basis in historical clinical reports that have highlighted the existence of a frank dementia in at least a small percentage of ALS patients. More recent evidence of extramotor involvement in ALS, derived from neurocognitive, neuropathologic, genetic, proteomic, and neuroradiologic perspectives, provides further support for these early observations and has drawn considerable attention to a possible association between ALS and frontotemporal dementia (FTD). Literature from these diverse clinical and basic scientific disciplines, when integrated, demonstrates commonalities between ALS and FTD and suggests that these disorders not only affect the same general neuroanatomic substrate, but also may represent two points on the same neuropathologic continuum. This review discusses this putative association between ALS and FTD and provides possible directions for future research in this area.     

Neurotrophic factors and amyotrophic lateral sclerosis

The cause of motor neuron death in amyotrophic lateral sclerosis (ALS) remains a mystery. Initial implications of neurotrophic factor impairment involved in disease progression causing selective motor neuron death were brought forward in the late 1980s. These implications were based on several in vitro studies of motor neuron cultures in which a near to complete rescue of axotomized neonatal motor neurons in the presence of supplementary neurotrophic factors were revealed. These findings pawed the way for extensive investigations in experimental animal models of ALS. Neurotrophic factor administration in rodent ALS models demonstrated a remarkable effect on survival of degenerating motor neurons and rescue of axotomized motor neurons, both in vivo and in vitro. In the absence of efficient therapy for ALS, some of these promising neurotrophic factors have been administered to groups of ALS patients, as they appeared available for clinical trials. Up to date, none of tested factors has lived up to expectations, altering the outcome of the disease. This review summarizes current findings on neurotrophic factor expression in ALS tissue and these factors' potential/debatable clinical relevance to ALS and the treatment of ALS. It also discusses possible interventions improving clinical trial design to obtain efficacy of neurotrophic factor treatment in patients suffering from ALS.     

氧化应激在肌萎缩侧索硬化中的研究进展

肌萎缩侧索硬化是一种致命的神经退行性疾病,其特征在于脊髓、皮层和脑干运动神经元的进行性退行性改变,导致肌肉无力、肌萎缩和痉挛。目前肌萎缩侧索硬化具体的机制不明,近年来,氧化应激是相关研究热点。现对氧化应激机制与肌萎缩侧索硬化之间的关系进行综述。     

MRI在肌萎缩侧索硬化应用的研究进展

肌萎缩侧索硬化(ALS)是罕见的进行性神经系统变性疾病,上、下运动神经元同时受累是其主要病理特征,目前其诊断主要依靠肌电图及临床评估等,但均有局限性和主观性。近年来MRI在ALS中的应用受到研究者的关注,众多研究表明,MRI对ALS的早期诊断及病情评估均有一定帮助,MRI有可能在将来成为ALS诊断和治疗的重要辅助手段。     

肌萎缩侧索硬化的临床研究进展

肌萎缩侧索硬化(ALS)是一类累及上、下运动神经元的慢性变性疾病.本病于1869年由Charcot首次报道.作为运动神经元病(MND)的代表疾病,肌萎缩侧索硬化(ALS)为全球分布,该病的平均发病年龄在59岁,并非这一年龄组独有,低龄成人也可罹患此病,尤其是有肌萎缩侧索硬化家族史者[1].本病患者男性多于女性,比率约为3∶2,性别差异的原因不明[2].ALS的年发病率约为每1O万人口1～3人,患病率约为每1O万人口5～9人[3],是成年人最常见的导致瘫痪的疾病之一.从症状发作至死亡平均存活时间为3～5年,5年存活率约25% [4].     

The physician-patient relationship in amyotrophic lateral sclerosis

The principal models of the physician-patient relationship are analysed in terms of their historical development. An outline is given of the clinical, psychological and ethical particularities of the approach to patients with amyotrophic lateral sclerosis. The peculiarities of this disease are so exclusive that they do not resemble other progressive diseases with a negative prognosis, and therefore require an equally exclusive approach to the physician-patient relationship. This approach should not only be informative, scientific and interpretative-deliberative, but most simultaneously be founded on a solid therapeutic alliance aimed at seeking the best interests of the patients while respecting their autonomy as well as their “good” (not only in the sense of physical well-being, but also in terms of respect for their personal values). This is the only way to confront the conflicts that inevitably arise (especially in advanced stages of the disease) without the risks associated with a desire to escape or to adopt extreme solutions (such as euthanasia and therapeutic insistence) and without the risk of burn-out. Received: 5 May 2000 / Accepted in revised form: 6 December 2000