肌萎缩侧索硬化症的诊断及病因分析(附238例病例报告)

目的探讨肌萎缩侧索硬化症(ALS)的诊断和发病机制.方法回顾性分析238例肌萎缩侧索硬化症的一般状况、临床表现、既往史、家族史以及实验室检查.结果本组患者均出现上下运动神经元损害的表现,肯定ALS为143例,拟诊ALS81例,可能ALS为14例.结论ALS的病因尚未明确.临床症状、体征以及肌电图是诊断的依据.     

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.     

家族性ALS的SOD1基因突变及发病机制研究进展

肌萎缩侧索硬化症(ALS)是运动神经元病的最常见类型。部分家族性肌萎缩侧索硬化症(familial ALS,FALS)与超氧化物歧化酶(SODl)基因突变相关。本文介绍人的正常 SODl 和突变 SODl 基因及其表达蛋白的特点,并简述人突变 SODl 的转基因模型,也对 FALS 中突变 SODI 的致病机制进行了总结和探讨,为 ALS 的研究和治疗策略提供新的思路。     

复元生肌颗粒治疗肌萎缩侧索硬化症的临床研究

目的观察复元生肌颗粒治疗肌萎缩侧索硬化症的临床疗效.方法临床上选择肌萎缩侧索硬化症患者35例,分为治疗组25例,对照组10例.分别服用复元生肌颗粒及力如太(riluzole)治疗,疗程为3个月.观察治疗前后患者的临床症状、ALS神经功能量表积分、中医症候评分,并进行肌电图检查,测定正中神经复合肌肉动作电位(CMAP)波幅大小.结果临床观察表明复元生肌颗粒能改善ALS患者的临床症状,降低中医症候临床积分(P＜0.05).两组ALS功能量表评分及正中神经复合肌肉动作电位波幅变化无统计学差异(P＞0.05).结论临床观察表明复元生肌颗粒是治疗ALS的有效中药制剂.     

功能锻炼配合辨证施护对肌萎缩性侧索硬化症患者身心状况的影响

目的 探讨功能锻炼配合辨证施护对肌萎缩性侧索硬化症患者身心状况的影响.方法 通过类实验性研究对52例肌萎缩性侧索硬化症患者进行问卷调查并给予功能锻炼配合辨证施护.结果 功能锻炼配合辨证施护改善了肌萎缩性侧索硬化症患者的临床症状,降低了患者心理抑郁水平.结论 科学的功能锻炼配合辨证施护对肌萎缩性侧索硬化症患者是可行和必要的.     

国内外肌萎缩性侧索硬化症发病相关蛋白研究进展

<正>肌萎缩性侧索硬化症(amyotrophic lateral sclerosis,ALS)是一种选择性侵犯脑与脊髓的上、下运动神经元的神经系统变性疾病,临床特点为全身进行性肌无力、肌肉萎缩、肌束震颤,多数患者将在出现临床症状后3⁵ y内死于呼吸肌麻痹。5%5 y内死于呼吸肌麻痹。5%¹0%肌萎缩侧索硬化症病例为家族性肌萎缩侧索硬化(FALS),FALS表现为常染色体显性遗传或常染色体隐     

肌萎缩侧索硬化9型个案报道及文献复习

<正>肌萎缩侧索硬化症(amyotrophic lateral sclerosis, ALS)是一种临床较为罕见的中枢神经系统疾病,主要表现为全身骨骼肌的进行性萎缩、无力、瘫痪^[1],因累及的神经元部位不同,临床表型也有所不同。近期我院收治了1例临床表现较为复杂,最后经基因检测证实为ALS9型患者,现将其诊治过程报道如下。1 临床资料患者,男,64岁。广州市退休国企干部,因“饮水呛咳并全身肌肉进行性萎缩3 m余”     

肌萎缩侧索硬化症的病因学与实验模型研究进展

肌萎缩侧索硬化症(amyotrophic lateral sclerosis, ALS)与脊髓性肌萎缩、进行性球麻痹和原发性侧索硬化症同属运动神经元病.其病变主要侵犯脊髓前角细胞、脑干运动神经核及锥体束,临床特征表现为上、下运动神经元同时受损[1].其病因复杂,治疗困难,为神经系统疾病研究热点之一,本文就ALS病因学与实验模型研究进展做一介绍.     

利美尼定促进突变SOD1蛋白质降解的作用研究

目的 利美尼定对家族性肌萎缩侧索硬化症(ALS)相关突变蛋白质SOD1G93A的作用机制.方法 用瞬时转染的方法在运动神经元样细胞系NSC-34中过表达WTSOD1,与家族型ALS相关的SOD1G93A突变蛋白,再给予自噬通路的特异性诱导剂和阻断剂,通过Western blot蛋白印迹法检测突变SOD1、自噬标记物的蛋...     

肌萎缩性侧索硬化与自身免疫

肌萎缩性侧索硬化症 (amyotrophiclateralsclerosis ,ALS)的病因尚未明了。ALS患者伴发免疫相关疾病或淋巴增殖性疾病的频率较正常人群高 ,免疫组化方法发现脊髓组织存在免疫反应 ,血清中发现抗GM 1、抗钙离子通道、抗Fas等抗体 ,以及自身免疫动物模型的建立 ,为ALS发病的自身免疫机制假说提供了一定依据     

Denervation study of synapses of organ of corti of old world monkeys

Fine structural characteristics of synapses in the spiral organ of Corti were examined, with reference to differences between inner and outer haircell systems, and to location of neurons of origin of efferent axons. Surgical interruption of crossed olivocochlear bundle, of vestibular nerve, of facial nerve, and excision of superior cervical ganglia were used to determine the pathways of efferent axons. Interruption of the vestibular nerve near the brainstem results in degeneration of all efferent terminals on outer hair cells. Mid-line lesions at, and caudal to, the facial colliculus result in degeneration of about half of these efferent terminals. Efferent synaptic bulbs to the inner hair-cell system are small, of the order of one micron, and form type 2 junctions with afferent dendrites. They tend to have more large dense-core vesicles (about 80 nm) than the large efferent terminals of the outer hair-cell system, and appear to be the terminals of axons in the habenula perforata, which exhibit varicosities laden with large dense core vesicles. The varicosities are unaffected by excision of the superior cervical ganglia. So far as our material can reveal, it appears that the varicosities in the habenula perforata do not survive vestibular root interruption, nor do the efferent processes in the internal spiral bundle or at the base of inner hair cells. Most interestingly, the afferent processes of the inner hair-cell system, as identified for example by their relation to pre-synaptic bodies in the inner hair cells, are subject to a trans-synaptic reaction after severance of the vestibular root. They undergo a dramatic cytological transformation, characterized by increase of volume, engorgement with microtubules, microfilaments, microvesicles of various sizes, and clusters of lysosomes. Thus, both the efferent and afferent terminals of the inner hair-cell system show marked cytological differences from the corresponding terminals of the outer hair cell system.     

Mechanism of action of tubocurarine on nicotinic cholinoreceptors of neurons of the sympathetic ganglia of rats

Tubocurarine (Tc) effect on membrane currents elicited by acetylcholine (ACh) was studied in isolated superior cervical ganglion neurons of rat using patch-clamp method in the whole-cell recording mode. The "use-dependent" block of ACh current by Tc was revealed in the experiments with ACh applications, indicating that Tc blocked the channels opened by ACh. Mean lifetime of Tc-open channel complex, tau, was found to be 9.8 +/- 0.5 s (n = 7) at -50 mV and 20-24 degrees C. tau exponentially increased with membrane hyperpolarization (e-fold change in tau corresponded to the membrane potential shift by 61 mV). Inhibition of the ACh-induced current by Tc (3-30 microM/1) was completely abolished by membrane depolarization to the level of 80-100 mV. Inhibition of ACh-induced current was augmented at increased ACh doses. It is concluded that the open channel block produced by Tc is likely to be the only mechanism for Tc action on nicotinic acetylcholine receptors in superior cervical ganglion neurons of rat.     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

Limits of deinstitutionalization--perspectives of relatives of psychiatric patients

After a hopeful beginning, the social process of the reintegration of those with severe mental illness has come to a standstill. I am led to wonder whether "the community" really wants to live together with people suffering from severe mental illness, and if so, how closely? As long as the medical treatment of mental illness provided by the general practitioners is fundamentally deficient, as they are not able to prescribe the necessary interventions--such as out-patient psychiatric nursing, and service providers in the out-patient sector are content with offering increasingly intensive forms of care for the less seriously ill at the cost of the Social Welfare System--the reintegration of those with serious mental illness remains an illusion--which is mainly to the benefit of providers of residential care in homes and hostels.