帕金森综合征中的肌张力障碍

<正>锥体外系疾患所致运动障碍(movement disorders)可概括为肌张力增高-运动减少症候群和肌张力减低-运动增多症候群两大类属性,前者临床表现以运动迟缓、肌肉僵直等为特征,代表疾病为帕金森病(Parkinson's disease,PD),而后者临床表现以舞蹈/投掷症、手足徐动症等为特征,代表疾病为亨廷顿病(Huntington's disease,HD)。肌张力障碍(dysto-     

壳核-内囊-尾状核的血管性病变所致的肌张力异常

肌张力异常代表累及运动系统的一组特殊疾病.Denny Brown确认是"一种固定或相对固定的形式,伴有其他锥体外系运动障碍."张力异常的肌肉畸变为其主要表现,因缺乏特有的病理学特征,致使任何病理生理的解释都很困难.然而,除这种     

重视肌张力障碍的诊断和治疗

肌张力障碍(dystonia)是一类以主动肌和拮抗肌不协调、持续收缩而引起的重复扭曲运动和(或)面部、颈部、躯干、肢体的姿势异常为特征的运动障碍疾病.肌张力障碍临床表现多样,当收缩时间短时,肌张力障碍性运动可表现很快,类似肌阵挛;当运动具有节律时,可表现为肌张力障碍性震颤,此外亦可在不同的活动或姿势时发生不同的改变,并可以是刻板性运动.     

应重视帕金森病的诊断与治疗

运动障碍性疾病(movement disorders)是一组以随意运动迟缓、不自主运动、肌张力异常、姿势步态障碍等运动症状为主要表现的神经变性疾病、主要分为肌张力增高运动减少[帕金森病(PD)]和肌张力降低-运动过多[亨廷顿病(HD)]两大症候群。随着人口老龄化,帕金森病作为典型的运动障碍性疾病,其发病率和患病率在全球尤其是我国老年人群中呈现明显增长趋势。该病是由于脑深部黑质     

应重视帕金森病的诊断与治疗

运动障碍性疾病（movement disorders）是一组以随意运动迟缓、不自主运动、肌张力异常、姿势步态障碍等运动症状为主要表现的神经变性疾病。主要分为肌张力增高．运动减少[帕金森病（PD）]和肌张力降低一运动过多[亨廷顿病（HD）]两大症候群。     

运动障碍疾病的外科治疗

<正> 运动障碍疾病又称锥体外系疾病,是基底节病变或功能异常导致的随意运动调节功能障碍,可表现为运动减少、运动增多和混合性运动障碍等症状。锥体外系疾病是神经系统常见病,如帕金森病(PD)、肌张力障碍、亨廷顿舞蹈病、抽动症等。随着分子生物学、功能影像技术、神经药理学等研究进展,肉毒毒素局部注射、立体定向毁损术、脑深部电刺激术(DBS)、干细胞移植和基因技术等方法在运动障碍疾病的诊断及治疗方面的研究取得较快发展。现主要对运动障碍疾病的外科治疗进行介绍。     

氨磺必利致迟发性肌张力障碍之动眼危象1例

<正>迟发性肌张力障碍(tardive dystonia,TDt)是由抗精神病药物所致的一组慢性持久的锥体外系症状,主要表现为单个或多个随意肌自主运动困难或因自主运动困难所致姿势异常。TDt可累及身体的任何部位,如颈部肌肉向各个方向扭转,睑痉挛,口周及下颌肌群、喉部、上肢、躯干及下肢肌张力障碍,以及动眼危象~[1]。一般迟发性肌张力障碍多     

扭转性痉挛和神经干细胞治疗

扭转性痉挛(torsion spasm,TS)又名变形性肌张力障碍、扭转性肌张力障碍或豆状核性肌张力障碍,主要表现为主动肌与拮抗肌不自主、反复发生的异常同步收缩,导致受累部分的肢体出现扭转运动或异常姿势。肢体近端症状重于远端,紧张时加重。TS的症状可局限于身体某一局部,也可累及多个部位或半侧躯体,甚至波及全身。本文就TS干细胞治疗情况汇报如下。     

脑深部电刺激术治疗Hallervorden-Spatz病

Hallervorden-Spatz病(HSD)是一种少见的常染色体隐性遗传病,可有散发[1],是铁盐在苍白球、黑质沉积所引起的一种变性性疾病.临床主要表现为锥体外系受累,起始症状为姿势异常、肌张力增高、步行困难和不自主运动(多为舞蹈、手足徐动或震颤).晚期则出现构音障碍、吞咽困难、锥体束征和智能障碍,症状进行性加重,多数患者因严重的并发症而死亡.     

发作性运动障碍的临床特征及发病机制

目的 探讨发作性运动障碍（PMD）的临床特征及发病机制。方法 回顾性分析5例发作性运动诱发舞蹈手足徐动症（PKC）和2例发作性持续运动诱发肌张力障碍（PED）患者的临床资料。结果 5例PKC发作均南突然运动诱发,表现肌肉僵直、肌张力增高3例,表现肢体扭动、肌张力不全3例（其中1例先为肢体僵直后扩展为周身扭动）。2例PED由持续运动诱发,表现为肢体不自主运动,持续数秒至数分钟缓解。脑电图（EEG）或动态脑电图（AEEG）示痫样放电5例,头部CT或MRI检查正常5例,异常2例。4例PKC予卡马西平治疗有效,1例PED予较大剂晕丙戊酸钠有效。结论PMD表现为发作性锥体外系症状,多由突然运动诱发。大部分病例的EEG有痢样放电,抗癫痫药物治疗有效。提示PMD的发病机制可能与癫痫类似或相同。     

Alzheimer disease and the role of free radicals in the pathogenesis of the disease

Oxidative stress occurs early in the progression of Alzheimer disease, significantly before the development of the pathologic hallmarks, neurofibrillary tangles and senile plaques. All classes of macromolecules (sugar, lipids, proteins, and nucleic acids) are affected by oxidative stress leading, inevitably, to neuronal dysfunction. Extensive data from the literature support the notion that mitochondrial and metal abnormalities are key sources of oxidative stress in Alzheimer disease. Furthermore, it has been suggested that in the initial stages of the development of Alzheimer disease, amyloid-beta deposition and hyperphosphorylated tau function as compensatory responses to ensure that neuronal cells do not succumb to oxidative damage. However, during the progression of the disease, the antioxidant activity of both agents is either overwhelmed or, according to others, evolves into pro-oxidant activity resulting in the exacerbation of reactive species production.     

Craniocervical large-artery occlusive disease in the spectrum of ischemic cerebrovascular disease

Atherosclerotic occlusive disease of the craniocervical arteries has particular importance within the overall spectrum of cerebrovascular disease, particularly as a predictor for the presence of systemic atherosclerosis. Patients with symptomatic stenosis or occlusion of these arteries, or those with “large-artery” ischemic stroke (IS), have specific clinical features. They may have (1) a risk factor profile that differs from other IS subtypes, (2) an increased association with cardiac and other systemic atherosclerosis, and (3) an increased risk of early stroke recurrence, and they may benefit from revascularization therapies in addition to medical management strategies.     

Coexistence of Creutzfeldt-Jakob disease and Alzheimer's disease in the same patient

We report the case of a 73-year-old patient in whom a diagnosis of Creutzfeldt-Jakob disease, suggested by the clinical course, was verified by the neuropathologic finding of widespread spongiform change and astrogliosis, the presence of proteinase-resistant protein in brain extracts, and the experimental transmission of spongiform encephalopathy to primates inoculated with brain tissue. However, neuropathologic examination also revealed a profusion of senile and neuritic plaques and neurofibrillary tangles that reacted with antibody to the amyloid beta-protein characteristic of Alzheimer's disease, but not with antibody to the scrapie amyloid protein characteristic of Creutzfeldt-Jakob disease.     

Parameters of cholinergic neurotransmission in the thalamus in Parkinson's disease and Alzheimer's disease

Loss of cholinergic cells in the basal forebrain is associated with commensurate reductions in cortical acetylcholine-related enzyme activities in both Alzheimer's disease (AD) and Parkinson's disease (PD). Nerve cell loss from the cholinergic pontine tegmental nuclei also occurs. As the latter nuclei project to the diencephalon, we used frozen tissue from 5 controls, 5 PD and 5 AD cases to study the distribution of ChAT, AChE and [3H]nicotine binding in the thalamus and subthalamic nucleus. The anterior nuclear group and the mediodorsal nucleus showed high activities of ChAT and AChE together with relatively high levels of [3H]nicotine binding. The centromedian nucleus and subthalamic nucleus contained equally high levels of ChAT but negligible levels of [3H]nicotine binding. There were no significant changes in the levels of ChAT, AChE and nicotine binding in the PD and AD groups indicating that involvement of the pedunculopontine tegmental nucleus is likely to be a secondary retrograde phenomenon rather than part of a systematic cholinergic fibre degeneration.     

Coping with the disease and disease course in schizophrenic psychoses

The influence of the patients' attitude toward their psychosis in relation to the outcome variable "risk of relapse" was investigated (schizophrenics n = 38, ICD and AMDP-selection). The aspect of coping with the disease was operationalized by 11 self-rating items divided into 3 categories by using a (nonmetric) multidimensional scaling: active coping style, passive coping style and self-centeredness. Statistical analysis (Kruskal-Wallis- and Mann-Whitney U-tests) showed a significant difference inpatient versus outpatients in the categorie "active coping style". The significantly higher "active coping style"-score in the inpatient group could be explained by the relief of everyday problems by multiprofessional health teams. Finally, a stepwise logistic regression analysis showed a main impact of coping style on the outcome of psychosis, although the statistical model could be optimated only by including the interaction of the factors "vocational training" and "length of illness". Insofar, the results of the study represent a confirmation of the previous findings in coping research indicating that coping style and strategies are a relevant predictor of the outcome in chronic and impairing diseases.     

Burden of disease related to Parkinson's disease in Spain in the year 2000.

We measured the burden caused by Parkinson's disease (PD) in Spain during the year 2000 and compared it against PD burden worldwide and in the European A subregion. Burden of disease (BoD) is an important factor in health policy. Disability-adjusted life years (DALY) as a measure of BoD is the result of adding years of life lost (YLL) and years lived with disability (YLD). The burden of PD (BPD) has not been studied in Spain. YLL were obtained from the Spanish death certificates and YLD from the estimated number of incident PD cases and the average PD duration. PD disability was calculated, using the Disability Weights for Diseases in the Netherlands. Prior PD DALY data for Europe and the world were obtained from the 2001 World Health Organization World Health Report. A discount rate of 3% and age-weighting modulation factor with K = 1 were used. In Spain, PD generated 67,582 DALY, comprising 6,351 (9.4%) YLL and 61,231 (90.6%) YLD. Most PD DALY (57.5%) occurred in the population 60 to 74 years of age. When PD DALY estimates were adjusted using the world population in 2000, Spain registered a PD DALY rate of 84 per 100,000 population, higher than both the world and European A subregion rates (24 and 35 per 100,000 population, respectively). PD burden in Spain in 2000 was high, with disability being the major contributing factor. Although BPD in Spain was greater than both world and European A subregion BPD, these differences should nevertheless be interpreted with caution.