目的 探讨直立性低血压对不同亚型多系统萎缩(MSA)患者认知功能的影响.方法 评估MSA患者两种亚型及每种亚型患者伴或不伴直立性低血压(OH)的认知障碍特点.研究纳入神经内科的43例MSA患者.收集患者临床数据、运动及非运动症状量表评估,予以全面性精神认知量表测评,同时将认知水平细分为7个认知域.结果 MSA小脑型(MSA-C)和帕金森病型(MSA-P)两组临床数据、认知水平和认知域得分方面均未见明显差异(P>0.05).而在MSA-C中按伴或不伴OH分组后,发现MSA-C-OH组蒙特利尔认知功能评估(MoCA)量表分数明显更低(P<0.045),相比MSA-C-OH(-)组,MSA-C-OH组在语言和相似性方面的得分显著下降(P<0.05).该现象在MSA-P患者中并没有被发现.结论 MSA患者可伴有认知障碍,这可能与OH的严重程度相关,MSA-C患者中,OH对认知障碍的影响更凸显,可能是一个风险因素,而MSA-C-OH患者主要损害在语言和相似性能力方面,提示OH可能参与了MSA-C患者额叶皮层的损害,从而加剧了额叶相关认知障碍的发生发展.

目的 帕金森病(Parkinson disease,PD)认知障碍患者的重要危险因素之一就是以自主神经功能失调为病理机制的体位性低血压(Oorthostatic hypotension,OH).但是,不同认知水平与神经源性OH在PD患者中的直接关系目前尚不清楚.本研究旨在PD患者中伴或不伴神经源性OH患者与认知障碍的关系,同时观察在不同认知域的评分上,有症状的和无症状的PD合并神经源性OH患者是否存在差异.方法 回顾性收集来自首都医科大学宣武医院神经内科病房的79 例PD患者,其中19 例有症状的OH患者,20 例无症状的OH患者,以及40 例不伴有OH的患者.所有患者完善逐波血压及激活站立实验去评估有无OH,同时应用OH问卷去鉴别有症状的和无症状的OH患者.应用全面的神经心理量表去评价认知功能.应用单因素方差分析及Kruskal-Wallis检验来确定OH与认知功能障碍的关系,以及不同认知域在不同类别患者中的特征.结果 在所有类型的PD患者中受教育年限与认知水平呈正相关.有症状的OH患者更容易在早期出现较差的词语延迟记忆功能.不伴有OH的PD痴呆更容易损害视空间/执行功能.结论 我们的发现为PD认知障碍患者提供了一个新的视角,有症状的OH患者更容易累及记忆功能,这可能和OH潜在的病理生理机制相关.

老年性聋大多表现为双侧、进行性、对称的感音神经性听力下降，该病以高频听力减退、言语识别率下降为主要特征。老年性聋的病变部位包括外周听觉系统特别是耳蜗以及听觉中枢的退行性改变。老年性聋表现出中枢处理听觉信息速度的减慢、噪声环境下言语识别困难，而且中枢听觉处理障碍是老年性聋的重要组成部分。功能性磁共振成像（functional magnetic resonance imaging，fMRI）是研究大脑中枢病变的新型手段。本文从老年性聋的诊疗现状以及fMRI应用在老年性聋中的研究进展做一综述，旨在探讨fMRI对于明确老年性聋中枢听觉处理机制以及为未来听觉康复干预提供指导等方面的应用前景。

多系统萎缩症(MSA)是一种成年发病的进行性神经退行性疾病，具有进展快、致残高等特点。其主要死亡原因包括支气管肺炎、尿毒症及睡眠中发生的猝死。而喘鸣作为疾病的一个特征，可能预示着MSA患者生存期的下降。2017年10月在意大利召开了该领域专家的国际共识会议(博洛尼亚共识会议)，就多系统萎缩患者喘鸣的定义，诊断标准、治疗以及预测价值达成了共识，为将来的临床研究提供系统的证据，并探寻出今后研究工作中需要继续深入探索的科研领域，对于老年患者的管理和研究具有重要的指导意义。

Parkinson's disease(PD)can lead to falls as the disease progresses, and falls increase the mortality of PD patients.Therefore, it is important to understand the risk factors for falls in PD and use prediction tools with high accuracy in order to predict falls and propose prevention strategies.This paper summarizes the risk factors for falls in Parkinson's disease and reviews studies evaluating the accuracy of prediction tools for falls, with the aim of providing help in clinical practice.

Human brain bank is the foundation for neurology and neuroscience, which provides research materials for medical scientists to explore what happen in human brains. Through voluntary postmortem brain tissue donation, brain bank collects, classifies, stores and distributes brain tissue samples for researchers with appropriate research proposals, so as to facilitate researchers to conduct human brain research. History showed that the act of brain only autopsy distinguished neurology as a distinct discipline. The emerges of brain banking accompanied with the advances of neurology and neuroscience. It facilitates the development of neurology from neuropathological examination feedback about the clinical presentations of the brain donor, plays an important role in the definition of novel neurological diseases, and also makes an extraordinary contribution to the disease mechanisms and novel therapeutic discoveries. This manuscript elaborated the history, current situation, and the impacts of human brain banking on current neurology. Furthermore, we discussed about the opportunities and challenges of brain banking in China. DOI:10.3969/j.issn.1672⁃6731.2020.10.012

衰弱是一种常见的老年综合征，与老年人不良健康结局密切相关。早期识别和干预衰弱，制定并实施针对衰弱患者的诊疗方案，能够延缓其向失能的进展。我国大陆地区老年衰弱综合征方面的研究虽起步较晚，但在衰弱评估工具及流行病学方面研究发展迅速，未来需加强老年衰弱发病机制及干预方法的研究，并建立衰弱老年人内在能力下降应对策略和综合照护体系，以实现健康老龄化。

介绍了老年医学的核心技术即老年综合评估的概念、内容和应用重大意义.着重阐述了在三级医院开展老年综合评估技术和多学科协作的老年门诊在应对人口老龄化和医疗主体对象日益增多、服务对象问题应对、老年医学发展需要以及生物-心理-社会医学模式的转变等方面的迫切需要.三级医院应积极应对人口老龄化,努力建立符合人口老龄化和我国人群特点的老年健康管理门诊和积极学习和应用老年患者综合评估技术,为国家面临的巨大医疗社会问题和医疗决策提供循证证据支持.

目的 观察灵芝提取物(Ganoderma lucidum extract,GLE)对1-甲基-4-苯基-吡啶离子(1-methyl-4-phenylpyridinium,MPP+)诱导的Neuro-2a细胞自噬的调节作用.方法 采用蛋白印迹检测自噬标志物LC3的转换(LC3-Ⅱ/LC3-Ⅰ)以及荧光显微镜检测LC3点状聚集物的形成.同时Western blotting法检测AMPK/mTOR/ULK1、PINK1/Parkin、NIX/LC3蛋白的表达水平.结果 激光共聚焦采集图像结果显示,Mpp+处理组细胞LC3-Ⅱ聚集于自噬体膜上,观察到呈点状聚集状态,而GLE干预组细胞点状聚集状态不明显.以LC3的点状聚集阳性细胞占总细胞的百分比评价自噬水平的高低.MPP+组呈绿色点状分布的LC3阳性细胞百分比明显升高,GLE组明显降低.Western blotting法检测结果显示GLE组LC3-Ⅱ/LC3-Ⅰ比值降低.同时,经GLE处理后,可以改善MPP+损伤引起的AMPKo/mTOR/ULK1、PINK1/Parkin通路各蛋白异常表达.结论 GLE能抑制MPP+诱导的Neuro-2a细胞的自噬反应,调节细胞自噬相关蛋白AMPK/mTOR/ULK1以及PINK1/Parkin的异常表达,表明GLE可能通过调节细胞自噬而发挥神经保护作用.

老年人衰弱程度可反映身体状况,发现其相关危险因素则有助于干预衰弱,改善老年人预后及生活质量.研究发现肥胖与老年衰弱相关,而体质量指数(BMI)和腰围(WC)可用来评估肥胖程度,BMI代表人体肥胖程度,WC与代谢障碍关系密切.本文通过BMI和WC两个指标综述了肥胖和老年人衰弱的研究进展,旨在阐明三者关系,为临床干预衰弱提供新思路.

Frailty is characterized as a decline in age-related reserve and function due to various factors and is associated with adverse outcomes such as falls,disability and mortality.Many studies have found that the coexistence of physical frailty and cognitive impairment in the elderly is very common.The concept of cognitive frailty underscores the clinical association between cognitive function and physiological decline in the elderly.Interactions between physical frailty and cognitive impairment lead to decreased physical and cognitive function,resulting in poor quality of life.Therefore,early screening and intervention of cognitive frailty may help reduce the incidence of adverse health outcomes,improve the quality of life,and promote healthy aging.

Neurological outcomes like learning, memory and cognition are influenced by the gut microbiota (GM). These commensal GM modulates behavior and brain development and has implications in many neurological disorders like Alzheimer’s disease, Parkinson’s disease (PD), anxiety, stress, multiple sclerosis, etc. PD is a neurodegenerative disease which causes dysbiosis, α-synucleinopathy and affects the gut-brain axis which includes CNS (Central nervous system), ANS (Autonomic nervous system) and ENS (Enteric nervous system). There is a bidirectional communication between the brain and the gut called “gut microbiota-brain axis (GMBA)” and its dysfunction causes numerous diseases. This review focuses on the inter-relationship between the gut microbiome and the Parkinson’s disease.

Objective To investigate the clinical characteristics,changes of dopamine function and genetic mutation in patients with early onset Parkinson's disease (EOPD),defined as parkinsonism starting before age 50 years.Methods Clinical data,11 C-CFT DAT PET scan and genetic mutation of 10 sporadic EOPD patients were analyzed retrospectively.The average age of onset was (31.4±-8.0) years old.The average disease duration was (3.0±0.8) years.Results All of the patients had rigidity and bradykinesia,6 patients with resting tremor,5 patients with foot dystonia,4 patients with hyperactive tendon reflex,4 patients with diurnal fluctuation of the symptoms,3 patients with depression,and remarkable effect of levodopa were found in 9 patients.11C-CFT DAT PET imaging was performed in 8 patients.There was a significant reduction of [-11C] CFT uptake in posterior of putamen.The reductions occurred on both the contralateral and ipsilateral side in 7 patients,localized predominantly on the contralateral in 4 patients.One patient showed a significant decrease in the contralateral posterior of putamen.In case 3,the deletion mutation was found in exon 3 and exon 4 of parkin gene,exon 4 of PINK1 gene presented a repeat mutation.Homozygous mutations occurred in exon 4 of parkin gene in case 8.Heterozygous mutations occurred in exon 3 or 4 of parkin gene in 3 patients.Conclusions EOPD has some unique clinical features and changes of dopamine function in striatum.Mutations in parkin gene and PINK1 gene are related to the pathogenesis of EOPD.

神经变性疾病大多以特定蛋白的异常聚集为特点,而异常蛋白聚集的成因一直没有被阐明[1].不仅如此,在不同神经变性疾病中,异常蛋白沉积往往有其特征性的发展顺序.与这种病理上的蔓延同步,疾病的表型从最轻微的前驱症状到典型症状,再发展到晚期多脑区功能受累.以阿尔茨海默病和帕金森病(PD)为例,在脑中,前者的异常蛋白Aβ遵循"从上到下"的发展顺序,而其tau蛋白以及后者的α-突触核蛋白则"由下而上"地发展[2].动物实验发现,在特定脑区注射的异常蛋白可在脑中播散至距离较远的其他区域[2-3],这种现象被描述为"朊蛋白样"的传播,是神经变性疾病发展的重要机制.也就是说,疾病的发展在空间上是有序的,如同多米诺骨牌,一旦启动就会循着一定的顺序扩散开去.这样一来,找到多米诺骨牌的起点就变得十分重要.这种有序的空间模式的起点在哪里,疾病又是通过什么机制启动的,回答这些问题对认识神经变性疾病,乃至找到有效的治疗方案都十分关键.

Hyponatremia is a common electrolyte disturbance in elderly critical patients and one of the complications in common critical geriatric illnesses.The incidence of hyponatremia in the elderly is higher in internal medicine and neuroscience diseases such as heart failure,severe pneumonia,chronic obstructive pulmonary disease and acute or chronic cerebral vascular disease, which normally suggests poor prognosis.Physiopathologic mechanism of hyponatremia in the elderly patients is a result of concurrent mechanisms whereas mechanism of neurosurgical ones are cerebral salt wasting syndrome and syndrome of inappropriate antidiuretic hormone .Diuretics are the most common cause of hyponatremia ,and the symptoms of hyponatremia in the elderly patients are often unobservable or even insidious and need closely moni-toring.

Parkinsonu0027s disease (PD) is a common neurodegenerative disease. It is characterized by a combination of motor symptoms and non-motor symptoms (NMS) based on clinical symptoms. More and more attention has been drawn to olfactory dysfunction as an early NMS in PD. It is believed that nicotine in cigarettes may lower the risk of getting PD and people with smoking history may have lower risk of olfactory dysfunction. So smoking may have protective effect on PD. The effect of smoking on olfactory function in PD patients may lead us to have a better understanding of the pathogenesis of PD. DOI: 10.3969/j.issn.1672-6731.2017.02.004

Levodopa revolutionized the treatment of Parkinson's disease (PD) and has been playing an important role as the gold standard of treatment for PD for decades.However,the problem of motor complications during levodopa treatment plagues the doctors and patients.With emergence of studies on mechanisms of motor complications,there is growing evidence indicating that continuous dopaminergic stimulation (CDS) can reduce the risk of motor complications with good curative effects,so it have attracted much attentions in PD treatment.A large number of studies now focus on the development of new preparations of levodopa which can produce CDS to explore the balance point between improving efficacy and reducing motor complications.The retrospect and progress of levodopa treatment for PD were reviewed.

Objective To investigate the correlation between depression and motor dysfunction in patients with Parkinson's disease (PD).Methods A retrospective case-control study was adopted in this study.800 PD patients in neurology clinic were enrolled in Beijing Tiantan Hospital from April 2008 to August 2015.All patients were assessed with Center for Epidemiologic Studies Depression Scale (CES-D) and divided into the depression group and the non-depression group.The two groups were compared with general data on demographics,unified Parkinson's disease rating scale (UPDRS),Hoehn-Yahr stage and mini-mental state examination (MMSE) scores respectively.The Spearman rank correlation coefficient was used to analyze the correlation between depression and motor dysfunction.Logistic regression was applied to explore risk factors of PD depression.Results 362 cases (45.3％) were identified as suffering from depression.In the depression group,the proportion of patients with Hoehn-Yahr stage greater than 2.5 was higher than that of the non-de pression group (PD).The UPDRS part Ⅲ scores,the UPDRS scores of rigidity items,tremor items and bradykinesia items of the depression group were significantly higher than those of the non-depression group (P ＜ 0.01).There was significant correlation between CES-D scores and UPDRS motor scores (r=0.405,P ＜ 0.01).Logistic regression analysis showed:female,higher score in UPDRS part Ⅰ/Ⅱ,gait and posture disturbance,faster progression of motor symptoms were risk factors of PD depression (P ＜ 0.05).Conclusion Depression is one of the common symptoms of PD and correlates with motor dysfunction.

众所周知眼球活动受大脑环路支配,其中包括大脑皮层、脑干和小脑.在神经系统变性疾病患者中会出现眼球运动的改变,眼震电图检查(Electronystagmogram,ENG)可发现早期轻微变化.ENG是一种简单易行的检查,可以得到准确、客观的数据,特异性改变作为神经系统变性疾病的特殊标记物能够协助反映疾病的进展程度,现做如下综述.

流行病学调查显示,约25%的帕金森病(Parkinson Disease, PD)患者合并痴呆[1],新诊断的PD患者普遍存在认知功能下降[2-3],病程超过20年的患者中痴呆比例高达80%[1,4].认知障碍相对于PD其他非运动症状更能预示不良结局.诸多数据表明,PD患者认知功能损伤的模式和发展方向很大程度受遗传因素影响[2-3,5].未来用于治疗PD认知障碍的药物有望以某些基因相关病理标记物[6]或者基因产物[7]为靶点.本文探讨两类影响PD认知功能的基因,一类促进PD本身发生发展同时还影响患者认知功能,另一类虽与PD发生无显著因果关系,但对患者的认知表型有不可忽视的修饰作用.