关键词： 诊断思路   疾病分类   神经病理   神经电生理监测   神经病学   神经系统疾病   神经内科   疾病症状  

摘要： 由上海交通大学医学院附属瑞金医院神经内科王刚教授主编,凝聚全国20余位著名神经病学专家临床诊疗实践经验的《神经病学诊断思路》(第一版)(ISSN:978-7-313-26800-6)已于2022年由上海交通大学出版社出版发行。《神经病学诊断思路》首次尝试从疾病症状和疾病分类两个维度对神经系统疾病的诊断实践及最新进展进行系统总结和介绍,围绕某一症状或疾病,引入神经影像学、体液标志物、神经电生理监测、基因检测以及神经病理检查等辅助诊断技术,全方位多视角介绍疾病的临床诊断思路和流程,尤其突出作者自身的临床实践。

关键词： 临床神经病学   微信公众平台   信息反馈   微信号   三种方式   杂志  

摘要： 为了加强与广大读者、作者以及专家的沟通,及时进行信息反馈,《临床神经病学杂志》微信公众平台于2015年8月正式开通,欢迎大家关注!您可通过以下三种方式关注《临床神经病学杂志》微信公众平台:1、搜索公众订阅号名称“临床神经病学杂志”添加关注;2、搜索微信号“lcsjbx”添加关注;3、扫描位于本刊目次页右上角的微信二维码。

关键词： 审稿专家   审稿人   临床神经病学   编辑部   编委   杂志   保质保量   年度  

摘要： 在2021年度,《临床神经病学杂志》得益于各位编委和审稿人的大力支持,保质保量地完成了审稿任务。本刊编辑部在此向以下各位审稿专家表示诚挚的谢意!

关键词： 中国医师协会   中华医学会   神经内科医师   非运动症状   帕金森病   临床评估   运动障碍   神经病学  

摘要： 推荐理由:睡眠障碍是帕金森病的一种常见非运动症状。近年来,对于帕金森病睡眠障碍的发病机制、临床评估、治疗方法均取得了新进展。中华医学会神经病学分会帕金森病及运动障碍学组、中国医师协会神经内科医师分会帕金森病及运动障碍学组共同制订了《中国帕金森病睡眠障碍管理专家共识》,旨在进一步规范我国帕金森病睡眠障碍的诊断与治疗。

摘要： 《临床神经病学杂志》系国家级神经病学专业性学术期刊。国内统一刊号CN 32-1337/R, 国际标准刊号ISSN 1004-1648。曾荣获第二届全国优秀科技期刊奖及连续多次江苏省优秀期刊奖,并已入选中国科技核心期刊。2005年、2006年先后被美国化学文摘(CA)、俄罗斯文摘杂志(РЖ)、波兰哥白尼索引(IC)以及美国《乌利希国际期刊指南》四大数据库收录。

关键词： 住院医师   小讲课   案例教学法   神经病学  

摘要： 目的:探讨住院医师小讲课结合案例教学法(CBL)在神经病学教学中的应用。方法:以一个典型的视神经脊髓炎谱系疾病小讲课为例,探讨如何做好神经病学住培小讲课。结果:从小讲课前准备、小讲课过程、小讲课课后等方面做好神经病学住培小讲课,包括重视病史采集、调动学习热情、归纳总结、介绍本专业新进展、新知识等。结论:住院医师小讲课结合CBL能提高住院医师的自主学习能力及诊疗思维,值得进一步实践。

关键词： autoimmune encephalitis   CIDP   daratumumab   myasthenia gravis   sporadic late onset nemaline myopathy  

摘要： Background and purpose A fraction of patients with antibody-mediated autoimmune diseases remain unresponsive to first-/second-line and sometimes even to escalation immunotherapies. Because these patients are still affected by poor outcome and increased mortality, we investigated the safety and efficacy of the plasma cell-depleting anti-CD38 antibody daratumumab in life-threatening, antibody-mediated autoimmune diseases. Methods In this retrospective, single-center case series, seven patients with autoantibody-driven neurological autoimmune diseases (autoimmune encephalitis, n = 5;neurofascin antibody-associated chronic inflammatory demyelinating polyneuropathy associated with sporadic late onset nemaline myopathy, n = 1;seronegative myasthenia gravis, n = 1) unresponsive to a median of four (range = 4-9) immunotherapies were treated with four to 20 cycles of 16 mg/kg daratumumab. Results Daratumumab allowed a substantial clinical improvement in all patients, as measured by modified Rankin Scale (mRS;before treatment: mRS =5, n = 7;after treatment: median mRS =4, range = 0-5), Clinical Assessment Scale in Autoimmune Encephalitis (from median 21 to 3 points, n = 5), Inflammatory Neuropathy Cause and Treatment disability score (from 7 to 0 points, n = 1), and Quantitative Myasthenia Gravis score (from 16 to 8 points, n = 1). Daratumumab induced a substantial reduction of disease-specific autoreactive antibodies, total IgG (serum, 66%, n = 7;cerebrospinal fluid, 58%, n = 5), and vaccine-induced titers for rubella (50%) and tetanus toxoid (74%). Treatment-related toxicities Grade 3 or higher occurred in five patients, including one death. Conclusions Our findings suggest that daratumumab provided a clinically relevant depletion of autoreactive long-lived plasma cells, identifying plasma cell-targeted therapies as promising escalation therapy for highly active, otherwise treatment-refractory autoantibody-mediated neurological diseases.

关键词： COVID-19   long COVID   neurological manifestations   SARS-CoV-2  

摘要： Background and purpose Neurological sequelae from coronavirus disease 2019 (COVID-19) may persist after recovery from acute infection. Here, the aim was to describe the natural history of neurological manifestations over 1 year after COVID-19. Methods A prospective, multicentre, longitudinal cohort study in COVID-19 survivors was performed. At a 3-month and 1-year follow-up, patients were assessed for neurological impairments by a neurological examination and a standardized test battery including the assessment of hyposmia (16-item Sniffin' Sticks test), cognitive deficits (Montreal Cognitive Assessment < 26) and mental health (Hospital Anxiety and Depression Scale and Post-traumatic Stress Disorder Checklist 5). Results Eighty-one patients were evaluated 1 year after COVID-19, out of which 76 (94%) patients completed a 3-month and 1-year follow-up. Patients were 54 (47-64) years old and 59% were male. New and persistent neurological disorders were found in 15% (3 months) and 12% (10/81;1 year). Symptoms at 1-year follow-up were reported by 48/81 (59%) patients, including fatigue (38%), concentration difficulties (25%), forgetfulness (25%), sleep disturbances (22%), myalgia (17%), limb weakness (17%), headache (16%), impaired sensation (16%) and hyposmia (15%). Neurological examination revealed findings in 52/81 (64%) patients without improvement over time (3 months, 61%, p = 0.230) including objective hyposmia (Sniffin' Sticks test <13;51%). Cognitive deficits were apparent in 18%, whereas signs of depression, anxiety and post-traumatic stress disorders were found in 6%, 29% and 10% respectively 1 year after infection. These mental and cognitive disorders had not improved after the 3-month follow-up (all p > 0.05). Conclusion Our data indicate that a significant patient number still suffer from neurological sequelae including neuropsychiatric symptoms 1 year after COVID-19 calling for interdisciplinary management of these patients.

关键词： AGREE   consensus   Delphi   guideline   neurology   rare diseases   recommendation  

摘要： Background and purpose Rare diseases affect up to 29 million people in the European Union, and almost 50% of them affect the nervous system or muscles. Delays in diagnosis and treatment onset and insufficient treatment choices are common. Clinical practice guidelines (CPGs) may improve the diagnosis and treatment of patients and optimize care pathways, delivering the best scientific evidence to all clinicians treating these patients. Recommendations are set for developing and reporting high-quality CPGs on rare neurological diseases (RNDs) within the European Academy of Neurology (EAN), through a consensus procedure. Methods A group of 27 experts generated an initial list of items that were evaluated through a two-step Delphi consensus procedure and a face-to-face meeting. The final list of items was reviewed by an external review group of 58 members. Results The consensus procedure yielded 63 final items. Items are listed according to the domains of the AGREE instruments and concern scope and purpose, stakeholder involvement, rigour of development, and applicability. Additional items consider reporting and ethical issues. Recommendations are supported by practical examples derived from published guidelines and are presented in two tables: (1) items specific to RND CPGs, and general guideline items of special importance for RNDs, or often neglected;(2) items for guideline development within the EAN. Conclusions This guidance aims to provide solutions to the issues specific to RNDs. This consensus document, produced by many experts in various fields, is considered to serve as a starting point for further harmonization and for increasing the quality of CPGs in the field of RNDs.

关键词： 神经病学   脑小血管病   认知障碍   影像学总负荷   血压变异性  

摘要： 随着近些年来血管性认知障碍(vascular cognitive impairment,VCI)患病率的日益增长,作为VCI最主要病因,脑小血管病(cerebral small vessel disease,CSVD)的相关研究越来越热门。CSVD相关认知功能障碍的早期预测、诊断、治疗等方面受到大家广泛关注,一些标记物与CSVD认知障碍的关系对疾病预测、诊断、治疗具有重要临床意义。本文旨在就影像学总负荷与血压变异性这两项标记物与CSVD认知障碍之间的相关性研究进展进行综述。