关键词： Spinal Stenosis   Spondylosis   Ossification of Posterior Longitudinal Ligament   Neck Pain   Spinal Fusion  

摘要： BackgroundAnterior decompression with fusion (ADF) has often been performed for degenerative cervical myelopathy (DCM) in patients with poor cervical spine alignment and/or anterior cord compression. We aimed to identify clinical and radiological predictors associated with neurological recovery after *** post-hoc analysis from a prospective multicenter study included patients who were scheduled for ADF for DCM. The patients who received other surgeries (laminoplasty, posterior decompression and fusion) were excluded. The associations between baseline clinical and radiographic variables (age, sex, body mass index, etiology, cervical lordosis, range of motion, C7 slope, C2-7 sagittal vertical axis [SVA], thoracic kyphosis [TK], lumbar lordosis, sacral slope, SVA, pelvic tilt, T1 pelvic angle [TPA], the Japanese Orthopedic Association score for the assessment of cervical myelopathy [C-JOA], European Quality of Life Five Dimensions Scale [EQ-5D], Neck Disability Index [NDI], Physical Component Summary of the SF-36 [PCS], and Mental Component Summary of the SF-36) and the recovery rates as the outcome variables were investigated in the univariate regression analysis. Then, the independent predictors for increased recovery rates were evaluated using a stepwise multiple regression *** total, 37 patients completed the 1 year follow-up. The recovery rate was significantly correlated with SVA (p=0.001) and TPA (p=0.03). Univariate regression analyses showed that age (Regression coefficient =- 0.92, p=0.049), SVA (Regression coefficient =- 0.57, p=0.004) and PCS (Regression coefficient =0.80, p=0.03) score were significantly associated with recovery rate. Then, a stepwise multiple regression analysis identified the independent predictors of recovery rate after ADF as TK (p=0.01), PCS (p=0.03), and SVA (p=0.03). According to this prediction model, the following equation was obtained: recovery rate=- 8.26+1.17x(TK) - 0.45x(SVA)+0.85x(PCS) (p=0.002, R-2

关键词： Rare diseases   Patient involvement   Research themes  

摘要： BackgroundPatient involvement in research increases the impact of research and the likelihood of adoption in clinical practice. A first step is to know which research themes are important for patients. We distributed a survey on research priorities to ERN-RND members, both patient representatives and healthcare professionals, asking them to prioritize five research themes for rare neurological diseases on a scale ranging from 1 (most important) to 5 (least important). A follow-up e-mail interview was conducted with patient representatives and professionals to assess potential reasons for differences in opinions between these two *** total, 156 responses were analysed: 61 from professionals and 95 from patient representatives. They covered all ERN-RND disease groups and came from 20 different EU countries. Almost half of the respondents considered 'Developing therapies and preventive strategies' the most important research theme. In particular, patient representatives prioritized this theme more often than professionals, while professionals prioritized 'Disease mechanisms and models'. Patient representatives indicated that therapies and prevention were of the utmost importance to them, because their lives are often heavily impacted by the disease and their main goal is to relief the burden of disease. Professionals indicated that investigating disease mechanisms will lead to more knowledge and is indispensable for finding new treatments. ConclusionsPatients and professionals have different opinions on which research theme should have priority. A qualitative follow-up shows that they respect each others' view points. Different stakeholders involved in research should be aware of their differences in research theme priority. Explaining these differences to each other leads to more understanding, and could improve patient engagement in research.

关键词： Extracorporeal membrane oxygenation   V-V ECMO   Neurological complications   Respiratory failure   Cannulation   Seizure   Propensity score  

摘要： BackgroundSingle- (SL) and double-lumen (DL) catheters are used in clinical practice for veno-venous extracorporeal membrane oxygenation (V-V ECMO) therapy. However, information is lacking regarding the effects of the cannulation on neurological *** retrospective observational study based on data from the Extracorporeal Life Support Organization (ELSO) registry. All adult patients included in the ELSO registry from 2011 to 2018 submitted to a single run of V-V ECMO were analyzed. Propensity score (PS) inverse probability of treatment weighting estimation for multiple treatments was used. The average treatment effect (ATE) was chosen as the causal effect estimate of outcome. The aim of the study was to evaluate differences in the occurrence and the type of neurological complications in adult patients undergoing V-V ECMO when treated with SL or DL *** a population of 6834 patients, the weighted propensity score matching included 6245 patients (i.e., 91% of the total cohort;4175 with SL and 20,270 with DL cannulation). The proportion of patients with at least one neurological complication was similar in the SL (306, 7.2%) and DL (189, 7.7%;odds ratio 1.10 [95% confidence intervals 0.91-1.32];p=0.33). After weighted propensity score, the ATE for the occurrence of least one neurological complication was 0.005 (95% CI -0.009 to 0.018;p=0.50). Also, the occurrence of specific neurological complications, including intracerebral hemorrhage, acute ischemic stroke, seizures or brain death, was similar between groups. Overall mortality was similar between patients with neurological complications in the two *** this large registry, the occurrence of neurological complications was not related to the type of cannulation in patients undergoing V-V ECMO.

关键词： SARS-CoV-2   Anosmia   Headache   Neurology   Seizure   Stroke  

摘要： Backgrounds The reports of neurological symptoms are increasing in cases with coronavirus disease 2019 (COVID-19). This multi-center prospective study was conducted to determine the incidence of neurological manifestations in hospitalized cases with COVID-19 and assess these symptoms as the predictors of severity and death. Methods Hospitalized males and females with COVID-19 who aged over 18 years were included in the study. They were examined by two neurologists at the time of admission. All survived cases were followed for 8 weeks after discharge and 16 weeks if their symptoms had no improvements. Results We included 873 participants. Of eligible cases, 122 individuals (13.97%) died during hospitalization. The most common non-neurological manifestations were fever (81.1%), cough (76.1%), fatigue (36.1%), and shortness of breath (27.6%). Aging, male gender, co-morbidity, smoking, hemoptysis, chest tightness, and shortness of breath were associated with increased odds of severe cases and/or mortality. There were 561 (64.3%) cases with smell and taste dysfunctions (hyposmia: 58.6%;anosmia: 41.4%;dysguesia: 100%). They were more common among females (69.7%) and non-smokers (66.7%). Hyposmia/anosmia and dysgeusia were found to be associated with reduced odds of severe cases and mortality. Myalgia (24.8%), headaches (12.6%), and dizziness (11.9%) were other common neurological symptoms. Headaches had negative correlation with severity and death due to COVID-19 but myalgia and dizziness were not associated. The cerebrovascular events (n = 10) and status epilepticus (n = 1) were other neurological findings. The partial or full recovery of smell and taste dysfunctions was found in 95.2% after 8 weeks and 97.3% after 16 weeks. The parosmia (30.9%) and phantosmia (9.0%) were also reported during 8 weeks of follow-up. Five cases with mild headaches and 5 cases with myalgia were reported after 16 weeks of discharge. The demyelinating myelitis (n = 1) and Guillain-Barre syndro

关键词： Neurological diseases   Ferroptosis   Iron   Lipid peroxide   Polyunsaturated fatty acids   Nervous system  

摘要： Ferroptosis is a newly identified form of nonapoptotic regulated cell death (RCD) characterized by irondependent accumulation of lipid peroxides which leads to oxidative stress and cell death. Recent studies have indicated that ferroptosis plays an essential role in the pathology of neurological diseases, such as intracerebral hemorrhage, ischemic stroke, epilepsy, neurodegenerative diseases, traumatic brain injury and brain cancer. This review focuses on the latest researches on the relationship of ferroptosis with nervous system diseases, highlighting the ferroptosis-based mechanisms, and elaborating the new perspective therapeutic targets of neurological disorders.

关键词： Acute intermittent porphyria   Porphyria   Cerebral vasospasm   Givosiran   Acute axonal neuropathy  

关键词： Transcriptional heterogeneity   Single cell   scRNA-seq   Neurological diseases   Neurodegenerative diseases   Huntington’   s disease   Pluripotent stem cells   Stem cell model   Smart-seq2  

摘要： Background Many neurodegenerative diseases develop only later in life, when cells in the nervous system lose their structure or function. In many forms of neurodegenerative diseases, this late-onset phenomenon remains largely unexplained. Results Analyzing single-cell RNA sequencing from Alzheimer's disease (AD) and Huntington's disease (HD) patients, we find increased transcriptional heterogeneity in disease-state neurons. We hypothesize that transcriptional heterogeneity precedes neurodegenerative disease pathologies. To test this idea experimentally, we use juvenile forms (72Q;180Q) of HD iPSCs, differentiate them into committed neuronal progenitors, and obtain single-cell expression profiles. We show a global increase in gene expression variability in HD. Autophagy genes become more stable, while energy and actin-related genes become more variable in the mutant cells. Knocking down several differentially variable genes results in increased aggregate formation, a pathology associated with HD. We further validate the increased transcriptional heterogeneity in CHD8+/- cells, a model for autism spectrum disorder. Conclusions Overall, our results suggest that although neurodegenerative diseases develop over time, transcriptional regulation imbalance is present already at very early developmental stages. Therefore, an intervention aimed at this early phenotype may be of high diagnostic value.

关键词： 线上线下   混合式教学   神经病学   理论教学   自主学习能力   传统教学  

摘要： 目的探讨线上线下混合式教学模式(以下简称混合式教学)在神经病学理论教学过程中对学生自主学习能力的培养效果。方法随机选取锦州医科大学2016级临床医学专业本科生96名作为教学对象,随机分为两组(n=48),实验组采用混合式教学,对照组采用传统教学法,教学结束后比较两组学生的自主学习能力及期末考试成绩。结果与对照组比较,实验组学生自主学习能力评分明显提高(P<0.05),期末考试理论成绩明显提高(P<0.05)。结论在临床医学本科生中应用线上线下混合式教学模式进行神经病学理论授课,能明显增强学生的自主学习能力,有效提高神经病学学习成绩。

关键词： PBL   神经病学   双语教学   八年制本科教育  

摘要： 八年制临床医学神经病学专业本科教育是一个互动性较强的教学过程。采取PBL结合双语教学的授课模式,能够较好地促进学生自主学习、活跃临床思维,教学效果理想。PBL结合双语教学为培养具有创新性和国际视野的高层次、高水平临床医学尖端人才奠定了厚实的基础。

关键词： 线上教学   作业治疗   神经病学   环境改造与辅助技术   翻转课堂  

摘要： 目的探讨基于线上教学模式下理论型与实践型康复治疗课程教学效果研究。方法选取2018-2020年广州医科大学第五临床学院学习并完成神经病学、环境改造与辅助技术两门课程考核的3个年级康复作业治疗(OT)学生作为研究对象,共70名学生。2016级和2017级采用传统面授模式,2018级为线上教学模式,通过对比3个年级的理论成绩、实践成绩及总成绩,联合教学模式和课程主效应对两门课程之间教学成效差异进行分析。结果 3个年级之间神经病学理论成绩比较,结果不存在统计学差异(Z=1.37,P=0.504);3个年级之间环境改造与辅具技术理论成绩比较,差异无统计学意义(Z=2.20,P=0.334);对于2018级OT学生,线上教学的神经病学实践成绩较传统面授的实践成绩有所下降,而线上教学环境改造与辅具技术实践成绩较传统面授的实践成绩有所提高。结论线上教学模式可满足康复OT方向教学要求,以讨论为主的实践型课程可能更适合于线上教学模式。