关键词： traumatic brain injury   saline solution   hypertonic   providing care according to standard   continuous intravenous infusion   glasgow outcome scale   intracranial hypertension  

摘要： Key PointsQuestionWhat is the effect of continuous infusion of hypertonic saline solution in patients with traumatic brain injury? FindingsIn this randomized clinical trial that included 370 adults with moderate to severe traumatic brain injury, treatment with continuous infusion of 20% hypertonic saline vs standard care resulted in an odds ratio for better neurological outcomes (based on the Extended Glasgow Outcome Scale) of 1.02 after 6 months;this was not statistically significant. MeaningAmong patients with moderate to severe traumatic brain injury, treatment with continuous infusion of 20% hypertonic saline compared with standard care did not result in a significantly better neurological status at 6 months. ImportanceFluid therapy is an important component of care for patients with traumatic brain injury, but whether it modulates clinical outcomes remains unclear. ObjectiveTo determine whether continuous infusion of hypertonic saline solution improves neurological outcome at 6 months in patients with traumatic brain injury. Design, Setting, and ParticipantsMulticenter randomized clinical trial conducted in 9 intensive care units in France, including 370 patients with moderate to severe traumatic brain injury who were recruited from October 2017 to August 2019. Follow-up was completed in February 2020. InterventionsAdult patients with moderate to severe traumatic brain injury were randomly assigned to receive continuous infusion of 20% hypertonic saline solution plus standard care (n=185) or standard care alone (controls;n=185). The 20% hypertonic saline solution was administered for 48 hours or longer if patients remained at risk of intracranial hypertension. Main Outcomes and MeasuresThe primary outcome was Extended Glasgow Outcome Scale (GOS-E) score (range, 1-8, with lower scores indicating worse functional outcome) at 6 months, obtained centrally by blinded assessors and analyzed with ordinal logistic regression adjusted for prespecified prognostic factors

关键词： Neurology   SLE   Neuropsychiatric   Lupus  

摘要： Highlights•SLE can affect the nervous system, most frequently the brain, in up to 50% of patients and the first step in assessment should be to ensure a robust diagnosis of SLE. •Heterogeneity and lack of specificity of clinical neuropsychiatric (NP) events in SLE patients obliges clinicians to consider and exclude non-SLE causes. •Pathogenetic mechanisms for NPSLE includes vascular ischemia and inflammation mediated by autoantibodies, inflammatory mediators and microglial activation. •The clinical diagnosis of NPSLE may be may be supported by neuroimaging, autoimmune serologycerebrospinal fluid and neuropsychological testing. •Treatment of NPSLE includes anticoagulation, corticosteroids and immunosuppression in addition to symptomatic therapies and treatment of relevant comorbidities.

关键词： Orbital inflammation   Hypertrophic pachymeningitis   Hypophysitis   IgG4-related disease  

摘要： Purpose of review: IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches. Recent findings: IgG4-RD is increasingly recognized as an important underlying pathophysiology in multiple disorders of neurologic interest, including orbital inflammation, infundibulo-hypophysitis, hypertrophic pachymeningitis, and even in rare cases CNS parenchymal disease and cranial vascular involvement. These were previously considered idiopathic and unrelated to any systemic disease but now known to share a common histopathology. New knowledge regarding the pathogenesis, clinical features and epidemiology of IgG4 is emerging, and new neurological manifestations continue to be described. Diagnostic progress includes CT-PET imaging, the use of flow cytometry for plasmablast quantification, and the use of reverse passive latex agglutination aiming to overcome the prozone phenomenon. Histopathologic confirmation of IgG4-RD remains the gold standard method of diagnosis but new diagnostic criteria for systemic and organ-specific disease are being proposed. Though glucorticoids remain the mainstay of therapy, relapses and incomplete recovery are frequent. Rituximab is a promising treatment in IgG4-RD that is severe, refractory or glucocorticoid dependent. Initiation of immunosuppression at an early stage of disease should be considered in order to avoid development of refractory fibrosis. Summary: The current review emphasizes the neurologic manifestations of IgG4-RD.

关键词： Paraneoplastic neurological syndromes   Immune-related adverse events   Myositis   Encephalitis   Immune checkpoint inhibitor therapy  

摘要： Neurological autoimmunity is increasingly recognized as a complication of immune checkpoint inhibitor (ICI) cancer immunotherapy. ICIs act by enhancing endogenous anti-tumor immune responses and can also lead to autoimmunity affecting all organs. ICI-related neurological autoimmunity is rare, most often manifests with neuromuscular involvement and more rarely affects the central nervous system. Neurological complications often often present in the first three months of ICI treatment but can also appear after ICI discontinuation. These can occur in patients with tumors not traditionally associated with paraneoplastic neurological autoimmunity, such as melanoma and renal-cell carcinoma and should be suspected when a new neurological symptoms present while on ICI and cannot be explained by disease progression or as a consequence of metabolic dysfunction. Treatment consists of ICI discontinuation or withdrawal depending on the severity with or without immunosuppression. Generally, improvement is observed depending on the patient's baseline characteristics and neurological presentation.

关键词： Inflammatory bowel disease   Ulcerative colitis   Crohn's disease   Neurological   Stroke   Cerebral venous thrombosis   Demyelinating   Epilepsy   Neuropathy   Myopathy Anti-TNF-alpha  

摘要： Inflammatory bowel diseases (IBD) are chronic inflammatory conditions affecting the digestive system, comprising two main distinctive entities, ulcerative colitis (UC) and Crohn's disease (CD). Besides gastrointestinal manifestations, IBD causes extraintestinal manifestations in the central and peripheral nervous system. The incidence of neurological complications in IBD ranges from 0.25% to 47.5%. The pathophysiology of neurological manifestations of IBD is mostly immune mediated, but dysfunction of the brain-gut axis, arterial and venous thromboembolism, infections, nutritional deficiencies and side-effects of medications (steroids, metronidazole, sulfasalazine, anti-TNF-alpha, anti-integrin antibodies) are other contributory mechanisms. Patients with IBD have an increased risk of arterial and venous stroke, mainly during periods of exacerbations. Vasculitis is extremely rare. There is a bidirectional association between multiple sclerosis and IBD, with a relative risk for comorbidity of 1.54, being 1.53 for the risk of multiple sclerosis in IBD and 1.55 for the risk of IBD in multiple sclerosis patients. Anti-TNF-alpha therapy is contraindicated in the treatment of patients who have both IBD and multiple sclerosis. Demyelinating disorders can also be a rare complication of anti-TNF-alpha therapy. Optic neuritis, transverse myelitis, progressive myelopathy, central nervous system infections, epilepsy and encephalopathy are among other uncommon neurological complications. Peripheral nervous system manifestations include peripheral neuropathy, either demyelination and axonal, myasthenia gravis and polymyositis/dermatomyositis and localized forms of myositis.

关键词： Neuroimmunology   Neuro-oncology   CAR T cells  

摘要： Chimeric antigen receptor (CAR) T cell therapy has become an indispensable tool in the treatment of advanced malignancy, however, it is associated with significant neurologic toxicity. The pathophysiology of CAR T-cell associated neurotoxicity is incompletely understood, and the specific risk factors have only recently begun to be characterized. Despite a growing clinical experience with CAR T cell therapy, the unpredictability of neurologic symptoms remains a source of great anxiety for patients and practitioners alike, and a major limitation for more widespread adoption of this important treatment modality. The purpose of this review is to familiarize clinicians with the typical clinical manifestations and salient features of CAR T cell associated neurotoxicity. We place an emphasis on highlighting the clinical and laboratory markers that may be helpful for predicting clinical course, allowing teams to anticipate necessary supportive measures. We will also review the appropriate diagnostic workup for CAR T cell neurotoxicity and current treatment recommendations.

关键词： Disease burden   Metachromatic leukodystrophy   Pontocerebellar hypoplasia type 2   Parents   Quality of life  

摘要： BackgroundRare and severe neurological disorders in childhood not only heavily affect the life perspective of the patients, but also their caregivers and families. The aim of this study was to investigate the impact of such diseases on the family, especially on the quality of life and life perspectives of parents, but also on the families' everyday life, based on the model of two diseases which have been well described in recent years with respect to symptoms and course: metachromatic leukodystrophy (MLD) and pontocerebellar hypoplasia type 2 (PCH2). PCH2 is a primary severe developmental disorder, while children with MLD initially develop normally and then progressively *** a semi-standardized questionnaire, 43 families with children suffering from MLD (n=30) or PCH2 (n=19) reported data on the severity of the illness/symptoms, on family support and the care situation, as well as on the circumstances of non-affected siblings and the parents' work situation. In addition, the quality of life of parents and general family functioning was assessed using the PedsQL (TM) Family Impact Module [23]. Results for the latter were compared to published data from families with children without any chronic condition using student's t-tests for independent samples. Potential factors influencing the PedsQL (TM) scores were analyzed using Spearman's rank *** of children with MLD and PCH2 reported significantly lower health-related quality of life (HRQOL) compared to parents of healthy children (P<0.001). Mothers showed significantly poorer HRQOL (P<0.05) and were significantly more dissatisfied with their professional development (P<0.05) than fathers, and this was seen in relation to their child's disease. Neither the form of disease ('primary' symptomatic PCH2 or 'secondary' symptomatic MLD), nor the severity of the child's illness (in terms of gross motor and speech function) had a specific impact on HRQOL in families. However, the tim

关键词： 糖尿病前期   老年痴呆症   助理教授   神经病学   记忆测试   高血糖   芭芭拉   胰岛素抵抗  

摘要： 《美国医学杂志·神经病学》刊登的一项新研究发现,与糖尿病前期(血糖水平偏高,但尚未达到2型糖尿病的程度)有关的高血糖会增加老年痴呆症风险。新研究中,美国威斯康星大学麦迪逊分校助理教授芭芭拉·本德琳博士及其同事对无精神障碍的150名成人参试者进行了记忆测试,研究人员还测量了这些平均年龄为61岁的参试者的胰岛素抵抗情况。

关键词： 混合教学模式   神经病学   教学模式   理论知识   临床操作能力  

摘要： 本文探析混合教学模式在神经病学教学中的应用效果。选择2019年9月—2021年1月时间段,抽取于本院接受神经病学教学的学生,例数为100例,根据入学的先后时间将其分为参照组(2016级57人)与研究组(2017级43人),其中参照组借助传统方式进行教学,研究组借助混合教学模式开展教学,对两组学生的教学效果与教学满意度进行分析和对比。研究组学生研究组理论成绩与操作技能分别为(90.01±5.79)分和(89.14±4.89)分,教学满意度为93.0%,其数据均显著优于参照组(t=6.504,P=0.000;t=7.947,P=0.000;χ^(2)=7.996,P=0.004)。神经病学教学中应用混合教学模式能够提升学生对理论知识的理解与运用能力,便于学生临床操作技能的提高,具有显著的教学应用价值。

关键词： Astrocyte   Gap junctions   Connexins   Hemichannels   CNS drugs   Neuropsychiatric diseases   Neurodegenerative diseases   Connexin-modulating drugs  

摘要： Astrocytes represent the reticular part of the central nervous system;gap junctions formed by connexins Cx43, Cx30- and Cx26 provide for homocellular astrocyte-astrocyte coupling, whereas connexins Cx30, Cx32, Cx43, and Cx47 connect astrocytes and oligodendrocytes. Astroglial networks are anatomically and functionally segregated being homologous to neuronal ensembles. Connexons, gap junctions and hemichannels (unpaired connexons) are affected in various neuropathologies from neuropsychiatric to neurodegenerative diseases. Manipulation of astrocytic connexins modulates the size and outreach of astroglial syncytia thus affecting astroglial homeostatic support. Modulation of astrocytic connexin significantly modifies pharmacological profile of many CNS drugs, which represents an innovative therapeutic approach for CNS disorders;this approach is now actively tested in pre-clinical and clinical studies. Wide combination of connexin modulators with CNS drugs open new promising perspectives for fundamental studies and therapeutic strategies.