关键词： 神经病学   癫痫   临床诊断   治疗效果  

摘要： 探讨神经内科癫痫的临床诊断及治疗效果。方法:选择2015年8月至2018年8月我院收治的104例神经性癫痫患者,分析其诊治方法。将患者随机分为对照组和实验组,每组52人。对照组和实验组分别用丙戊酸钠缓释片联合苯巴比妥钠进行治疗。开始治疗之前和之后分析并记录症状。结果:对照组治疗后不良反应发生率为32.69%,实验组不良反应发生率为5.77%,实验组结果明显好于对照组。结论:神经内科有多种类型的癫痫病,而且临床表现非常复杂。临床诊断应根据患者的身体状况和病史,结合多种辅助检查和临床症状,以确保判断的有效性。

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.