心血管领域-心律失常方向

METHODS:BACKGROUND AND PURPOSE:Recent studies showed thalamic atrophy in the early stages of MS. We investigated the impact of intracortical lesions on the volumes of subcortical structures (especially the thalamus) compared with other lesions in MS. MATERIALS AND METHODS:Seventy-one patients with MS were included. The volumes of intracortical lesions and white matter lesions were identified on double inversion recovery and FLAIR, respectively, by using 3D Slicer. Volumes of white matter T1 hypointensities and subcortical gray matter, thalamus, caudate, putamen, and pallidum volumes were calculated using FreeSurfer. Age, MS duration, and the Expanded Disability Status Scale score were assessed. RESULTS:Patients with intracortical lesions were older (P = .003), had longer disease duration (P < .001), and higher Expanded Disability Status Scale scores (P = .02). The presence of intracortical lesions was associated with a significant decrease of subcortical gray matter volume (P = .02). In our multiple regression model, intracortical lesion volume was the only predictor of thalamic volume (R2 = 0.4, b* = -0.28, P = .03) independent of white matter lesion volume and T1 hypointensity volume. White matter lesion volume showed an impact on subcortical gray matter volume in patients with relapsing-remitting MS (P = .04) and those with disease duration of <5 years (P = .04) and on thalamic volume in patients with Expanded Disability Status Scale scores of <4.0 (P = .01). By contrast, intracortical lesion volume showed an impact on subcortical gray matter and thalamic volumes in the secondary-progressive MS subgroup (P = .02 and P < .001) in patients with a long-standing disease course (P < .001 and P = .001) and more profound disability (P < .001 and P < .001). CONCLUSIONS:Thalamic atrophy was explained better by intracortical lesions than by white matter lesion and T1 hypointensity volumes, especially in patients with more profound disability.

METHODS:OBJECTIVE:This study assessed the efficacy and safety of biological agents in patients with giant cell arteritis (GCA). MATERIALS AND METHODS:A meta-analysis of 6 randomized clinical trials (RCTs) (260 patients and 193 controls) to examine the efficacy and safety of tocilizumab, tumor necrosis factor (TNF) inhibitors, and abatacept relative to that of placebo in GCA patients was performed. RESULTS:The remission rate was significantly higher for tocilizumab-treated patients than that for placebo-treated controls (odds ratio (OR) 7.009, 95% confidence interval (CI) 3.854 - 12.75, p < 0.001). In addition, the relapse rate was significantly lower for the tocilizumab group than that for the placebo group (OR 0.222, 95% CI 0.129 - 0.381, p < 0.001). Further, no significant difference in remission and relapse was observed between groups treated with TNF inhibitors, abatacept, and placebo. The number of serious adverse events (SAEs) was significantly lower in tocilizumab-treated patients than that in placebo-treated controls (OR 0.539, 95% CI 0.296 - 0.982, p = 0.044). However, there was no significant difference in SAEs among patients treated with TNF inhibitors, abatacept, and placebo. The infection rate was significantly higher in TNF inhibitor-treated patients than in those treated with placebo (OR 2.407, 95% CI 1.168 - 4.960, p = 0.017), while there was no significant difference in infection rate between individuals treated with tocilizumab, abatacept, and placebo. CONCLUSION:Tocilizumab was found to be more effective than placebo in GCA patients, but TNF inhibitors and abatacept were not. Further, TNF inhibitors were associated with a higher risk of infection.

METHODS::Objective: To investigate the clinical features, imaging findings and prognosis of children with overlapping syndrome of myelin oligodendrocyte glycoprotein (MOG) antibody disease and anti-N-methyl-D aspartate receptor (NMDAR) encephalitis (MNOS). Methods: The clinical manifestations, immunological antibodies in blood and cerebrospinal fluid, cranial image, treatment and follow-up of 11 patients diagnosed as MNOS in the Department of Neurology, Beijing Children's Hospital from January 2011 to April 2019 were analyzed retrospectively. Results: A total of 11 patients, including 4 males and 7 females were analyzed, the age of onset was (10.4±2.3) years. A total of 29 episodes occurred in 11 children. At the last follow-up, 8 cases showed relapsed remission course, the interval of recurrence was 3 to 60 months. The onset symptoms of 11 patients included convulsions (10 cases), lethargy (6 cases), psychosis (6 cases). Among 29 episodes, the common symptoms were convulsions (16 episodes), psychosis (13 episodes),and lethargy (10 episodes). According to the diagnostic criteria of anti-NMDAR encephalitis and MOG-antibody disease, 29 episodes were divided into three phenotypes, including anti-NMDAR encephalitis(4 episodes), MOG-antibody diseases (10 episodes) and overlapping types (15 episodes).Twenty-seven times of acute stage cranial magnetic resonance imaging (MRI) were available, common lesions included cortical focus (22 times), subcortical white matter (7 times), brainstem (9 times). All patients were sensitive to first-line immunotherapy. Eight patients had recurrence during glucocorticoid reduction, 6 of them were treated with additional second-line immunosuppressive therapy, including cyclophosphamide (1 case) and mycophenolate mofetil (5 cases). The follow-up time of patients were 5-99 months. At the last follow-up, all patients were in remission, the pediatric cerebral performance category (PCPC) score was 1 (10 cases) and 2 (1 cases). Conclusions: MNOS mainly affects older children. In the period of acute episodes, convulsions and psychosis are common. The cranial MRI showed extensive brain involvement and mainly in the cortex. The recurrence rates of MNOS are relatively high, patients are sensitive to first-line immunotherapy. No significant neurological dysfunction was left in the remission stage. :目的： 探讨儿童髓鞘少突胶质细胞糖蛋白（MOG）抗体病与抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎重叠综合征（MNOS）病例的临床特点、影像学表现及预后情况。 方法： 回顾性总结2011年1月至2019年4月北京儿童医院神经内科11例MNOS病例的临床表现、血及脑脊液免疫学抗体和头颅影像学的特点以及治疗随访情况。 结果： 11例患儿中男4例、女7例，首次发病年龄（10.4±2.3）岁。11例患儿共有29次发作事件。至末次随访，8例患儿呈复发缓解病程，复发间隔3~60个月。11例患儿首次发作以抽搐（10例）、嗜睡（6例）、精神行为异常（6例）常见；29次发作事件常见的表现为抽搐（16例次）、精神行为异常（13例次）及嗜睡（10例次）。根据抗NMDAR脑炎及MOG抗体病的诊断标准，29次发作临床分型抗NMDAR脑炎4例次，MOG抗体病10例次，重叠型15例次。头颅磁共振成像检测27例次，常见受累部位包括皮层（22例次），皮层下白质（7例次），脑干（9例次）。11例患儿对一线免疫治疗均敏感，8例患儿在激素减量过程中出现复发，6例复发患儿加用二线免疫抑制剂治疗，包括环磷酰胺1例，吗替麦考酚酯5例。随访5~99个月，末次随访时，所有患儿均处于缓解期，儿童脑功能评分1分10例，2分1例。 结论： MNOS以年长儿为主，急性发作期以抽搐、精神行为异常常见。头颅磁共振成像显示受累广泛，以皮层受累为主。复发率相对较高，对免疫治疗敏感，缓解期未发现明显神经系统功能障碍。.

METHODS:BACKGROUND:Obesity is linked to greater physical disability and increased comorbidities among patients with multiple sclerosis (MS). Its contribution to cognition in this group is unclear. This observational study examines the link between obesity and processing speed in a large sample of patients with MS (PwMS). METHODS:As part of routine clinical care at our center, PwMS completed the Processing Speed Test (PST), an electronic implementation of the Symbol Digit Modalities Test (SDMT). Height and weight were used to calculate body mass index (BMI). Bivariate correlations were conducted to examine the association between PST and BMI in the group overall and in subgroups based on demographic and clinical variables. A one-way ANOVA examined differences in PST by BMI categories (normal weight, overweight, obese). RESULTS:The sample included 8,713 patients. No association between PST and BMI was found in the entire sample (r = .01), nor within subgroups based on demographic and disease variables. No difference in PST score was found between BMI categories. CONCLUSIONS:No association between BMI and processing speed was found among PwMS regardless of demographic or disease-specific patient characteristics.

METHODS::"Myasthenia gravis (MG) is the most common autoimmune neuromuscular disorder. This article highlights several cases that the practicing neurologist may encounter in the treatment of MG. Diagnostic uncertainty continues to be an issue in patients who are seronegative to the 2 most common antibodies, acetylcholine receptor and muscle-specific tyrosine kinase (MuSK). Specific populations of patients with MG including MuSK MG, thymomatous MG, refractory MG, and pregnant women also require special consideration. This article reviews specific cases and an update on current management."

METHODS::Multiple sclerosis (MS) is the most common human demyelinating disease of the central nervous system. The IL-12 family of cytokines has four members, which are IL-12 (p40:p35), IL-23 (p40:p19), the p40 monomer (p40), and the p40 homodimer (p402). Since all four members contain p40 in different forms, it is important to use a specific monoclonal antibody (mAb) to characterize these molecules. Here, by using such mAbs, we describe selective loss of p40 in serum of MS patients as compared to healthy controls. Similarly, we also observed decrease in p40 and increase in IL-12, IL-23, and p402 in serum of mice with experimental autoimmune encephalomyelitis (EAE), an animal model of MS, as compared to control mice. Interestingly, weekly supplementation of mouse and human recombinant p40 ameliorated clinical symptoms and disease progression of EAE. On the other hand, IL-12, IL-23, and p402 did not exhibit such inhibitory effect. In addition to EAE, p40 also suppressed collagen-induced arthritis in mice. Using IL-12Rβ1-/-, IL-12Rβ2-/-, and IL-12Rβ1+/-/IL-12Rβ2-/- mice, we observed that p40 required IL-12Rβ1, but not IL-12Rβ2, to suppress EAE. Interestingly, p40 arrested IL-12-, IL-23-, or p402-mediated internalization of IL-12Rβ1, but neither IL-12Rβ2 nor IL-23R, protected regulatory T cells, and suppressed Th1 and Th17 biasness. These studies identify p40 as an anti-autoimmune cytokine with a biological role different from IL-12, IL-23, and p402 in which it attenuates autoimmune signaling via suppression of IL-12Rβ1 internalization, which may be beneficial in patients with MS and other autoimmune disorders.

METHODS:BACKGROUND:Myasthenia gravis (MG), a chronic neuromuscular disorder, can adversely affect patients' health-related quality of life (HRQoL), especially in women. The study aimed to evaluate the difference in HRQoL of women and men MG patients and explore the factors that mediate the relationship between gender and HRQoL. METHODS:A cross-sectional study was conducted among 1815 patients with MG in China. The revised 15-item MG quality of life scale (MG-QOL15r) was used to access patients' HRQoL in overall, physical, social and emotional domains. Socio-demographic information, diagnosis and treatment history, comorbidities, social support, active lifestyle and the MG activities of daily living scale (MG-ADL) were recorded and compared between women and men using the Student's t-test and Pearson's Chi-square test. Multivariable regression analyses were conducted to identify independent contributors to HRQoL, especially those affecting different gender. RESULTS:On average, female patients with MG reported a lower MG-QOL15r score than the males (44.49 ± 29.10 vs 49.32 ± 29.18). The association between gender and patients' HRQoL interacted with the number of comorbidities across the overall, physical and social domains of patients. As the number of comorbidities increased, the scores of HRQoL decreased and it was faster among females than the males (p < 0.05). Moreover, unemployment, exacerbation of the disease, and active lifestyle contributed to the patients' HRQoL across all domains. Unemployment (β = - 4.99 [95%CI, - 7.80 to - 2.18], p < 0.001) and exacerbations (β = - 8.49 [95%CI, - 11.43 to - 5.54], p < 0.001) were correlated with poorer HRQoL; while an active lifestyle had a positive impact on HRQoL (β = 0.28 [95%CI, 0.16 to 0.40], p < 0.001). CONCLUSIONS:The results indicate that the HRQoL of women MG patients was lower than that of men. The relationship between gender and HRQoL is modulated by the number of comorbidities. Thus, to improve the HRQoL of women MG patients, symptomatic treatments might not be enough, their comorbid conditions should be considered as well. Additionally, employment status, MG exacerbations, and an active lifestyle have been found as determining factors of the patients' HRQoL, which suggests future interventions should cope with these factors to improve their quality of life.

METHODS:INTRODUCTION:Numerous cases have been reported of patients with symptoms of Guillain-Barré syndrome associated with COVID-19, but much information is still lacking on this association and its implications. The objective of this review is to analyse the available evidence on this topic in the adult population. MATERIAL AND METHODS:A systematic review was conducted of studies published on scientific databases: PubMed, Cochrane, Science Direct, Medline, and WHO COVID-19 database. RESULTS:We identified 47 studies, which were analysed and completed using the Covidence platform; the final analysis included 24 articles, with a total of 30 patients. CONCLUSIONS:We found a strong association between both conditions; furthermore, the studies analysed highlight differences in the presentation of the disease, with greater severity of symptoms in Guillain-Barre syndrome associated with COVID-19.

METHODS:PURPOSE:The relationship between fluid intake and lower urinary tract symptoms in individuals with neurogenic bladder is unknown. We investigated the association between fluid intake and urinary symptoms in patients with multiple sclerosis. MATERIALS AND METHODS:A prospective cross-sectional study of patients with multiple sclerosis presenting to the neurology office was conducted. Fluid intake and lower urinary tract symptoms were assessed by the questionnaire based voiding diary and the American Urological Association Symptom Score, respectively. The relationship between fluid intake and lower urinary tract symptoms was assessed using univariate and multivariate analyses. RESULTS:Among 200 individuals with multiple sclerosis the mean total daily fluid intake was 2,489 ml (SD 1,883) and did not differ according to severity (ie mild, moderate, severe) of lower urinary tract symptoms (F=0.30, p=0.74). Fluid restricting behavior to control urinary symptoms was reported by 47% of subjects. Subjects who reported fluid restricting were more likely to have worse urinary symptoms (OR 1.95, 95% CI 1.53-2.47, p <0.01). After accounting for fluid restricting behavior on multivariate analysis, there was a minimal relationship between caffeinated fluid intake and lower urinary tract symptom severity (OR 1.00, 95% CI 1.00-1.01, p=0.01), and there was no relationship between total fluid intake and lower urinary tract symptom severity (OR 1.00, 95% CI 1.00-1.00, p=0.07). CONCLUSIONS:Caffeinated fluid intake has a minimal effect on lower urinary tract symptoms in patients with multiple sclerosis. On average, patients with multiple sclerosis do not hydrate excessively and a considerable proportion restrict fluid intake to control urinary symptoms. Fluid intake may not contribute considerably to lower urinary tract symptoms in patients with multiple sclerosis.

METHODS:BACKGROUND:It is popularly believed that myasthenia gravis (MG) patients show acetylcholine receptor antibody (AChRAb) production associated with the thymus (germinal centers, approximately 80%). It has been suggested that thymectomy can remove the area of autoantibody production. This study aimed to determine whether the solid volume of the thymus calculated using three-dimensional (3D) imaging could be used to predict the efficacy of thymectomy. Additionally, the study assessed the relationships of the solid volume with germinal centers, change in the serum AChRAb level, postoperative MG improvement, and prednisolone (PSL) dose reduction extent. METHODS:This retrospective study included 12 consecutive non-thymomatous MG patients (9 female and 3 male patients), who underwent extended thymectomy at our institution over the last 10 years. The mean patient age was 43.3 ± 14.2 years (range, 12-59 years). The study assessed the number of germinal centers per unit area, change in the serum AChRAb level, postoperative MG improvement, PSL dose reduction extent, and solid volume of the thymus. RESULTS:The number of germinal centers per unit area was significantly correlated with the solid volume of the thymus. The PSL dose reduction extent tended to be correlated with the solid volume. CONCLUSIONS:Our findings suggest that the solid volume of the thymus can possibly predict steroid dose reduction. Additionally, the solid volume of the thymus in 3D images is the most important indicator for predicting the efficacy of extended thymectomy.