心血管领域-心律失常方向

METHODS:BACKGROUND:People with multiple sclerosis (PwMS) report impaired hand movements and coordination. With an engineered glove we demonstrated altered finger movements in PwMS; increasing age resulted in decreased performance in healthy subjects (normative data). This study aims at investigating aging effects on finger motor performance in PwMS, in relation to disease duration and Expanded Disability Status Scale (EDSS). METHODS:Ninety-six PwMS performed repetitive finger opposition movements with the dominant hand and both hands at maximal velocity or metronome-paced. Performance was compared with the norms, and correlation coefficients between finger motor parameters, age, disease duration and EDSS were calculated. RESULTS:The majority of subjects was outside of the normal range according to age and probability increased with level of disability. Age significantly correlated with the glove parameters (r ranged in absolute value between 0.22-0.31; p-value in the range 0.002-0.049). Older subjects with lower disability showed worse performance than younger (p = 0.044 and 0.02), whilst younger subjects with higher disability performed similarly to older (p = 0.72 and 0.49). CONCLUSION:Finger motor performance assessment provides important hints about upper limb disability, which should be evaluated in relation to age, disease duration and EDSS.

METHODS:BACKGROUND:Persons with multiple sclerosis (PwMS) report difficulties with emotion regulation and empathy. Regular physical activity (RPA) improves dimensions of psychological well-being in PwMS, but it remains unclear if regular physical activity has effects on emotion regulation and empathy. The present study investigated the effect of regular physical activity on emotion regulation and empathy, and explored, if endurance training or coordinative training are better than an active control condition. METHODS:92 female PwMS (mean age: 37.4 years; age range: 20-57 years; mean EDSS: 2.43) took part in this study. Participants were randomly assigned into endurance training, coordinative training, or active control conditions that all lasted 8 weeks and were yoked on frequency, duration, and social contact. Participants completed questionnaires on emotion regulation, empathy, depression and fatigue before and after the 8-week conditions. RESULTS:Regulation and control of emotions and empathy improved over time, but more so in the exercising groups, compared to the active control group. No changes over time and between groups were observed for perception and acknowledgement of emotions, emotional expressivity, and empathy, as measured with Reading in the Eyes test. These changes were not influenced by control for depression and fatigue as covariates. CONCLUSIONS:Both endurance and coordinative exercise training had favorable effects on some aspects of emotion regulation and social cognition such as empathy in PwMS. Such initial results support for examination of exercise training for the treatment of issues of emotion regulation and social interactions in PwMS.

METHODS:BACKGROUND:There is a lack of head-to-head studies comparing the efficacy of fingolimod (FIN) and natalizumab (NTZ) as second-line therapy for relapsing-remitting multiple sclerosis (RRMS). METHODS:Multicenter, observational study, in which, information of 388 patients randomly selected and treated with FIN or NTZ in routine clinical practice was retrospectively collected with the main objective of comparing the annualized relapse rate (ARR) over the first year, after FIN or NTZ treatment initiation. RESULTS:Mean ARR during the first year of treatment was 0.28 in FIN group and 0.12 in NTZ group (p = 0.0064); nevertheless, the difference between groups lost statistical significance when the propensity score analysis was performed. Time to disability -progression was similar in both treatment groups (12.3 ± 6.7 months in FIN, and 12.8 ± 0.1 months in NTZ; p = 0.4654). Treatment persistence after the first year of treatment was higher in patients treated with FIN (95%) than in those treated with NTZ (84%; p = 0.0014). CONCLUSIONS:After 12 months of treatment, both FIN and NTZ reduced the ARR, but ARR percent reduction was significantly higher with NTZ. Treatment persistence was higher in patients receiving FIN.

METHODS:OBJECTIVES:Approximately 10%-15% of patients with myasthenia gravis (MG) are refractory to standard treatment. A sizable chunk of these patients is due to muscle-specific tyrosine kinase (MuSK) antibody-positive MG which often runs a severe course with frequent relapses and poor response to conventional treatment. We report six patients with refractory MuSK-positive MG who responded well to the treatment with rituximab. PATIENTS AND METHODS:In this prospective institute-based observational study, we report six MuSK antibody-positive MG patients, who did not achieve remission with standard treatment and were later started on rituximab infusion. RESULTS:There was a significant clinical improvement in all patients after starting rituximab. CONCLUSION:Rituximab is an effective immunomodulatory therapy in MuSK antibody-positive MG patients who are not responding to the standard treatment.

METHODS::Dimethyl Fumarate (DMF), known for its mechanism of action targeting Nrf2 and related redox homeostasis, is an approved immunotherapy for patients with Multiple Sclerosis (PwMS) in the relapsing form. We assessed how DMF modulates immune cell functions, namely the cytokine profile of co-cultured B and T cells, and the chemokine-mediated migration of immune cells. Following DMF therapy, LTα+, TNFα+ and IFNγ+ B cells were reduced while TGFβ and IL10 expression elevated. B cells from DMF-treated patients increased TGFβ and LTα expression on T cells, while DMF directly reduced TNFα+ and IFNγ+ T cells. CXCL12/CXCL13-mediated migration of B cells, Monocytes, CD4 and CD8 T cells was reduced, with altered CXCR5 and CXCR4 expression. Induction of regulatory B and T cells and reduced migration of immune cells may be part of the beneficial mechanism of DMF in PwMS.

METHODS::Predisposing factors before the onset of neuromyelitis optica spectrum disorders (NMOSD) have not been systematically evaluated by now. We investigated the detailed pre-onset history in consecutive NMOSD patients. Thirteen of the enrolled 53 NMOSD patients (24.5%) had accompanying autoimmune diseases, such as Sjögren's syndrome. History of malignancy was seen in 8 of the 53 patients (15.1%). Recent history of non-neurological clinical episodes, such as systemic allergic reaction, systemic infection, surgical operation, or traumatic injury, was seen in 23 of the 53 patients (43.4%). NMOSD patients are likely to have pre-onset history of other autoimmune diseases, malignancy, or recent non-neurological systemic conditions, which may predispose or trigger the onset of NMOSD.

METHODS::Demographic and clinical characteristics of Familial Multiple Sclerosis (FMS) have not been fully investigated yet in Abu Dhabi. The aim of this single center exploratory study was to investigate demographic and clinical characteristics of FMS compared to sporadic MS (SMS) in Abu Dhabi. A chart review single center study was conducted in 98 patients with MS. Group comparisons were performed using Mann-Whitney and Chi-Square tests as appropriate. A p < 0.05 was considered statistically significant. 24.5% were patients with FMS and 83% were Emirates. No significant differences in demographic and clinical characteristics were found between patients with FMS and SMS in overall all MS patients and in the Emirati group analyzed alone. Patients with FMS did not differ in demographic and clinical characteristics compared to patients with SMS. Further prospective studies are needed to elucidate environmental and genetic risk factors contributing to FMS in the Emirati population.

METHODS::The mechanisms regulating inflammation in large vessels vasculitis (LVV) are poorly understood. Interleukin 33 (IL-33) has been shown to license innate and adaptive immunity by enhancing Th2 cytokines production. We aimed to examine the role of IL-33 in the immunomodulation of T cell activation in LVV. T cell homeostasis and cytokines production were determined in peripheral blood from 52 patients with giant cell arteritis (GCA) and 50 healthy donors (HD), using Luminex assay, flow cytometry, quantitative RT-PCR and by immunofluorescence analysis in inflammatory aorta lesions. We found increased level of IL-33 and its receptor ST2/IL-1R4 in the serum of patient with LVV. Endothelial cells were the main source of IL-33, whereas Th2 cells, Tregs and mast cells (MC) express ST2 in LVV vessels. IL-33 had a direct immunomodulatory impact by increasing Th2 and Tregs. IL-33 and MC further enhanced Th2 and regulatory responses by inducing a 6.1 fold increased proportion of Tregs (p = 0.008). Stimulation of MC by IL-33 increased indoleamine 2 3-dioxygenase (IDO) activity and IL-2 secretion. IL-33 mRNA expression was significantly correlated with the expression of IL-10 and TGF-β within aorta inflammatory lesions. To conclude, our findings suggest that IL-33 may exert a critical immunoregulatory role in promoting Tregs and Th2 cells in LVV.

METHODS::Acute vestibular syndrome is most often caused by vestibular neuritis or stroke, although demyelinating diseases may be responsible for 4% of all AVS episodes. The authors present the case of a previously healthy 17-year-old female patient complaining of spontaneous vertigo and right-sided hearing loss. Otoneurological examination suggested a peripheral vestibular cause and video head impulse test revealed a reduced vestibulo-ocular reflex gain. The presence of sensorineural hearing loss raised the suspicion of a central cause and prompted imaging evaluation. A brain MRI evidenced demyelinating lesions in the right middle cerebellar peduncle and the patient was ultimately diagnosed with Multiple Sclerosis.

METHODS::Multiple sclerosis (MS) is the most common inflammatory neurodegenerative disease. Neurofilament light chain (NFL) is a novel adverting biomarker of axonal damage that suggested as a useful assistant in the monitoring of MS patients. It has been shown that the auto/mitophagy associated with MS pathogenesis. In this study, we aimed to study correlation between ATG5 and Parkin, as markers of autophagy and mitophagy respectively, with NFL and ANT1 in serum and cerebrospinal fluid (CSF) in MS subjects. ATG5, Parkin, NFL, and ANT1 levels were measured in a cross-sectional study of 40 MS patients compared with gender, age and BMI matching healthy volunteers. Based on our results, levels of ATG5, Parkin, and NFL significantly were elevated in both serum and CSF of MS patients comparing control individuals (p < 0.0001) but ANT1 levels significantly was decreased in both serum and CSF of MS patients comparing control individuals (p < 0.0001). The correlation indices between NFL, ANTI1, ATG5 and Parkin in both case and control groups showed a direct and moderate the correlation between ANTI1 and ATG5 in the CSF level of the control group (r = 0.554, P = 0.011). Our data support the feasibility of quantifying of NFL as a sensitive and clinically meaningful serum/CSF biomarker to follow-up nerve tissue injury in MS condition.