心血管领域-心律失常方向

METHODS:BACKGROUND AND PURPOSE:The inhomogeneous magnetization transfer technique has demonstrated high specificity for myelin, and has shown sensitivity to multiple sclerosis-related impairment in brain tissue. Our aim was to investigate its sensitivity to spinal cord impairment in MS relative to more established MR imaging techniques (volumetry, magnetization transfer, DTI). MATERIALS AND METHODS:Anatomic images covering the cervical spinal cord from the C1 to C6 levels and DTI, magnetization transfer/inhomogeneous magnetization transfer images at the C2/C5 levels were acquired in 19 patients with MS and 19 paired healthy controls. Anatomic images were segmented in spinal cord GM and WM, both manually and using the AMU40 atlases. MS lesions were manually delineated. MR metrics were analyzed within normal-appearing and lesion regions in anterolateral and posterolateral WM and compared using Wilcoxon rank tests and z scores. Correlations between MR metrics and clinical scores in patients with MS were evaluated using the Spearman rank correlation. RESULTS:AMU40-based C1-to-C6 GM/WM automatic segmentations in patients with MS were evaluated relative to manual delineation. Mean Dice coefficients were 0.75/0.89, respectively. All MR metrics (WM/GM cross-sectional areas, normal-appearing and lesion diffusivities, and magnetization transfer/inhomogeneous magnetization transfer ratios) were observed altered in patients compared with controls (P < .05). Additionally, the absolute inhomogeneous magnetization transfer ratio z scores were significantly higher than those of the other MR metrics (P < .0001), suggesting a higher inhomogeneous magnetization transfer sensitivity toward spinal cord impairment in MS. Significant correlations with the Expanded Disability Status Scale (ρ = -0.73/P = .02, ρ = -0.81/P = .004) and the total Medical Research Council scale (ρ = 0.80/P = .009, ρ = -0.74/P = .02) were observed for inhomogeneous magnetization transfer and magnetization transfer ratio z scores, respectively, in normal-appearing WM regions, while weaker and nonsignificant correlations were obtained for DTI metrics. CONCLUSIONS:With inhomogeneous magnetization transfer being highly sensitive to spinal cord damage in MS compared with conventional magnetization transfer and DTI, it could generate great clinical interest for longitudinal follow-up and potential remyelinating clinical trials. In line with other advanced myelin techniques with which it could be compared, it opens perspectives for multicentric investigations.

METHODS:OBJECTIVE:The aim of this study is to determine the effectiveness of an extended cognitive rehabilitation program in group's sessions in multiple sclerosis. DESIGN:Double-blind multicenter randomized trial. PARTICIPANTS:People with multiple sclerosis of 18 to 60 years, Expanded Disability Status Scale ⩽6.0, mild to moderate cognitive impairment. INTERVENTIONS:They were randomized into cognitive rehabilitation program (ProCog-SEP) or in a placebo program. ProCog-SEP comprises 13 group's sessions over 6 months and includes psychoeducational advices and cognitive exercises. Placebo program included non-cognitive exercises. No strategy and no cognitive advice were provided. MAIN MEASURES:The primary endpoint was the percentage of verbal memory learning measured by the Selective Reminding Test. A comprehensive neuropsychological assessment is carried out before and after interventions by a neuropsychologist blinded to intervention. Effectiveness of the ProCog-SEP versus Placebo has been verified using linear regression models. RESULTS:In total, 128 participants were randomized and 110 were included in the study after planning session in groups; 101 completed this trial (77.2% females); mean age: 46.1 years (±9.6); disease duration: 11.8 years (±7.5). ProCog-SEP was more effective in increasing in learning index (9.21 (95% confidence interval (CI): 1.43, 16.99); p = 0.02) and in working memory on manipulation (0.63 (95% CI: 0.17, 1.09); p = 0.01), and updating capacities (-1.1 (95% CI: -2.13, -0.06); p = 0.04). No difference was observed for other neuropsychological outcomes. Regarding quality of life outcomes, no change was observed between the two groups. CONCLUSION:These findings suggest that ProCog-SEP could improve verbal learning abilities and working memory in people with multiple sclerosis. These improvements were observed with 13 group sessions over 6 months.

METHODS:BACKGROUND:Temporal arteritis (TA) is a systemic inflammatory vasculitis of unclear etiology that affects medium-sized vessels. The gold standard for diagnosis has traditionally been histological, by temporal artery biopsy. Improved imaging modalities have been increasingly used to aid diagnosis and are recommended in the newest 2018 European (EULAR) guidelines.1 We hypothesize that a negative TA biopsy result does not change management in patients for whom TA is strongly suspected and that duplex ultrasound can be successfully used as a screening tool. METHODS:This is a retrospective review of patients who underwent TA biopsy between May 1, 2012 and June 1, 2017. We reviewed patient's demographics, comorbidities, symptoms, histology, and treatment. We also present a small series of patients for whom ultrasound of the bilateral temporal arteries was performed. Radiology and pathology reports on these 7 patients were reviewed. RESULTS:A total of 264 patients underwent temporal artery biopsies over the study period. Histology was positive in 21 (8.0%) and negative in 243 (92%) patients. In 74 (41%) patients with negative biopsies on steroids preoperatively, steroids were continued despite negative biopsy result. In prospective series, 7 patients underwent duplex ultrasound evaluation before scheduling for biopsy. Biopsy followed ultrasound in 4 cases, and in all 4 cases, histology was congruent with ultrasound findings. CONCLUSIONS:The yield of temporal artery biopsy is low, and a negative biopsy alone often does not lead to termination of steroid therapy. Ultrasound may present a viable diagnostic tool to reduce the number of unnecessary temporal artery biopsies performed.

METHODS:INTRODUCTION:Neuromyelitis optica spectrum disorder (NMO-SD) has been recognized for the past decade. Biomarkers such as anti-Aquaporin 4 antibodies (AQP4) and anti-Myelin Oligodendrocyte Glycoprotein (MOG) have been able to classify NMO-SD into several groups. METHODS:A retrospective study was performed in the Strasbourg University Medical Center among patients with AQP4+, MOG+ and double-seronegative NMO to compare their clinical, epidemiological and paraclinical features. RESULTS:Thirty-two patients with NMO were included. The AQP4+ NMO patients had a median of age of 45 years, with associated myelitis in 62.5% of cases and other autoantibodies in 37.5% of cases. The mean number of relapses by clinical history was 3. The mean initial visual acuity during an exacerbation was 0.3 LogMAR, and the visual acuity after an exacerbation was 0.1 LogMAR. MOG+NMO patients had a median age of 23 years, with severely impaired initial visual acuity (0.6 LogMAR) but better recovery (0 LogMAR); optic disc edema was present in 80% of cases; the mean number of relapses on clinical history was 1. AQP4-/MOG- NMO's were more common in women (70%) and were bilateral in 40% of cases. CONCLUSION:The diagnostic characteristics of NMO-SD are becoming increasingly differentiated, with a positive impact on functional prognosis and long-term progression. Other biomarkers have yet to be identified to improve the diagnosis and treatment of these disorders.

METHODS:BACKGROUND:Falls are common and serious health concern in people with multiple sclerosis (MS). Various types of falls prevention interventions are currently investigated in people with MS. OBJECTIVE:To assess the effectiveness of interventions to reduce falls in people with MS. METHODS:To summarize falls prevention interventions from the published Cochrane Review "Interventions for preventing falls in people with multiple sclerosis" conducted by Hayes et al. Best available evidence was discussed from the rehabilitation perspective. RESULTS:Overall 13 RCTs with 839 participants were included. The interventions evaluated included: exercise, education, and functional electrical stimulation alone or in combination. Majority of the included studies demonstrated high risk of bias. The findings suggest that the evidence was uncertain regarding the effects of evaluated interventions on preventing or reducing falls. CONCLUSIONS:The evidence for any falls prevention interventions in people with MS is sparse and uncertain, and more robust studies are needed.

METHODS::Please verify term, "alternative". Chronic immune-mediated demyelinating polyneuropathy (CIDP) is a treatable immune-related demyelinating polyneuropathy. Approximately 20% of cases do not respond to first-line therapies; most of these cases are due to alternative diagnoses, although some of them are due to severe CIDP. Unfortunately, a lack of universally accepted diagnostic criteria complicates the course of diagnosis and treatment. This article discusses videos of cases referred to a tertiary medical center for "refractory CIDP" and pitfalls in the diagnosis and management of this condition.

METHODS:OBJECTIVE:To test the association between physical function and the social environment in multiple sclerosis (MS), we quantified personal social networks. METHODS:In this cross-sectional study, we analyzed data from 2 academic MS centers, with center 1 serving as a discovery group and center 2 as the extension group. We performed a meta-analysis of the centers to extend the analysis. We used responses from a questionnaire to map the structure and health habits of participants' social networks as well as the NIH Patient-Reported Outcomes Measurement Information System (PROMIS) physical function scale (0-100, mean 50 for US general population) as the primary outcome. We applied multivariable models to test the association between network metrics and physical function. RESULTS:The discovery cohort included 263 patients with MS: 81% were women, 96% non-Hispanic European, 78% had relapsing MS, average age was 50 (12.4) years, and mean disease duration was 17 (12.3) years. The extension group included 163 patients, who were younger, more racially diverse, and less physically disabled, and had shorter disease duration. In the meta-analysis, higher network constraint, a measure of tightly bound networks, was associated with worse physical function (β = -0.163 ± 0.047, p < 0.001), while larger network effective size, a measure of clustered groups in the network, correlated with better physical function (β = 0.134 ± 0.046, p = 0.003). CONCLUSIONS:Our study highlights personal networks as an important environmental factor associated with physical function in MS. Patients with close-knit networks had worse function than those with more open networks. Longitudinal studies are warranted to evaluate a causal relationship between network structure and physical impairment.

METHODS:BACKGROUND:So far, little is known about the properties of human epididymis protein 4 (HE4) in multiple sclerosis (MS). This type 4 glycoprotein belongs to a family of genes encoding proteins whose expression is associated with the process of spermatogenesis in the epididymis. The biological function of HE4 is not fully understood. Overexpression of HE4 has been found in several malignant tumors, particularly in ovarian cancer, as well as in mesothelioma, lung, endometrial, breast, and kidney cancers. OBJECTIVES:To evaluate serum HE4 in patients with relapsing-remitting multiple sclerosis (RRMS) as compared to healthy controls. MATERIAL AND METHODS:Fifty patients with RRMS undergoing first-line immunomodulatory treatment were enrolled in the prospective study. We analyzed correlations between serum HE4 levels and gender, age, disease duration, the Expanded Disability Status Scale (EDSS), annualized relapse rate (ARR), and magnetic resonance imaging (MRI) lesions. RESULTS:The patients from the study group had higher concentrations of HE4 than the subjects from the control group. Patients with EDSS > 2 had significantly higher concentrations of HE4. Positive correlations were found between HE4 concentrations and age as well as between HE4 concentrations and disease duration. No significant correlations were found between HE4 concentrations and EDSS or between HE4 concentrations and ARR. CONCLUSIONS:The results of the study indicate a novel aspect of the HE4 protein in the pathomechanisms of MS.

METHODS::PET targeting the translocator protein (TSPO) expression is an interesting approach to detect neuroinflammation, as TSPO is upregulated in activated macrophages and microglia. Considering the variable pathophysiology of multiple sclerosis (MS) variants, we compare TSPO PET using F-GE-180 in 3 different demyelinating diseases of the central nervous system: relapsing-remitting MS, tumefactive MS, and Baló's concentric sclerosis. Visualization of neuroinflammation and its PET patterns in addition to MRI may contribute to accurate distinction and monitoring of central nervous system demyelination.

METHODS::Specific therapies targeting B lymphocytes in multiple sclerosis (MS) have demonstrated reductions in disease activity and disability progression. Several observational studies have also shown the effects of targeting B lymphocytes in other rare CNS inflammatory diseases, such as neuromyelitis optica spectrum disorder (NMOSD) and autoimmune encephalitis (AE). However, some drugs targeting cytokine receptors involved in B-lymphocyte maturation and proliferation resulted in negative outcomes in MS. These apparently conflicting findings have stimulated research on the pathophysiologic mechanisms of B lymphocytes in CNS inflammatory diseases. It has been demonstrated that B lymphocytes participate in the pathogenesis of these conditions as antigen-presenting cells, producing proinflammatory cytokines that induce Th1 and Th17 responses and producing antibodies. However, they are also able to produce anti-inflammatory cytokines, such as interleukin-10, functioning as regulators of autoimmunity. Understanding these diverse effects is essential for the development of focused treatments. In this review, we discuss the possible mechanisms that underlie B-lymphocyte involvement in MS, NMOSD, and AE and the outcomes obtained by treatments targeting B lymphocytes.