心血管领域-心律失常方向

METHODS:BACKGROUND:Multiple sclerosis (MS) can manifest itself in many ways, all of which can affect the independent outdoor mobility of persons with MS (pwMS). In most studies, mobility of pwMS is defined by the ability to walk. However, mobility comprises more than walking alone. This systematic review provides an overview of the literature on several types of independent outdoor mobility of pwMS. We aimed to identify which specific factors may influence outdoor mobility and how the lives of pwMS may be affected by a reduced mobility. METHODS:A systematic literature search was performed, using three databases (PubMed, PsychInfo and Web of Science). Studies had to describe a group of pwMS sclerosis and had to concern some type of mobility other than walking. RESULTS:The 57 studies that fulfilled the criteria included in total 10,394 pwMS and in addition, 95,300 pwMS in separate prevalence study. These studies showed that pwMS as a group have a decreased fitness to drive, make use of a wheelchair or mobility scooter more often and have difficulties making use of public transport. Mobility problems especially occur in patients with cognitive problems, secondary progressive MS or high disability scores. CONCLUSIONS:The reduced mobility may prevent pwMS participating in society. However, few studies investigating interventions or rehabilitation options to improve mobility were found in the existing literature, highlighting an until now under recognised unmet need.

METHODS:BACKGROUND:The prevalence of trigeminal neuralgia (TN) in Multiple Sclerosis (MS) patients is higher than in the general population and its management can be particularly challenging. Our aim is to describe the characteristics, treatment and prognostic factors of MS-related TN in a retrospective multicentre study. METHODS:Neurologists members of the RIREMS group (Rising Researchers in MS) enrolled MS patients with a TN diagnosis and filled out a spreadsheet comprising their clinical data. RESULTS:Population consisted of 298 patients. First-choice preventive treatments were carbamazepine and oxcarbazepine. A surgical procedure was performed in 81 (30%) patients, most commonly gamma knife stereotactic radiosurgery (37%), followed by microvascular decompression (22%) and radiofrequency thermocoagulation (21%); one third of patients underwent at least two procedures. Surgery was associated with higher disability, male sex and longer interval between MS and TN onset. Patients (77%) who stayed on at least one preventive medication at most recent follow-up, after a mean period of 8 years, had a higher disability compared to the untreated group. Furthermore, patients with higher disability at TN onset were less likely to discontinue their first preventive medication due to pain remission, had bilateral TN more frequently and underwent surgical interventions earlier. CONCLUSION:MS patients with a higher disability at TN onset and with a longer interval between MS and TN onset had differing clinical features and outcomes: pain was more frequently bilateral, surgery was more frequent and anticipated, and preventive medication discontinuation due to pain remission was less common.

METHODS:BACKGROUND:Cognitive impairment is highly prevalent in multiple sclerosis (MS). Due to the lack of specialized neuropsychological resources in many MS clinics, a brief cognitive monitoring tool that can be administered by other MS clinic staff is needed. BICAMS (Brief International Cognitive As-sessment for Multiple Sclerosis) has been developed and recommended by MS experts to monitor MS-related cognitive impairment. International validations of the tool are warranted. OBJECTIVE:The primary aim of the study was to establish a Danish translation of BICAMS as a feasible cognitive monitoring tool and to provide a Danish contribution to the international validation of BI-CAMS. A secondary aim was to determine if BICAMS correlated with self-reported cognition. The study population comprised people with MS (pwMS) with relatively early MS and newly diagnosed. METHODS:65 pwMS were compared to healthy controls (HCs) matched on age, sex and education. PwMS and controls completed the BICAMS test battery which includes the Symbol Digit Modalities Test (SDMT, oral version), California Verbal Learning Test-II (CVLT-II) and the Brief Visuospatial Memory Test-Revised (BVMT-R). In addition, self-reported cognition, fatigue, depression and quality of life were assessed. To evaluate the reliability of the BICAMS test, all participants were retested 2-3 weeks later with alternate versions of the tests. RESULTS:Mean age of the MS group was 37.2 years; 63% were female and all pwMS had a relapsing-remitting disease course. MS disease duration was relatively short; mean disease duration was 3.9 years and 32 of 65 pwMS (49%) were newly diagnosed with MS, i.e. diagnosed within the last 2 years. Mean EDSS was 1.8 with a span from 0-4. Comparison of the groups showed that the MS group performed significantly below the control group on the 3 BICAMS measures: SDMT (p<0.005), CVLT-II (p<0.05) and BVMT-R (p<0.05). When the results were controlled for influence from depression and fatigue by regression analysis, group differences were limited to the SDMT (p<0.05) and the BVMT-R (p<0.05) and these group differences were only found at the retest session. The BICAMS measures were reliable over time (r = 0.90 for SDMT, r = 0.82 for CVLT-II and r = 0.68 for BVMT-R). 32.3% of the MS population was cognitively impaired on at least one of the 3 BICAMS tests (defined as -1.5 SD compared to HCs). In the MS group 20% were impaired on the SDMT; 16.9% were impaired on the BVMT-R and 10.7% were impaired on the CVLT-II. There was no relationship between BICAMS test-scores and subjectively reported cognition, fatigue or depression. CONCLUSION:The Danish translation of BICAMS was a reliable and feasible cognitive assessment tool. This finding was confirmed even in an MS population characterized by relatively early MS and high cognitive reserve. Frequency of cognitive dysfunction detected by BICAMS in this study was relatively low due to population characteristics.

METHODS::Fulminant demyelinating disease including acute disseminating encephalitis, multiple sclerosis (MS) variants, and neuromyelitis optica spectrum disorder (NMOSD) are often managed with similar acute treatment such as intravenous methylprednisolone and plasma exchange. On the other hand, long-term management varies. The choice of the drug is based on several factors including the activity and severity of the disease course. Tocilizumab (TCZ), which is a humanized anti-interleukin-6 receptor antibody, is one of the promising therapies for NMOSD because of decreasing the relapse rates and possibly the neurological disability. However, the efficacy of TCZ for MS with tumefactive lesion is unknown. Here, we describe the clinical course of a 12-year-old Japanese boy who was diagnosed with fulminant MS with a tumefactive cervical lesion. Our case was refractory to aggressive immunosuppressive therapies and developed dependent on an intermediate dose of oral prednisolone (PSL) for relapse prevention. His neurological condition worsened with every attempt of tapering the PSL dose. Thus, we started treatment with tocilizumab, which allowed of tapering of the PSL dose without his symptom exacerbations, and effectively improved his Expanded Disability Status Scale score. Our findings may indicate that TCZ is effective for fulminant MS patients with a tumefactive cervical lesion.

METHODS:PURPOSE:Longer latency of postural response in multiple sclerosis (MS) may be linked to imbalance and increased likelihood of falls. It may be caused by the compromised microstructural integrity in the spinal cord, as evidenced by slowed somatosensory conduction in the spinal cord. Thus, the purpose of this study is to investigate the correlation between latency of postural responses and microstructural integrity of the cervical spinal cord, the region particularly related to the disease severity in MS, using diffusion tensor imaging (DTI) metrics. METHODS:Seventeen persons with MS with mild-to-moderate disease severity were enrolled in this study. Postural response latencies of each patient were measured using electromyography of the tibialis anterior muscle (TA) and gastrocnemius muscle (GN) in response to surface perturbations. Cervical spinal cord DTI images were obtained from each patient. DTI mean, radial, axial diffusivity, and fractional anisotropy (FA) were measured between segments C4 and C6. Correlations of DTI metrics with postural response latencies, expanded disability status scale (EDSS) scores, and 25-foot walk (T25FW) were assessed using the Spearman's rank correlation coefficient at α = 0.05. RESULTS:Lower FA was significantly correlated with longer latencies measured on right TA in response to forward postural perturbations (r = -0.51, p = .04). DTI metrics showed no significant correlations with EDSS scores (r = -0.06-0.09, p = .73-0.95) or T25FW (r = -0.1-0.14, p = .6-0.94). DTI metrics showed no significant differences between subjects with and without spinal cord lesions (p = .2-0.7). CONCLUSIONS:Our results showed a significant correlation between lower FA in the cervical spinal cord and longer latencies measured on right TA in response to forward postural perturbations in persons with MS, suggesting that impaired cervical spinal cord microstructure assessed by DTI may be associated with the delayed postural responses.

METHODS:BACKGROUND:Studies show that dysphagia is a common problem in patients with demyelinating diseases. However, there are no published studies on dysphagia in this group of patients, which would include the individual phases or the safety and effectiveness of the swallowing process. OBJECTIVE:The main objective of this study was to assess the prevalence of swallowing disorders and to characterize them based on subjective assessment by the study subjects with multiple sclerosis and Devic's syndrome. METHOD:The study included 72 patients (47 F, 25 M). Patients at risk of dysphagia were identified using the DYMUS, EAT-10 and SDQ questionnaires. To assess the type of oral- and pharyngeal-stage dysphagia, questions in the questionnaires were classified into groups according to symptoms typical of each stage. RESULTS:The risk of dysphagia and the need for instrumental examination were identified in 37.5% of the study subjects. Pharyngeal-stage dysphagia (repeated swallowing, increased effort of swallowing, cough, a feeling of food sticking in the throat) was reported to occur at a significantly higher frequency. However, no differences were found between difficulty in swallowing liquids and difficulty in swallowing solid food. CONCLUSION:There is a need for further research, which should include a detailed dysphagia-oriented diagnosis, with a view to gaining a detailed insight into the pathophysiology of deglutition in this group of patients.

METHODS:BACKGROUND:Anti-MAG polyneuropathy (anti-MAG PN) is an immune-mediated peripheral sensorimotor neuropathy characterized by distal demyelination and ataxia. However, this disorder, unlike other immune-mediated neuropathies, is difficult to treat in most cases. METHOD:We retrospectively collected all anti-MAG PN patients followed in two hospitals for a period of 12 years to determine prognostic factors, especially those that indicated a good response to the various therapeutic strategies used. RESULTS:Forty-seven patients were included in the study; of these, 61% had a classical 'distal demyelinating pattern', 34.2% had a 'CIDP-like pattern', and the others had an 'axonal pattern'. The most commonly used treatments were intravenous immunoglobulin (IVIg) as the first-line treatment and rituximab as the second- or third-line treatment. No prognostic factor was identified for IVIg, but electrophysiological parameters at onset were better in patients with a good response to rituximab than in non-responder patients, even though mild or high disability was observed in nearly half the patients at last examination. CONCLUSION:Even though disability seems to progress in most cases despite the treatments used, our results suggest that an early electrophysiological reduction in sensory nerves could be considered a 'red flag' for the prompt initiation of rituximab to try to delay long-term disability.

METHODS::We report a case of neuromyelitis optica spectrum disorders (NMOSD) with complete loss of vision in the left eye in a patient who was not satisfied with the effect of methylprednisolone therapy, which was improved by HA280 immunoadsorption (IA) therapy. HA280 is a relatively cheaper IA column (made in China) often used in the treatment of rheumatoid immune-related diseases. HA280 can effectively remove inflammatory markers in serum. However, whether the HA280 IA column is suitable for NMOSD is unknown. This case suggests that the HA280 IA column has a potential therapeutic effect on NMOSD and may be an alternative treatment for steroid-resistant NMOSD. There may be therapeutic targets other than anti-AQP4 antibody. Identifying the inflammatory substances that could be removed to contribute to NMOSD recovery is worthy of further study, and the results could provide new ideas for acute NMOSD treatment. Moreover, the HA280 IA column is relatively cheap and allows lower-income families to use it.

METHODS:BACKGROUND:Determining the frequency of longitudinally-extensive transverse myelitis (LETM: T2-lesion ≥3 vertebral segments) in multiple sclerosis (MS) is essential to assess its utility in differentiating from aquaporin-4-IgG (AQP4-IgG) positive neuromyelitis optica spectrum disorder (NMOSD) and myelin-oligodendrocyte-glycoprotein-IgG (MOG-IgG) myelitis. We sought to determine the frequency of LETM in MS during a myelitis attack. METHODS:We identified Olmsted County (MN, USA) residents on 12/31/2011 with inflammatory demyelinating disease. Inclusion criteria were: 1) Clinical myelitis episode accompanied by a new spinal magnetic resonance imaging (MRI) lesion (≤6 weeks from onset); 2) MS diagnosis by 2010 McDonald criteria; 3) Seronegative for AQP4-IgG and MOG-IgG. MRI characteristics were determined. RESULTS:Sixty-seven patients (median age at myelitis: 41 years [range, 16-65]; 76% females) with 92 myelitis attacks accompanied by a new MRI spinal cord lesion were identified. The frequency of LETM was 0%. The median T2-hyperintense lesion length in vertebral segments was 1.0 (range, 0.5-2.5) and 82/92 (89%) were peripheral in location on axial sequences; 58% had associated gadolinium enhancement. Two patients (2% of attacks) had multiple short lesions resembling LETM on sagittal images but axial sequences confirmed multiple non-contiguous short lesions. CONCLUSION:LETM is rare in adult MS myelitis and its presence should prompt evaluation for AQP4-IgG, MOG-IgG or other etiologies. Careful scrutiny of axial images is important as coalescence of multiple short lesions may lead to the artifactual appearance of an LETM.

METHODS:OBJECTIVES:Neuroinflammation represents one of the two major pathological components of multiple sclerosis (MS). The aim of our study was to find the role of the late pro-inflammatory cytokine HMGB1 (high mobility group box) in MS pathogenesis. SUBJECTS AND METHODS:A total of 165 patients from three MS centers in Slovakia were enrolled in the study. Patients underwent a complex clinical investigation and their plasma levels of HMGB1 were analyzed by a sandwich ELISA test. RESULTS:MS patients had 4.5 times higher plasma level of HMGB1 (median, 13.529 ng/mL; IQR = 2.330-113.45) than healthy controls (median, 2.999 ng/mL; IQR = 1.686-9.844; P < 0.0001). The concentrations of HMGB1 were significantly associated with increased number of affected areas diagnosed by MRI (P < 0.0001) (the median for one affected area, 4.205 ng/mL; median for five affected areas, 17.843 ng/mL; P < 0.05). Patients with at least one active lesion in any of the affected areas in the brain had significantly higher plasma levels of HMGB1 (median, 20.118 ng/mL; IQR, 3.693-100.12) than those without any active lesion (median, 16.695 ng/mL; IQR, 3.255-113.45; P < 0.0235). We found also a very highly significant association of HMGB1 plasma levels with clinical condition expressed as EDSS (expanded disability status scale) (P < 0.0001); patients with higher EDSS had higher levels of HMGB1 (EDSS ≤ 2.5, 11.648 ng/mL vs. EDSS ≥ 3, 17.549 ng/mL; P = 0.0115). CONCLUSION:Our results suggest chronic low-grade inflammation in MS patients that correlates with clinical conditions of MS patients, and for HMGB1 as a possible target molecule in future therapy.