神经领域-神经系统自身免疫性疾病方向

METHODS:OBJECTIVE:To investigate the difference of fatigue and pain in patients with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). METHODS:Data from the Modified Fatigue Impact Scale (MFIS) and Pain Effects Scale (PES) were compared between 51 NMOSD and 85 MS patients. Each score was compared in each disease group with or without clinical abnormalities. Since almost no MS patients are without brain magnetic resonance imaging abnormalities, volumetry analysis by the Lesion Segmentation Tool and statistical parametric mapping 12 were added to obtain total lesion volume and intracranial volume in MS patients, and the correlations between total lesion volume/intracranial volume and each score were investigated. RESULTS:Compared to the MS group, the NMOSD group showed a higher PES score (median, 15.0 vs. 7.0, P = 0.045), no difference in MFIS, and an increased percentage of patients with extended spinal cord lesions (58.8% vs. 8.2%, P < 0.001). Moreover, NMOSD and MS patients with extended spinal cord lesions tended to demonstrate higher PES scores than those without. A positive correlation between MFIS and PES were found in patients with NMOSD and MS. On the other hand, MS patients showed a higher percentage of brain abnormalities (80.4% vs. 97.6%, P = 0.001) and a positive correlation between total lesion volume/intracranial volume and MFIS (Spearman's ρ = 0.50, P = 0.033). CONCLUSIONS:The origin of fatigue may be associated with spinal cord lesions causing pain in NMOSD patients, but with brain lesions in MS patients.

METHODS:OBJECTIVE:Investigate the effects of photobiomodulation (PBM) on the expression of IL-10 and nitrites in individuals with Relapsing-Remitting multiple sclerosis (MS), as these biomarkers play a fundamental role in the physiopathology of the disease. The modulation of IL-10 and nitrites through treatment with PBM may be a novel treatment modality for MS. METHODS:A randomized, uncontrolled, clinical trial was conducted involving 14 individuals with a diagnosis of Relapsing-Remitting MS and a score of up to 6.0 on the Expanded Disability Status Scale (EDSS). THE PARTICIPANTS WERE RANDOMIZED TO TWO GROUPS:Group 1 -PBM in the sublingual region; Group 2 -PBM over the radial artery. Irradiation was administered with a wavelength of 808 nm and output power of 100 mW for 360 seconds twice a week, totaling 24 sessions. Peripheral blood was analyzed for the determination of serum levels of IL-10 and nitrites. RESULTS:After treatment with PBM, the expression of IL-10 increased in both the sublingual group (pre-treatment: 2.8 ± 1.4 pg/ml; post-treatment: 8.3 ± 2.4 pg/ml) and the radial artery group (pre-treatment: 2.7 pg/ml ± 1.4; post-treatment: 11.7 ± 3.8 pg/ml). In contrast, nitrite levels were not modulated in the sublingual group (pre-treatment: 65 ± 50 nmol/mg protein; post-treatment: 51 ± 42 nmol/mg protein) or the radial artery group (pre-treatment: 51 ± 16 nmol/mg protein; post-treatment: 42 ± 7 nmol/mg protein). CONCLUSION:Treatment with PBM positively modulated the expression of IL-10 but had no effect on nitrite levels. Further studies should be conducted with a larger sample and a control group, as PBM may be a promising complementary treatment for the management of MS. This trial is registered at ClinicalTrials.gov. Identifier: NCT03360487.

METHODS:BACKGROUND:Blood-brain barrier (BBB) disruption and ensuing immune activation are central to the pathogenesis of central nervous system (CNS) inflammatory diseases. However, the influence of BBB permeability on the clinical signs and prognosis of newly diagnosed neuromyelitis optica spectrum disorder (NMOSD) has not been examined. We investigate the relationships between BBB permeability as showed by the albumin quotient (qalb) and clinical features of NMOSD. METHODS:Demographic and clinical data of 46 patients, including peripheral blood (PB) measures (serum albumin concentration and total leukocyte, neutrophil, total lymphocyte, CD4+ T cell, and CD8+ T cell counts, complement C3 and C4 concentrations, AQP4-IgG titer),autoimmune antibody titers (ANA/SSA/SSB/Ro-52), and cerebrospinal fluid (CSF) parameters (total leukocyte count, total protein and albumin concentrations, AQP4-IgG titer), were compared between qalb(BBB permeability) increased and normal groups. Complete measures were not obtained from 9 patients, but all other measures were included in the analysis. RESULTS:According to the calculated qalb, 15 patients with albumin quotient (qalb) > (4 + age/15) × 10-3 were assigned to the qalb increased (high BBB permeability) group (33%) and the remainder to the qalb normal group. Compared to the qalb normal group, the qalb increased group exhibited significantly lower serum albumin (P=0.001) and CD4+ T cell count (P=0.044), CD8+ T cell count (P=0.014), and total T lymphocyte count (P=0.016). The qalb increased group proved higher CSF albumin, total protein, leukocyte count, and IgG titer (all P=0.000). Optic neuritis and optic nerve abnormalities on magnetic resonance images were also more frequent in the qalb increased group (P=0.037 and 0.038, respectively). Patients in the qalb increased group showed significantly poorer treatment response as indicated by the lower post-treatment change in Expanded Disability Status Scale (EDSS) score compared to the qalb normal group. CONCLUSIONS:BBB permeability is strongly associated with the clinical features and treatment response of newly diagnosed NMOSD. The qalb is a potentially valuable indicator of disease severity and an index to guide personalized treatment.

METHODS:INTRODUCTION:An Australian case series study demonstrated the effectiveness of the REsilience and Activities for every DaY for people with multiple sclerosis (READY for MS), a resilience group training program based on Acceptance and Commitment Therapy, in improving quality of life in people with MS. This study aimed to evaluate the feasibility and acceptability of the Italian READY for MS program, and to preliminary assess its efficacy when compared to an active control intervention (group relaxation). METHODS:Single-blind phase II randomized controlled trial (RCT) and nested qualitative study (ISRCTN registration number: 38971970). Health-related quality of life (primary study outcome), mood, resilience, psychological flexibility and its protective factors were measured at baseline, after seven, 12 and 24 weeks. READY participants completed the purpose-built satisfaction questionnaire after 12 weeks. After trial completion, the control group also received READY. One-to-one participant interviews were conducted within three months of finishing the READY groups. RESULTS:Four intervention groups were conducted with 39 participants (20 READY, 19 relaxation). Two patients (READY) withdrew before beginning the intervention due to unexpected work commitments. Feasibility and acceptability of READY were good, with high participant engagement and satisfaction. No statistical effects of READY were detected vs relaxation. Thirty participants were interviewed (18 READY; 12 relaxation + READY). Content data analysis revealed seven overarching themes: "Attitudes towards participation"; "Perceptions of program composition"; "Program impacts on life domains"; "Program active elements"; "Program improvement trajectories"; "Program differences and similarities"; "Suggested READY improvements". CONCLUSION:READY was well accepted by MS patients with varied socio-demographic and clinical characteristics. Qualitative (but not quantitative) data provided evidence in favour of READY. Our findings will inform methodological and intervention refinements for the multi-centre RCT that will follow.

METHODS:BACKGROUND:The aim of this study was to explore the association between CD24 Ala/Val polymorphism and susceptibility of multiple sclerosis (MS). METHODS:A comprehensive literature search for relevant studies was performed on google scholar, PubMed, Web of science, Embase, the Chinese National Knowledge Infrastructure and the Chinese Biology Medicine. This meta-analysis was conducted using the STATA 11.0 software and the pooled odds ratio with 95% confidence interval was calculated. RESULTS:Seven case-control studies were included in this meta-analysis. The results showed significant association between CD24 Ala/Val polymorphism and susceptibility to MS. Stratified analysis by areas also showed significant association in Asians. However, no association was found in Europeans. CONCLUSION:This study suggested that the CD24 Val allele was associated with an increased risk of MS and larger-scale studies of populations are needed to explore the role of CD24 Ala/Val polymorphism during the pathogenesis of MS.

METHODS::The coronavirus 2019 (COVID-19) pandemic has potential to disproportionately and severely affect patients with neuromuscular disorders. In a short period of time, it has already caused reorganization of neuromuscular clinical care delivery and education, which will likely have lasting effects on the field. This article reviews (1) potential neuromuscular complications of COVID-19, (2) assessment and mitigation of COVID-19-related risk for patients with preexisting neuromuscular disease, (3) guidance for management of immunosuppressive and immunomodulatory therapies, (4) practical guidance regarding neuromuscular care delivery, telemedicine, and education, and (5) effect on neuromuscular research. We outline key unanswered clinical questions and highlight the need for team-based and interspecialty collaboration. Primary goals of clinical research during this time are to develop evidence-based best practices and to minimize morbidity and mortality related to COVID-19 for patients with neuromuscular disorders.

METHODS::Novel outbreak with coronavirus 2019 began since 31 December 2019. Coronaviruses can cause multiple systemic infections that respiratory complications are the most obvious symptoms. In this report, we describe the symptoms of Guillain Barre syndrome (GBS) in one infected patient with COVID-19, for the first time. We reported a 65-years- old male patient with complaints of acute progressive symmetric ascending quadriparesis. Two weeks prior to hospitalization, the patient suffered from cough, fever, and RT-PCR was reported positive for COVID-19 infection. The electrodiagnostic test showed that the patient is an AMSAN variant of GBS. COVID-19 stimulates inflammatory cells and produces various inflammatory cytokines and as a result, it creates immune-mediated processes. GBS is an immune-mediated disorder and molecular mimicry as a mechanism of autoimmune disorder plays an important role in creating it. It is unclear whether COVID-19 induces the production of antibodies against specific gangliosides. Further investigations should be conducted about the mechanism of GBS in patients with COVID-19, in the future.

METHODS::To identify the risk factors of pneumonia in patients with Anti-N-methyl-D-aspartate (Anti-NMDA) receptor encephalitis.This is a retrospective study.Department of Neurology in West China Hospital of Sichuan University.Patients with a definitive diagnosis of anti-NMDA receptor encephalitis.Risk factors associated with pneumonia were examined by bivariate analysis and multivariate logistic regression model.A total of 104 patients were included in this study, of which 41% patients (n = 43) were diagnosed with pneumonia at 7 days (range: 4-40 days) after admission. The occurrence of pneumonia was associated with prolonged hospital stays, a higher rate of poor outcome, and extra healthcare costs. Risk factors associated with pneumonia included Glasgow coma scale score (GCS), abnormal movements and hypokalemia.Pneumonia is a common complication in anti-NMDA receptor encephalitis. In the present study, we found that disorders of consciousness, abnormal movements, and hypokalemia were independent risk factors for pneumonia in inpatients with anti-NMDA receptor encephalitis. Pneumonia prolongs the patients' hospital stay, hospitalization expenditures, and affects the patients' prognosis.

METHODS:BACKGROUND:The aim of this systematic review with meta-analysis is to determine the efficacy and security of acupuncture in treatment of ocular myasthenia gravis and find out whether or not the quick short-term efficacy of acupuncture exists. METHODS:The following electronic databases will be searched by 2 independent reviewers: PubMed, Cochrane Library, EMBASE, Springer, China National Knowledge Infrastructure, Wanfang, and Chinese Biomedical Literature Database. All randomized controlled trials on acupuncture for ocular myasthenia gravis published in electronic databases from inception to March 1, 2020, with language restricted in Chinese and English will be included in the study.Methodologic quality is assessed by 2 blinded reviewers independently screen and score the articles using the PEDro scale and the Cochrane Collaboration risk of bias tool. A meta-analysis was performed when there is sufficient clinical homogeneity in at least 2 studies. The Grading of Recommendations Assessment, Development and Evaluation approach is used to rate the body of evidence in each meta-analysis. When the quantitive evaluation is not available, a qualitative description of the results of single study is provided. RESULTS:An evidence of variety of acupuncture treatment methods for treating ocular myasthenia gravis will be illustrated using subjective reports and objective measures of performance. The primary outcomes consisted of effective rate, MGFA PIS, QMG, and MG-composite. Secondary outcomes involve clinical absolute and relative score, titers of AchR antibodies, and the side effects. The treatment frequency and courses will be measured. CONCLUSION:This protocol will present the evidence of whether acupuncture is an effective and safe intervention for ocular myasthenia gravis. TRIAL REGISTRATION NUMBER:CRD42019141325.

METHODS:BACKGROUND:The Zika virus (ZIKV) has been associated with Guillain-Barré syndrome (GBS) in epidemiological studies. Whether ZIKV-associated GBS is related to a specific clinical or electrophysiological phenotype has not been established. To this end, we performed a systematic review and meta-analysis of all published studies on ZIKV-related GBS. METHODS:We searched Pubmed, EMBASE and LILACS, and included all papers, reports or bulletins with full text in English, Spanish or Portuguese, reporting original data of patients with GBS and a suspected, probable or confirmed recent ZIKV infection. Data were extracted according to a predefined protocol, and pooled proportions were calculated. RESULTS:Thirty-five studies were included (13 single case reports and 22 case series, case-control or cohort studies), reporting on a total of 601 GBS patients with a suspected, probable or confirmed ZIKV infection. Data from 21 studies and 587 cases were available to be summarized. ZIKV infection was confirmed in 21%, probable in 22% and suspected in 57% of cases. ZIKV PCR was positive in 30% (95%CI 15-47) of tested patients. The most common clinical features were: limb weakness 97% (95%CI 93-99), diminished/absent reflexes 96% (95%CI 88-100), sensory symptoms 82% (95%CI 76-88), and facial palsy 51% (95%CI 44-58). Median time between infectious and neurological symptoms was 5-12 days. Most cases had a demyelinating electrophysiological subtype and half of cases were admitted to the Intensive Care Unit (ICU). Heterogeneity between studies was moderate to substantial for most variables. CONCLUSIONS:The clinical phenotype of GBS associated with ZIKV infection reported in literature is generally a sensorimotor demyelinating GBS with frequent facial palsy and a severe disease course often necessitating ICU admittance. Time between infectious and neurological symptoms and negative PCR in most cases suggests a post-infectious disease mechanism. Heterogeneity between studies was considerable and results may be subject to reporting bias. This study was registered on the international Prospective Register of Systematic Reviews (CRD42018081959).