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The Japanese Society for Neuroimmunology has been honoring I Clinical and Experimental Neuroimmunology i (CENI) Best Article Award to encourage members of the society to submit their excellent papers to CENI since 2013. The article entitled "Quotient of cerebrospinal fluid/serum immunoglobulin G as a predictive factor for non-responders to intravenous methylprednisolone therapy in patients with relapsing neuromyelitis optica spectrum disorder: Implication for early initiation of plasmapheresis", which appears in I Clin Exp Neuroimmunol i 7: 272-280, 2016, was written by Dr Satoru Oji. In this article, we described that the quotient of cerebrospinal fluid/serum IgG was a predictive factor for the efficacy of intravenous methylprednisolone in patients with relapsing neuromyelitis optica spectrum disorders (NMOSD), which implied the early institution of plasmapheresis. [Extracted from the article]

Objective: This is a critical review of studies aiming to assess the safety and efficacy of monoclonal antibodies as compared with the classical regimen in patients with neuromyelitis optica spectrum disorder. Methods: Various electronic databases were searched for original articles reporting results from the use of monoclonal antibodies in neuromyelitis optica spectrum disorder. The Expanded Disability Status Scale and annualized relapse rate score before and after treatment were the primary effect measures. The pooled standardized mean difference with 95% CI was calculated using the random effects model. The heterogeneity of the included studies was calculated using Cochran's Q test and I2 statistics. Results: Of 36 included studies, meta‐analysis was carried out from 27 studies. The pooled analysis of 1010 patients showed a mean reduction in the mean annualized relapse rate ratio after tocilizumab therapy −2.45 (95% CI −3.13 to −1.77) to be higher compared with rituximab −1.49 (95% CI −1.81 to −1.17). Likewise, the mean reduction in the Expanded Disability Status Scale after tocilizumab was higher −1.10 (95% CI −1.75 to −0.44) compared with rituximab −0.80 (95% CI −1.11 to −0.48). Conclusion: Tocilizumab has a greater effect than rituximab in terms of the reduction of the annualized relapse rate and Expanded Disability Status Scale in neuromyelitis optica spectrum disorder patients. The greater efficacy of tocilizumab could result from its multiple dynamic pharmacodynamics (i.e. its effect on interleukin‐6‐dependent inflammatory processes, involving CD20‐negative plasmablasts, pathogenic T cells and regulatory T cells) and to some degree due to heterogeneity in our study. Satralizumab (monotherapy or add‐on), eculizumab and inebilizumab (monotherapy) are effective in aquaporin‐4‐positive cases with good safety profiles. Ublituximab, bortezomib, bevacizumab and C1‐esterase inhibitors are both effective and safe add‐on drugs. [ABSTRACT FROM AUTHOR]

The article offers information on the papers presented at the 2nd MS Summer College held in Sapporo, Japan on August 1-2, 2015 on topics including genetic and environmental influence on multiple sclerosis (MS), neuromyelitis optica, and astrocyte injury and secondary demyelination. It informs that opening remarks were made by Seiji Kikuchi, and researchers Douglas S. Goodin and Tatsuro Misu delivered lectures on the epidemiology of MS.

Cognitive impairment is one of the most important non‐physical symptoms of patients with multiple sclerosis (MS), and few studies have reported on cognitive impairments in patients with neuromyelitis optica spectrum disorders. Adequate batteries are required to assess cognitive function in MS, and cognitive impairments in patients with MS are likely not adequately evaluated. As cognitive impairment usually affects employment status and activities of daily living in patients with MS and neuromyelitis optica spectrum disorders, the effect of this symptom on daily activities should be closely examined. The present review focuses on the prevalence and characteristics of cognitive impairments in patients with MS and neuromyelitis optica spectrum disorders, as well as the methods used to evaluate cognitive function in such patients. As cognitive impairment usually affects employment status and activities of daily living in patients with multiple sclerosis and neuromyelitis optica spectrum disorders, the effect of this symptom on daily activities should be closely examined. This review focuses on the prevalence and characteristics of cognitive impairments in patients with multiple sclerosis and neuromyelitis optica spectrum disorders, as well as the methods used to evaluate cognitive function in such patients. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To determine the epidemiology and characteristics of transverse myelitis (TM) and neuromyelitis optica spectrum disorders (NMOSD) in Abu Dhabi, United Arab Emirates. Methods: Retrospective chart review at four large government‐run hospitals in Abu Dhabi between 2010 and 2016. Data collected included year of onset, presentation, laboratory results including aquaporin‐4 immunoglobulin G (IgG)/myelin oligodendrocyte glycoprotein IgG antibodies and the occurrence of any relapses. Results: A total of 46 individuals were identified. Of these, 23 (50%) were Emirati citizens. Within the overall group including pediatrics, the crude prevalence rate for monophasic TM was 1.0 per 100 000, and for NMOSD was 0.34 per 100 000. Incidence rates within the overall group for TM and NMOSD were 0.18 per 100 000 and 0.05 per 100 000, respectively. For Emirati citizens aged ≥20 years, the prevalence rate for monophasic TM was 2.46 per 100 000 and 1.76 per 100 000 for NMOSD, and the incidence was 0.57 per 100 000 and 0.17 per 100 000, respectively. The incidence of monophasic TM and NMOSD within the Emirati pediatric population (aged ≤19 years) was 0.18 per 100 000 and 0.06 per 100 000, respectively. The mean age of onset for monophasic TM was 36 years, and for NMOSD was 43 years. Nine patients had a positive aquaporin‐4 IgG or anti‐myelin oligodendrocyte glycoprotein IgG antibody result. Of the 30 participants with available laboratory cerebrospinal fluid analysis, 36.6% had elevated white blood counts (>5.0 × 106/L), and 43% had elevated protein levels. A total of 19 participants had documentation of oligoclonal bands or IgG index, and just four (21%) had either oligoclonal bands or elevated IgG index. Conclusion: The present study describes the epidemiology and characteristics of TM and NMOSD among populations in Abu Dhabi. The adult prevalence rate for Emirati citizens was 2.46 per 100 000 for monophasic TM, and 1.76 per 100 000 for NMOSD. The overall incidence was 0.18 per 100 000 and 0.05 per 100 000, respectively. [ABSTRACT FROM AUTHOR]

The article discusses the 9th Pan-Asian Committee for Treatment and Research in Multiple Sclerosis (PACTRIMS) meeting held at the Millennium Hilton Bangkok Hotel in Bangkok, Thailand from October 27-29, 2016. Topics include the presentation of several topics including the outcomes of neuromyelitis optica spectrum disorders (NMOSD) treatment through rituximab, the T cell repertoire on multiple sclerosis (MS) patients, and neuroimaging approach for demyelinating disease.

Multiple sclerosis ( MS) pathogenesis results from both genetic and environmental factors. Genome-wide association studies ( GWAS) have contributed considerably to our understanding of MS susceptibility through identification of genetic variants influencing risk and quantitation of their effect sizes. Immunologically relevant genes, including genes in the major histocompatibility complex region, are the primary genetic contributors to MS susceptibility. MS prevalence differs according to population, and most GWAS carried out to date have used datasets from people of European descent. Interpretation and application of GWAS results from Europeans to other populations require a good understanding of the genetic characteristics of each population, such as linkage disequilibrium patterns. The heterogeneity in global MS prevalence is partially explained by differences in risk allele frequencies in populations, which are due to the effects of migration, adaptation to new environments and genetic heterogeneity. Genetic studies of different populations can be expected to narrow down the putative causative regions and improve our understanding of MS pathogenesis. The literature record of genetic studies of neuromyelitis optica is much shorter than that of MS. Genes associated with neuromyelitis optica have been reported in the major histocompatibility complex region, as well as at other genetic loci, but only the susceptibility associated with the HLA- DPB1*05:01 and HLA- DRB1*03 alleles, and the association of a CYP7A single nucleotide polymorphism have been replicated. Here, we describe important genetic studies of MS and neuromyelitis optica in Europeans, African Americans, and Asians, and discuss the implications for disease incidence and future directions for genetic research. [ABSTRACT FROM AUTHOR]

Multiple sclerosis ( MS) and neuomyelitis optica ( NMO) are the two main autoimmune and inflammatory diseases of the central nervous system. They have long been considered to be autoimmune diseases that primarily affect the white matter ( WM). However, renewed interest in gray matter ( GM) pathology is emerging. This review focuses on the current knowledge of the details of GM pathology, and its clinical aspects including cognitive impairment in MS and NMO. Patients with MS and NMO have similar cognitive profiles as represented by the latent start of impairment from a very early stage of the disease course. Pathological assessments show that substantial cortical demyelination is prominent in all stages or courses of MS, and cortical neurodegeneration is also present not only in cortical demyelination, but also in normal-appearing GM in MS. In contrast, cortical neurodegeneration is present in NMO and involves unique dynamics of astrocytes, but no cortical demyelination is seen in NMO. Thus, two distinct diseases, MS and NMO, each have unique characteristic features of pathological modalities in the brain. These important hallmarks of MS and NMO might influence neuronal and cognitive function in the brain, given different detrimental effects including cytokine diffusion, disruption of water homeostasis associated with aquaporin-4 dynamics, or other unidentified mechanisms. Although the cause of GM lesions in MS and NMO has not been fully determined, an increase in knowledge of the structure of GM lesions, and the pathomechanisms in MS and NMO brains will result in more targeted pharmacotherapeutic approaches to these diseases. [ABSTRACT FROM AUTHOR]

Objectives There has been no large-scale study of methylprednisolone pulse therapy in Asian patients with multiple sclerosis ( MS) or neuromyelitis optica ( NMO), despite it being widely used for acute relapse. We aimed to clarify treatment response of MS and NMO patients to methylprednisolone pulse therapy and post-pulse oral corticosteroids in real clinical practice in a multicenter study in Japan. Methods Investigators at 28 institutions collected changes in neurological symptoms/signs and Expanded Disability Status Scale ( EDSS) scores before and within 1 week of completion of methylprednisolone pulse therapy carried out in 2010, and after post-pulse oral corticosteroids therapy, by retrospective review of medical records. Results In 345 patients (95.1% of all registered patients), 457 series of methylprednisolone pulse therapy were carried out for treatment of acute relapse. EDSS scores improved by 0.8 ± 1.1 (mean ± SD) after the first course. The second and third courses also produced sufficient improvements (by 0.7 and 0.6, respectively), but much smaller improvements were observed thereafter. The target neurological symptoms and signs improved in 79.5% of patients. Improvement rates were 5-20% lower after a course of pulse therapy than after a series of pulse therapy. A half dose (500 mg/day) produced less improvement than a standard dose (1000 mg/day; 65.9 vs 79.5%). During post-pulse oral corticosteroid therapy, EDSS scores decreased by 0.6 ± 0.9. No significant adverse effects were observed. Conclusions Methylprednisolone pulse therapy is beneficial in nearly 80% of Japanese MS and NMO patients, and EDSS score improvements after therapy are compatible with those in Western MS patients. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica (NMO) is an idiopathic severe necrotic inflammatory disease that mainly causes optic neuritis and myelopathy. Although it usually shows typical magnetic resonance imaging (MRI), the spinal lesions in NMO might sometimes mimic neoplasms or multiple sclerosis (MS) plaques. We present two NMO patients who showed marked hypermetabolism using 18-fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging at spinal cord lesions, corresponding to the gadolinium-enhanced site. As MS plaques typically show hypometabolism, FDG-PET imaging might be a useful and specific tool for the differential diagnosis of MS and NMO. Furthermore, NMO lesions should not be misdiagnosed as neoplasms simply because of the presence of hypermetabolism in FDG-PET imaging. (Clin. Exp. Neuroimmunol. doi: 10.1111/j.1759-1961.2011.00026.x, 2012) [ABSTRACT FROM AUTHOR]

An open study was conducted to evaluate the safety of bevacizumab, antivascular endothelial growth factor antibody, in patients with neuromyelitis optica developing signs of relapse. [ABSTRACT FROM AUTHOR]

I started my career as a clinical neurologist at the Institute of Brain Diseases, Tohoku University School of Medicine in 1975. Since then, I have been closely associated with the Neuroimmunological Research Committee of the Ministry of Health, Labor and Welfare, and the Japanese Society for Neuroimmunology. In this commentary, I will summarize an untold story regarding multiple sclerosis and neuromyelitis optica in Japan. [ABSTRACT FROM AUTHOR]