Inflammatory Demyelinating Diseases Of Central Nervous System Research Articles

Top 100 Publications as Measured by Altmetrics in the Field of Central Nervous System Inflammatory Demyelinating Disease.

Background Altmetrics analyze the visibility of articles in social media and estimate their impact on the general population. We performed an altmetric analysis of articles on central nervous system inflammatory demyelinating disease (CIDD) and investigated its correlation with citation analysis. Methods Articles in the 91 journals comprising the “clinical neurology,” “neuroscience,” and “medicine, general, and internal” Web of Science categories were searched for their relevance to the CIDD topic. The Altmetric Explorer database was used to determine the Altmetric.com Attention Score (AAS) values of the selected articles. The papers with the top 100 AAS values were characterized. Results Articles most frequently mentioned online were primarily published after 2014 and were published in journals with high impact factors. All articles except one were dealt with the issue of multiple sclerosis. Most were original articles, but editorials were also common. Novel treatments and risk factors are the most frequent topics. The AAS was weakly correlated with journal impact factors; however, no link was found between the AAS and the number of citations. Conclusions We present the top 100 most frequently mentioned CIDD articles in online media using an altmetric approach. Altmetrics can rapidly offer alternative information on the impact of research based on a broader audience and can complement traditional metrics.

Inflammatory demyelinating diseases of the central nervous system in Niger

BackgroundIn sub-Saharan Africa (SSA), few studies have been reported on inflammatory demyelinating diseases of the central nervous system (CNS). Neuromyelitis optica spectrum disorders (NMOSD) seems to be the most frequent inflammatory demyelinating disease of CNS in sub-Saharan Africans or people of sub-Saharan African descent. MethodsWe report the observations of seven patients from Niger diagnosed with inflammatory demyelinating diseases of CNS over a period of 21 years (1996–2017). ResultsThey were four women and three men aged 19 to 66 years (mean age: 37 years), with no known past medical history. Four patients were diagnosed with NMOSD (2 men and 2 women) and the three other patients with multiple sclerosis (MS, 2 women and 1 man). The three patients diagnosed with MS had the relapsing-remitting form. The cerebrospinal fluid study revealed the presence of oligoclonal bands in the three patients. Of the patients diagnosed with NMOSD, two patients negative anti-aquaporin 4 antibodies (anti-MOG antibodies not done), one of whom had bilateral optic neuritis (ON) with longitudinally extensive transverse myelitis (LETM) and the other unilateral ON with LETM. Two patients with MS were treated with interferon beta-1a and the third patient with azathioprine. The Expanded Disability Status Scale ranged from 1 to 2 in these three patients at the time of initiation of background treatment. Azathioprine was the treatment prescribed in the four patients with NMOSD. We did not find any case of acute disseminated encephalomyelitis. ConclusionOur case series suggests the rarity of inflammatory demyelinating diseases of CNS in Niger, and NMOSD seems to be more frequent than MS.

Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: Follow-up study

Tumefactive demyelinating disease is a rare inflammatory demyelinating disease (IDD) of the central nervous system (CNS). The literature lacks a clear and consistent description of the clinical and radiological spectrum of this disorder, and few Chinese cases have been studied. Here we report 17 Chinese patients, with pathology confirmed CNS IDD, who had distinct clinical and imaging features from those in previous reports. Median age at onset was 47years, with a female to male ratio of 1.1:1. Multifocal lesions were present in nine cases (53%) on their pre-biopsy magnetic resonance imagings (MRIs), with locations predominantly involving periventricular white matter (41%), subcortical white matter (41%), juxtacortical regions (41%), and cortical gray matter (35%). Moderate to severe perilesional edema and/or mass effect were present in 35% of cases. A variety of enhancement patterns were observed; most were heterogenous, including ring-like, patchy, venular-like, nodular, punctate, and diffuse in a decreased frequency. Perilesional restriction on diffusion-weighted images (DWI) were evident in 70% cases. Clinical course prior to biopsy was a first neurological event in 82% cases. During a median follow-up of 4.1years, 76% of cases remained as isolated demyelinating syndrome, and 70% experienced a total or near-total recovery regardless of whether they received immunotherapy. Further studies are needed, especially concerning series with pathological confirmation and long-term follow-up information.

B-Cell Depletion Attenuates White and Gray Matter Pathology in Marmoset Experimental Autoimmune Encephalomyelitis

This study investigated the effect of CD20-positive B-cell depletion on central nervous system (CNS) white and gray matter pathology in experimental autoimmune encephalomyelitis in common marmosets, a relevant preclinical model of multiple sclerosis. Experimental autoimmune encephalomyelitis was induced in 14 marmosets by immunization with recombinant human myelin oligodendrocyte glycoprotein in complete Freund adjuvant. At 21 days after immunization, B-cell depletion was achieved by weekly intravenous injections of HuMab 7D8, a human-anti-human CD20 antibody that cross-reacts with marmoset CD20. In vivo magnetic resonance imaging showed widespread brain white matter demyelination in control marmosets that was absent in CD20 antibody-treated marmosets. High-contrast postmortem magnetic resonance imaging showed white matter lesions in 4of the 7 antibody-treated marmosets, but these were significantly smaller than those in controls. The same technique revealed gray matter lesions in 5 control marmosets, but none in antibody-treated marmosets. Histologic analysis confirmed that inflammation, demyelination, and axonal damage were substantially reduced in brain, spinal cord, and optic nerves of CD20 antibody-treated marmosets. In conclusion, CD20-postive B-cell depletion by HuMab 7D8 profoundly reduced the development of both white and gray matter lesions in the marmoset CNS. These data underline the central role of B cells in CNS inflammatory-demyelinating disease.

Beneficial Plasma Exchange Response in Central Nervous System Inflammatory Demyelination

Plasma exchange (PLEX) is a beneficial rescue therapy for acute, steroid-refractory central nervous system inflammatory demyelinating disease (CNS-IDD). Despite the approximately 45% PLEX response rate reported among patients with CNS-IDD, determinants of interindividual differences in PLEX response are not well characterized. To perform an exploratory analysis of clinical, radiographic, and serological features associated with beneficial PLEX response. Historical cohort study. Neurology practice, Mayo Clinic College of Medicine, Rochester, Minnesota. Patients All Mayo Clinic patients treated with PLEX between January 5, 1999, and November 12, 2007, for a steroid-refractory CNS-IDD attack. The PLEX response in attack-related, targeted neurological deficit(s) assessed within the 6-month period following PLEX. We identified 153 patients treated with PLEX for a steroid-refractory CNS-IDD, of whom 90 (59%) exhibited moderate to marked functional neurological improvement within 6 months following treatment. Pre-PLEX clinical features associated with a beneficial PLEX response were shorter disease duration (P = .02) and preserved deep tendon reflexes (P = .001); post-PLEX variables included a diagnosis of relapsing-remitting multiple sclerosis (P = .008) and a lower Expanded Disability Status Scale score (P < .001) at last follow-up. Plasma exchange was less effective for patients with multiple sclerosis who subsequently developed a progressive disease course (P = .046). Radiographic features associated with a beneficial PLEX response were presence of ring-enhancing lesions (odds ratio = 4.00; P = .03) and/or mass effect (odds ratio = 3.00; P = .02). No association was found between neuromyelitis optica-IgG serostatus and PLEX response. We have identified clinical and radiographic features that may aid in identifying patients with fulminant, steroid-refractory CNS-IDD attacks who are more likely to respond to PLEX.

Negative association of Epstein-Barr virus or herpes simplex virus-1 with tumefactive central nervous system inflammatory demyelinating disease

Central nervous system (CNS) demyelination has been suggested to be associated with infections caused by the Epstein-Barr virus (EBV) or herpes simplex virus (HSV)-1. CNS inflammatory demyelinating disease (IDD) rarely presents as a large lesion. We evaluated samples of serum and cerebrospinal fluid (CSF) by enzyme-linked immunosorbent assay to detect recent infection with these viruses and analyzed CSF and brain specimens by polymerase chain reaction (PCR) or immunohistochemical studies for evidence of these viruses in three patients with biopsy-proven CNS IDD. The results of PCR tests for EBV and HSV in CSF or brain specimens were negative. Elevated anti-EBV or -HSV antibody levels were not found in serum or CSF in any patient. Immunohistochemical studies showed that IDD lesions were negative for latent membrane protein (LMP)-1, Epstein-Barr nuclear antigen (EBNA)-2, and EBNA noncoding RNA (EBER)-1. These results suggest a negative association between CNS IDD and EBV or HSV.

Autoantibodies in inflammatory demyelinating diseases of the central nervous system.

The determination of disease-specific autoantibodies (Abs) is a challenge in any autoimmune disease. The significance of Abs detected in inflammatory demyelinating diseases (IDD) of the central nervous system (CNS), such as multiple sclerosis (MS), is still unclear. Histopathological reports have demonstrated that a humoral (Abs)-mediated pattern of demyelination is detected in >50% of MS patients and is consistently associated with active demyelination. The observation that these patients specifically respond to plasmapheresis reinforces the hypothesis of a specific humoral MS subtype. One of the most intensively studied antigen targets in MS is a glycoprotein of the myelin sheath called the Myelin Oligodendrocyte Glycoprotein (MOG). Recent advances have shown that epitope specificity of MOG is crucial in terms of specificity of the Ab response. Several other auto-Abs, including anti-myelin, oligodendrocyte and neuronal Abs have been studied in MS. These auto-Abs may have pathogenic or protective properties, but could also have no functional role. Recently, the demonstration of a highly specific auto-Ab in an IDD of the CNS called neuromyelitis optica (NMO), directed against the aquaporin-4 (AQP-4) located at the blood brain barrier (BBB), has allowed a refinement of the diagnostic criteria of NMO and classification of this disease as an autoimmune channelopathy. These recent advances have reinforced the interest in tracking the role of the humoral response in the different IDD of the CNS.