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Background A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). Objective To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis ( n  = 50) as well as attack and long-term treatment outcomes. Methods Retrospective multicenter study. Results The sex ratio was 1:2.8 (m:f). Median age at onset was 31 years (range 6-70). The disease followed a multiphasic course in 80% (median time-to-first-relapse 5 months; annualized relapse rate 0.92) and resulted in significant disability in 40% (mean follow-up 75 ± 46.5 months), with severe visual impairment or functional blindness (36%) and markedly impaired ambulation due to paresis or ataxia (25%) as the most common long-term sequelae. Functional blindness in one or both eyes was noted during at least one ON attack in around 70%. Perioptic enhancement was present in several patients. Besides acute tetra-/paraparesis, dysesthesia and pain were common in acute myelitis (70%). Longitudinally extensive spinal cord lesions were frequent, but short lesions occurred at least once in 44%. Fourty-one percent had a history of simultaneous ON and myelitis. Clinical or radiological involvement of the brain, brainstem, or cerebellum was present in 50%; extra-opticospinal symptoms included intractable nausea and vomiting and respiratory insufficiency (fatal in one). CSF pleocytosis (partly neutrophilic) was present in 70%, oligoclonal bands in only 13%, and blood-CSF-barrier dysfunction in 32%. Intravenous methylprednisolone (IVMP) and long-term immunosuppression were often effective; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal. Full recovery was achieved by plasma exchange in some cases, including after IVMP failure. Breakthrough attacks under azathioprine were linked to the drug-specific latency period and a lack of cotreatment with oral steroids. Methotrexate was effective in 5/6 patients. Interferon-beta was associated with ongoing or increasing disease activity. Rituximab and ofatumumab were effective in some patients. However, treatment with rituximab was followed by early relapses in several cases; end-of-dose relapses occurred 9-12 months after the first infusion. Coexisting autoimmunity was rare (9%). Wingerchuk’s 2006 and 2015 criteria for NMO(SD) and Barkhof and McDonald criteria for multiple sclerosis (MS) were met by 28%, 32%, 15%, 33%, respectively; MS had been suspected in 36%. Disease onset or relapses were preceded by infection, vaccination, or pregnancy/delivery in several cases. Conclusion Our findings from a predominantly Caucasian cohort strongly argue against the concept of MOG-IgG denoting a mild and usually monophasic variant of NMOSD. The predominantly relapsing and often severe disease course and the short median time to second attack support the use of prophylactic long-term treatments in patients with MOG-IgG-positive ON and/or myelitis.

Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been described in patients with neuromyelitis optica spectrum disorders (NMOSD) without aquaporin-4 antibodies (AQP4-IgG). We aimed to identify the proportion of AQP4-IgG-negative NMOSD patients who are seropositive for MOG-IgG. In a cross sectional study, we reviewed all patients seen in the National NMO clinic over the last 4 years (after the availability of MOG-IgG testing), including clinical information, MRI, and antibody tests. 261 unique patients were identified. 132 cases satisfied the 2015 NMOSD diagnostic criteria. Of these, 96 (73%) were AQP4-IgG positive and 36 (27%) were AQP4-IgG negative. These 36 patients were tested for MOG-IgG and 15/36 (42%) tested positive. 20% (25/125) of the patients who did not satisfy NMOSD criteria had MOG-IgG. Approximately half of seronegative NMOSD is MOG-Ig seropositive and one in five of non-NMOSD/non-MS demyelination is MOG-IgG positive. Since MOG-associated demyelinating disease is likely different from AQP4-IgG disease in terms of underlying disease mechanisms, relapse risk and possibly treatment, testing for MOG-IgG in patients with AQP4-IgG-negative NMOSD and other non-MS demyelination may have significant implications to management and clinical trials.

The comparative clinical and demographic features of neuromyelitis optica (NMO) are not well known. In this review we analyzed peer-reviewed publications for incidence and prevalence, clinical phenotypes, and demographic features of NMO. Population-based studies from Europe, South East and Southern Asia, the Caribbean, and Cuba suggest that the incidence and prevalence of NMO ranges from 0.05–0.4 and 0.52–4.4 per 100,000, respectively. Mean age at onset (32.6–45.7) and median time to first relapse (8–12 months) was similar. Most studies reported an excess of disease in women and a relapsing course, particularly in anti-aquaporin 4 antibody (anti AQP4-IgG)-positive patients. Ethnicity may have a bearing on disease phenotype and clinical outcome. Despite limitations inherent to the review process, themes noted in clinical and demographic features of NMO among different populations promote a more global understanding of NMO and strategies to address it.

Background We aimed to assess the clinical, paraclinical, imaging and prognostic features of patients with late-onset neuromyelitis optica spectrum disorder (LO-NMOSD; ≥ 50 years at disease onset) LO-NMOSD, compared with early onset-NMOSD (EO-NMOSD, ≤ 49 years at disease onset), in Latin American (LATAM). Methods We retrospectively reviewed the medical records of patients with NMOSD, as defined using the 2015 validated diagnostic criteria. We included patients from Argentina, Brazil and Venezuela. They were divided into: LO-NMOSD and EO-NMOSD and comparison among the groups were performed. Results Among these 140 NMOSD patients, 24 (17.1%) were LO-NMOSD; 64% were positive for aquaporin-4 antibodies; and 41.5% of this population cohort was non-Caucasian. Severe disability [expanded disability status scale (EDSS) ≥ 6] at the last follow-up and presence of comorbidities were significantly associated with LO-NMOSD, compared with EO-NMOSD. LO-NMOSD patients had a shorter median time to EDSS ≥ 4 than EO-NMOSD patients (46 vs. 60 months; log-rank test p  = 0.0006). Furthermore, we observed a positive correlation between age at onset and EDSS score at the last follow-up (Spearman r  = 0.34, p  < 0.0001). Conclusion LO-NMOSD patients from LATAM developed early severe disability, compared with EO-NMOSD. Therefore, age at onset could have important implications for the long-term prognosis of NMOSD patients.

Background We aimed to characterize the phenotype of neuromyelitis optica spectrum disorder (NMOSD) in the presence and absence of Sjogren's disease (SjD) and to develop a predictive nomogram to evaluate the risk of coexisting SjD within a single tertiary-center cohort of NMOSD patients. Methods Paraclinical and clinical features of patients with SjD were compared between NMOSD patients with SjD and those without SjD. Zstats v1.0 was utilized to randomly allocate participants into a derivation group (108 patients) and a validation group (47 patients) at a ratio of 7:3. Logistic regression analysis was used to assess the effectiveness of our predictive model, and a nomogram was created to illustrate the findings. Results A total of 155 NMOSD patients who were serologically positive for AQP4-IgG were cross-sectionally recruited (70 NMOSD patients with SjD [45.16%] and 85 NMOSD patients without SjD [54.84%]). Independent predictors of coexisting SjD were age upon recruitment ( P  = 0.002); Expanded Disability Status Scale (EDSS) score ( P  = 0.023); blood white blood cell (WBC) count ( P  = 0.049); and rheumatoid factor (RF) ( P  = 0.049), anti-SSA (Ro), and anti-SSB (La) antibody positivity ( P  < 0.001; P  = 0.012). The nomogram had an area under the receiver operating characteristic (ROC) curve (AUC) (95% confidence interval [CI]) of 0.95 (0.89, 1.00) in the derivation cohort and 0.91 (0.79, 1.00) in the validation cohort. Survival curve analysis revealed that the EDSS score in the NMOSD patients with SjD was associated with clinical relapse, and these patients reached an EDSS score of 4.0 earlier than those without SjD. Conclusion NMOSD patients with SjD manifested more severe disease at attack and relapsed earlier than those without SjD. The nomogram established by combining age upon recruitment; EDSS score; blood WBC count; and RF, anti-SSA (Ro), and anti-SSB (La) antibody levels can significantly predict the risk of NMOSD combined with SjD.

BackgroundNeuromyelitis optica spectrum disorder (NMOSD) is a progressive demyelinating disease of the central nervous system that has overlapping symptoms with multiple sclerosis (MS) but differs from it in a variety of ways. Previous studies have reported conflicting results trying to estimate the number of individuals affected by them which is why we designed this systematic review and meta-analysis to estimate the worldwide prevalence and incidence of NMOSD/NMO based on current evidence.MethodsWe searched PubMed, Scopus, EMBASE, Web of Science, and gray literature including references from the identified studies, review studies, and conference abstracts which were published up to February 1, 2022. We used all MeSH terms pertaining to “NMOSD,” “NMO,” and all the terms on “prevalence,” “incidence,” and “epidemiology” to identify the search components. Pooled effect sizes were measured using random-effect model by DerSimonian-Laird.ResultsThe prevalence and incidence rates of NMOSD/NMO ranged from 0.07 to 10 and 0.029 to 0.880 per 100,000 population, respectively. The overall pooled prevalence of NMO per 100,000 population was 1.54 (I2: 98.4%, 95% CI: 1.13–1.96, P< 0.001) based on the 2006 criteria, 1.51 (I2: 99.4%, 95% CI: 1.21–1.81, P < 0.001) based on the 2015 criteria and 2.16 (I2: 89.4%, 95% CI: 1.46–2.86, P < 0.001) based on the 2006/2015 criteria. The overall annual incidence of NMO per 100,000 population was 0.155 (I2: 95%, 95% CI: 0.115–0.195, P < 0.001) based on the 2006 criteria and 0.278 (I2: 100%, 95% CI: 0.135–0.420, P < 0.001) based on the 2015 criteria. The prevalence rates were highest in French West Indies and South Korea, and lowest in Cuba and Australia, based on the 2006 and 2015 criteria, respectively. Also, the highest annual incidence rates were obtained for Sweden and Slovak republic and the lowest for Cuba and Australia based on the 2006 and 2015 criteria, respectively. All estimated rates were higher among females compared to males.ConclusionAlthough rare, NMOSD/NMO impact affected individuals in devastating ways. Several large-scale prospective studies are required to reach a comprehension of the epidemiological aspects of these notorious demyelinating conditions.

Background/aimsTo investigate the clinical features of Chinese patients with seropositive myelin oligodendrocyte glycoprotein antibody (MOG-Ab) optic neuritis (ON) and patients with seropositive aquaporin-4 antibody (AQP4-Ab) ON.MethodsIn this retrospective observational study, sera from patients with demyelinating ON were tested for MOG-Ab and AQP4-Ab with a cell-based assay. Clinical characteristics were compared between MOG-Ab-related ON (MOG-ON) and AQP4-Ab-related ON (AQP4-ON), including visual performances, serum autoantibodies and features on MRI.ResultsA total of 109 affected eyes from 65 patients with demyelinating ON (20 MOG-ON and 45 AQP4-ON) were included. The onset age of MOG-ON was younger than AQP4-ON (MOG-ON: 20.2±17.4 years old, AQP4-ON: 35.6±15.7 years old, P=0.001). Onset severity was not different between these two groups (P=0.112), but patients with MOG-ON showed better visual outcomes (P=0.004). Half of the MOG-ON had a relapsing disease course. Nineteen per cent of patients with AQP4-ON presented coexisting autoimmune disorders, but there were no coexisting autoimmune disorders among patients with MOG-ON. Optic nerve head swelling was more prevalent in patients with MOG-ON (P<0.01). Retrobulbar segment involvement of the optic nerve were more common in patients with MOG-ON according to our MRI findings (P<0.01). Patients with MOG-ON showed longitudinally extensive lesion in 30% and chiasm and optic tract involvement in 5%.ConclusionsMOG-ON is not rare in Chinese demyelinating patients. It underwent a severe vision loss at onset but had relatively better visual recovery than patients with AQP4-ON. MOG-ON might have an unique pathogenesis different from AQP4-ON.

Stronger adaptive immune responses in females can be observed in different mammals, resulting in better control of infections compared to males. However, this presumably evolutionary difference likely also drives higher incidence of autoimmune diseases observed in humans. Here, we summarize sex differences in the most common autoimmune diseases of the central nervous system (CNS) and discuss recent advances in the understanding of possible underlying immunological and CNS intrinsic mechanisms. In multiple sclerosis (MS), the most common inflammatory disease of the CNS, but also in rarer conditions, such as neuromyelitis optica spectrum disorders (NMOSD) or neuronal autoantibody–mediated autoimmune encephalitis (AE), sex is one of the top risk factors, with women being more often affected than men. Immunological mechanisms driving the sex bias in autoimmune CNS diseases are complex and include hormonal as well as genetic and epigenetic effects, which could also be exerted indirectly via modulation of the microbiome. Furthermore, CNS intrinsic differences could underlie the sex bias in autoimmunity by differential responses to injury. The strong effects of sex on incidence and possibly also activity and progression of autoimmune CNS disorders suggest that treatments need to be tailored to each sex to optimize efficacy. To date, however, due to a lack of systematic studies on treatment responses in males versus females, evidence in this area is still sparse. We argue that studies taking sex differences into account could pave the way for sex-specific and therefore personalized treatment.

Background The aim of our study was to estimate the frequency of autoimmune comorbidities, in NMOSD patients from the national Serbian NMOSD Registry. Methods Our study comprises 136 patients with NMOSD, diagnosed according to the NMOSD criteria 2015. At the time of the study, in the Registry were collected demographic and clinical data, including those related to the coexisting comorbidities and pathogenic autoantibodies. Not all patients were tested for all autoimmune antibodies. None of the seronegative aquaporin4-IgG (AQP4-IgG) NMOSD patients, included in the Registry, were positive for the myelin oligodendrocyte glycoprotein IgG. Results: Among 136 NMOSD patients, 50 (36.8%) had at least one associated autoimmune disorder. AQP4-IgG was present in the sera from 106 patients (77.9%), the proportion of NMOSD patients with autoimmune comorbidities being significantly higher in the AQP4-IgG positive subgroup in comparison to the AQP4-IgG negative ( p  = 0.002). AQP4-IgG seropositive NMOSD patients had 5.2-fold higher risk of comorbid autoimmune diseases (OR = 5.2, 95% CI 1.4–18.5, p  = 0.012). The most frequently reported diseases were autoimmune thyroid disease (15.4%), Sjogren’s syndrome (11.0%), systemic lupus erythematosus (5.1%), myasthenia gravis (4.4%), and primary antiphospholipid antibody syndrome (2.9%). Antinuclear antibodies (ANAs) were frequently detected in the subgroup of NMOSD patients tested for this antibody (50/92; 54.3%). The higher frequency of ANAs and anti-extractable nuclear antigen autoantibodies, in the subgroups of AQP4-IgG-positive patients compared to the AQP4-IgG negative, tested for these antibodies, was statistically significant ( p  = 0.009, and p  = 0.015, respectively). Conclusion In conclusion, based on our results, in a defined cohort with European ethnical background, a wide spectrum of autoimmune diseases is frequently associated with AQP4-IgG seropositive NMOSD patients.