Diagnosis Of Neuromyelitis Optica Spectrum Disorder Research Articles

The association between dietary total antioxidant capacity and NMO-IgG seropositivity in patients with neuromyelitis optica spectrum disorder

ObjectivesThere is growing evidence highlighting the role of environmental risk factors of NMO-IgG seropositivity in patients with neuromyelitis optica spectrum disorder (NMOSD). The present study investigated the possible association between dietary total antioxidant capacity (TAC) and NMO-IgG seropositivity in NMOSD patients. MethodsFifty-six patients with a definite diagnosis of NMOSD were included in the study. Data on patients’ age, gender, height, weight, cigarette smoking status, and alcohol consumption were collected and recorded. Body mass index (BMI) was also calculated. In addition, dietary habits of patients were evaluated using an adjusted semi-quantitative food frequency questionnaire (FFQ) that consists of 168 food items. Dietary TAC was calculated using the oxygen radical absorption capacity (ORAC) method. Enzyme-linked immunosorbent assay (ELISA) method was used to determine the NMO-IgG serum status. The association between dietary TAC and odds of NMO-IgG seropositivity was measured using the logistic regression analysis. ResultsThe mean of dietary TAC was 8362.8 (μmolTE/1000 kcal) in seronegative patients and 6609.9 (μmolTE/1000 kcal) in seropositive patients and had a significant difference between the mentioned groups of patients (P: 0.02). An inverse association was found between dietary TAC and odds of NMO-IgG seropositivity in all three regression models. The higher dietary intake of antioxidant resulted in significant findings as follows: 92% (95% CI: 0.01–0.53), 97% (95% CI: 0.00–0.34), and 97% (95% CI: 0.00–0.32) lower odds of NMO-IgG seropositivity in the fourth quartiles of the first, the second, and the third regression model, respectively. Moreover, the inverse association between fruit intake and odds of NMO-IgG seropositivity was significant in the third quartile (OR:0.10; 95%CI: 0.01–0.97). ConclusionThe present study indicated a significant inverse association between dietary TAC and NMO-IgG seropositivity of NMOSD patients. As no definite treatment can be offered for NMOSD and nutrition is a modifiable factor in this regard, specification of dietary factors affecting the risk of NMOSD is of great value.

Patient-reported safety and tolerability of the COVID-19 vaccines in persons with rare neuroimmunological diseases

BackgroundThe COVID-19 vaccines are currently recommended for people with rare neuroimmunological diseases such as neuromyelitis optica spectrum disorder (NMOSD), MOG-antibody disease (MOGAD), and transverse myelitis. However, the safety profile of the vaccines in this population is uncertain. ObjectiveTo report real-world safety data of the COVID-19 vaccines in persons with rare neuroimmunological diseases. MethodsAn anonymous survey was distributed to patients recruited on social media. Participants answered general demographic and disease-related questions, and specific questions about their experiences with the COVID-19 vaccines. Results438 participants completed the questionnaire. The median age was 51 (range 18–82 years); 366 were female (83.6%); 102 (23.3%) had associated comorbidities, and 354 (80.1%) were treated with immunotherapies. 242 participants (55.3%) reported a diagnosis of NMOSD; 99 (22.6%) had MOGAD; 79 (18%) had transverse myelitis. 239 participants (66.2%) were younger than 55 years of age. 138 participants (31.5%) reported earlyadverse events. Of these, 93 (67.4%) were < 55 years old, and 45 (32.6%) were > 55 years old (p=0.0086). The most common adverse events were local reactions, including pain, redness, and swelling at the injection site, reported by 155 participants (35.4%). 73 participants (16.7%) reported new or worsening neurological symptoms following the vaccination. Most symptoms occurred within the first week after vaccination and resolved within three days. ConclusionsThis survey indicates an overall favorable safety and tolerability profile of the COVID-19 vaccines among persons with rare neuroimmunological diseases. Longer-term studies are warranted to confirm these data.

Incidence and prevalence of neuromyelitis optica spectrum disorders in Slovakia

ABSTRACT Objective : Neuromyelitis optica spectrum disorders (NMOSDs) are a group of rare, inflammatory, demyelinating diseases that affect the central nervous system. Neither the incidence nor the prevalence of NMOSD has been determined in Slovakia thus far. The aim of this study was to determine both the incidence and the prevalence of NMOSD in Slovakia using the 2015 International Panel of NMOSD diagnosis (IPND) criteria. Methods : We performed a population-based study in Slovakia to estimate both the incidence and the prevalence of NMOSD during the period from 1 January 2006 through 31 December 2019. NMOSD cases were reported from multiple sources and the diagnosis was subsequently verified using the IPND criteria by a joint commitee of three neurologists. The prevalence is reported as number of cases per 100,000 inhabitans and the incidence as number of new cases per 1,000,000 person-years. Age-adjusted rates to the WHO standard population 2005–2025 were also calculated. Results : We identified 63 NMOSD cases. The crude point-prevalence rate was 1.37 (95% CI 1.03–1.71) per 100,000 inhabitants. The crude indidence rate was 0.88 (95% CI 0.65–1.12) per 1,000,000 person-years. The age-adjusted point-prevalence rate was 1.42 (95% CI 1.07–1.84) per 100,000 persons and the age-adjusted incidence rate was 0.96 (95% CI 0.72–1.25) per 1,000,000 person-years. Conclusion : The NMOSD epidemiological situation in Slovakia is comparable to those reported from other Caucasian populations.

P-EV002. Visual evoked potentials in neuromyelitis optica spectrum disorders

Introduction. Neuromyelitis optica spectrum disorders (NMOSD) are rare antibody-mediated disorders of the central nervous system with a predilection for the spinal cord and optic nerves. The clinical utility of evoked potential recordings (EPs) has already been established for multiple sclerosis, in particular, the abnormal visual evoked potentials (VEP) a key criterion in the McDonald diagnostic criteria for MS. On the other hand, there have been few reports on EPs in patients with NMOSD. Methods. The VEP were prospectively performed in 13 patients with NMOSD. All the patients were recruited from the outpatient clinic of a demyelinating diseases center. The recorded was done as recommended by the International Federation of Clinical Neurophysiology. All studies were conducted in accordance with ethical principles after obtaining patient informed consent. Results. We evaluated 12 women with mean age of 42 years old and one man of 25 years old. In 6 the examined eye, a response was not obtained. In the remaining 20, we found a significant increase in P100 latency without amplitude change. The relation to the VEP variable (Normal vs Abnormal), there was a statistical difference in relation to P100 Oc latencies. Conclusion. VEP is a well-known noninvasive tool for investigating the function of the visual system. Decreased amplitude and prolonged latency of VEP recording is believed to reflect the damage of axon and demyelination in the optic nerve. VEP showed a significant increase in P100 latency. VEP is a non-invasive, painless, fast and low-cost exam which provides neurophysiological data for diagnosis of NMOSD. Our results reinforce the concept of VEP evaluation is currently the standard method used to confirm the presence of clinical and subclinical Optic Neuritis.

Magnetic resonance imaging markers for multiple sclerosis and neuromyelitis optica spectrum disorders

AbstractMultiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are two common inflammatory, demyelinating and neurodegenerative diseases of the CNS. It is clinically crucial to differentiate these two diseases since they have distinct treatment strategy and prognosis. MRI has an increasingly important role in the differential diagnosis of NMOSD from MS. Conventional MRI is sensitive and its objective is to demonstrate the lesion characteristics, including lesion number, size, and distribution, while the advanced MRI can offer the structural, functional, and other microscopic information. We herein review the current advances in the understanding of MRI study of the brain, spinal cord, and optic nerve in MS and NMOSD.

Very late onset neuromyelitis optica spectrum disorders.

Neuromyelitis optica spectrum disorder (NMOSD) often presents in the elderly with an insidious onset of symptoms and aggressive progression. There have been anecdotal cases of very late onset (VLO)-NMOSD, but case series reports are rare. The aim of this retrospective study was to clarify the clinical features of VLO-NMOSD. According to the age at onset, we classified patients with NMOSD into three subgroups: ≤49years, early onset NMOSD (EO-NMOSD); 50-69years, late onset NMOSD (LO-NMOSD); and ≥70years, VLO-NMOSD. We evaluated the clinical characteristics, magnetic resonance imaging (MRI) findings, laboratory data, and immunotherapies of the groups. Overall, 12 men and 64 women with a median (interquartile range) age at onset and duration of disease of 42.0 (29.0-55.8) years and 70.0 (16.3-143.0)months, respectively, were included. Eight (11%) patients had VLO-NMOSD, 22 (29%) had LO-NMOSD, and 46 (61%) had EO-NMOSD. Patients with EO-NMOSD had a significantly longer interval between episodes as well as time between the first symptom and diagnosis of NMOSD than did those with VLO-NMOSD and LO-NMOSD (p=0.046). Optic neuritis and nerve lesions on MRI were significantly less frequent in patients with VLO-NMOSD than in those with LO-NMOSD and EO-NMOSD (p=0.002 and p=0.028, respectively). In contrast, patients with VLO-NMOSD had higher nadir Expanded Disability Status Scale and Nurick scale scores and a significantly longer spinal lesion length than did those with LO-NMOSD and EO-NMOSD (p=0.029, p=0.049, and p=0.032, respectively). Patients with VLO-NMOSD tend to develop severe myelitis with long cord lesions but not optic neuritis.

Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder primarily associated with optic neuritis, myelitis or area postrema syndrome. Several lines of evidence suggest that NMOSD is a humoral immune disease mainly caused by aquaporin-4 antibody and related complement-dependent cytotoxicity against astrocytes. Therefore, NMOSD is distinct from multiple sclerosis (MS). In the diagnosis of NMOSD, it is recommended to examine by high-sensitive cell-based assay targeting against M23-AQP4 but we always have to be careful for the possibility of false negative or false positive result due to each assay. To prevent relapse of the disease, it is best to avoid disease-modifying drugs used for treatment of MS because of possible acute exacerbation of the disease activity. Usually, it is recommended to start treatment with administration of oral steroids and then gradually move to immunosuppressants. However, side effects of such treatments need to be evaluated. Currently, there are additional options for therapy with biopharmaceutical agents such as eculizumab, satralizumab, rituximab, or inebilizumab to prevent relapse of the disease. These new options can clearly exceed or surpass the usual treatments and should be considered positively in aggressive cases of NMOSD.

Neuromyelitis Optica Spectrum Disorders (NMOSD) and Connective Tissue Disease (CTD): an Update for the Rheumatologist.

To review the pathophysiology, presentation, and treatment of neuromyelitis optica spectrum disorder (NMOSD) and its association with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS). NMOSD is an autoimmune disorder of the central nervous system that primarily targets astrocytes. Although the prevalence is unknown, the coexistence of NMOSD and SLE/SS is well-recognized. Patients with both NMOSD and SLE or SS require may require unique approaches to diagnosis and management. Coexistence of NMOSD and SLE/SS is important for the rheumatologist and neurologist to be able to recognize. For the rheumatologist, NMOSD and its neurologic symptoms represent a distinct disease process from neurologic complications of the patient's underlying connective tissue disease, and it requires distinct acute and chronic management. For the neurologist, the coexistence of SLE and SS can help to establish a diagnosis of NMOSD, or in some situations, the development of neurologic symptoms secondary to NMOSD can lead to the diagnosis of connective tissue disease.

Perception of Stigma in Patients with Neuromyelitis Optica Spectrum Disorder.

BackgroundPerception of stigma was associated with low self-esteem, psychological problems, and decreased health-seeking behavior among patients with different neurological disorders. The purpose of this study was to assess stigmatization and its impact in patients with neuromyelitis optica spectrum disorder (NMOSD).MethodsA non-interventional study was conducted at thirteen neuroimmunology clinics in Spain. Patients with a diagnosis of NMOSD (2015 Wingerchuk criteria) were included. The 8-item Stigma Scale for Chronic Illness (SSCI-8), the Expanded Disability Status Scale (EDSS), the 29-item Multiple Sclerosis Impact Scale (MSIS-29), the Beck Depression Inventory-Fast Screen (BDI-FS), the MOS Pain Effects Scale (MOS-PES) and the Fatigue Impact Scale for Daily Use (D-FIS) were used to assess the perception of stigma, disability, quality of life, mood, pain, and fatigue, respectively. Associations between outcome measures were analyzed using Spearman’s rank correlation.ResultsSeventy-one patients were studied (mean age: 47.4 years ± 14.9, 81.7% female, mean time since disease onset: 9.9 years ± 8.1). The median EDSS score was 3.0 (interquartile range 1.5, 4.5). Stigma prevalence was 61.4% (n=43). Thirty-one patients (43.6%) had depression. The SSCI-8 score showed a significant correlation with both physical (rho=0.576, p<0.0001) and psychological (rho=0.608, p<0.0001) MSIS-29 scales scores, EDSS score (rho=0.349, p=0.0033), BDI-FS score (rho= 0.613, p<0.0001), MOS-PES score (rho= 0.457, p<0.0001), and D-FIS score (rho=0.556, p<0.0001).ConclusionStigma is a common phenomenon affecting over 6 out of 10 patients with NMOSD. Understanding stigma may be useful to develop educational strategies improving NMOSD knowledge.

Sleep disorders as a possible predisposing attack factor in neuromyelitis optica spectrum disorder (NMOSD): A case-control study

BackgroundSleep disturbances are common in neuromyelitis optica spectrum disorder (NMOSD) and have a great impact on patients’ quality of life. According to a report, there is a 64% prevalence of poor sleep quality in NMOSD patients. Therefore, this study was done to evaluate the effect of sleep disturbances on NMOSD acute exacerbations. Materials and MethodsThis case-control study was conducted at Sina Hospital in 2019. A total of 60 patients with NMOSD diagnosis were enrolled in the study (30 patients were in the remission phase while 30 patients were hospitalized due to acute attacks). Sleep disorders were evaluated in both groups. Sleep quality was assessed during the last month using the Pittsburgh Sleep Quality Index (PSQI) questionnaire. Data were analyzed by SPSS software version 21. ResultsAmong 60 patients who were evaluated in both the control and attack groups, 86.7% were female. The duration of the disease was 68.23 ± 42.89 months in the control group and 69.83 ± 6.90 in the attack group. The mean age of patients was 34.15 years old. Sleep quality was unfavorable in 30% and 56% of patients in control and attack groups, respectively. There were significant differences between the two groups in sleep latency, habitual sleep efficiency, sleep duration, and sleep disturbance. ConclusionThe present study revealed that there was a significant difference in sleep quality between controls and attack patients and could show a direct relationship between sleep disorders and NMOSD attacks.

Longitudinally Extensive Transverse Myelitis: A Retrospective Study Differentiating Neuromyelitis Optica Spectrum Disorder From Other Etiologies.

BackgroundLongitudinally extensive transverse myelitis (LETM) is characterized by contiguous immune-mediated inflammatory lesion of the spinal cord extending more than three vertebral segments. Neuromyelitis optica spectrum disorder (NMOSD) is the most common and important cause of LETM. This study aims to evaluate the demographic profile, clinical presentations, neuroimaging features, laboratory parameters, and etiologies of LETM and differentiates NMOSD from other etiologies of LETM.MethodologyThis retrospective cross-sectional study was conducted at the Department of Neurology, Tribhuvan University Teaching Hospital, Kathmandu, Nepal. After receiving clearance from the ethical committee, a retrospective chart review was conducted and records of all the inpatient LETM cases admitted from March 2018 to June 2020 were obtained. From the patient records, the following information was extracted: the demographic profile, clinical presentations, neuroimaging features, cerebrospinal fluid analysis, serum anti-aquaporin-4 (AQP4) immunoglobulin G status, hemogram, infectious disease profile, inflammatory markers, and auto-immune panels. Descriptive analysis of data was performed with SPSS statistics 23.0 version (IBM Corp, Armonk, NY, USA).ResultsIn our study, the mean age of LETM patients was 36.58 years, and 12 out of 19 (63.15%) patients were young, with an age less than 40 years. A total of 13 (68.40%) patients were male, with a male-to-female ratio of 2.16. Seven (36.80%) patients had a clinical diagnosis of NMOSD with anti-AQP4 antibody-positive status, four (21.10%) had unknown etiology, three (15.8%) had post-infectious transverse myelitis, and three (15.80%) had a diagnosis of idiopathic transverse myelitis. There was a single case (5.30%) of cervical spondylotic myelopathy and leukemic transverse myelitis each. The common presenting symptoms of LETM were bladder dysfunction, paraparesis, quadriparesis, and visual impairment. Visual involvement, either unilateral or bilateral, was common in NMOSD and LETM of unknown etiology category. Similarly, brain lesion was common in patients with NMOSD and LETM of unknown etiology category.Conclusion LETM is a heterogeneous disorder with diverse etiologies and clinical presentations. NMOSD is an important cause of LETM that predominantly affects females. Optic neuritis can be seen in LETM of various etiologies, but it is more common in anti-AQP4-positive NMOSD patients. Brain lesions in LETM are common in anti-AQP4-positive NMOSD.

Effectiveness of treatments in Neuromyelitis optica to modify the course of disease in adult patients. Systematic review of literature.

Neuromyelitis Optica spectrum disorder (NMOSD) is an inflammatory disease, which manifests mostly as recurrent episodes of optic neuritis or myelitis that cause important disability. Early diagnosis and prompt initiation of immunosuppressive therapy are crucial in reducing relapses, disability, and mortality. Even though, there are few prospective randomized controlled trials, several drugs have proved to be both effective and safe. Azathioprine and Rituximab represent the standard of care and are used as first-line treatment agents worldwide. However, recent studies have unveiled new therapies, such as monoclonal antibodies. To make treatment recommendations and management guidelines, it is imperative to define an appropriate standard of care. A systematic literature review was performed in MEDLINE, EMBASE, and LILACS databases using the following terms: "(NMO OR Devic OR Neuromyelitis Optica) AND (Azathioprine OR Prednisone OR Rituximab OR Tocilizumab OR Bortezomib OR Inebilizumab OR Eculizumab OR Satralizumab)" including both, randomized clinical trials and observational studies published between January 2006 and January 2021. The inclusion criteria comprised patients aged 18 or older, NMOSD diagnosis following the Wingerchuck criteria, two or more therapies been compared, and the evaluation of both efficacy and safety outcomes. All studies comparing treatment only with placebo were excluded. Quality was assessed according with the design of the study, and results were synthesized through comparative tables for each outcome evaluated, differentiating the results of randomized and non-randomized studies. Thirteen studies with 1447 patients were included. Twelve studies evaluated the expanded disability status scale (EDSS) before and after treatment; in five of seven evaluating rituximab, it outperformed its comparators in improving the disability degree. Eleven studies assessed the annual relapse rate (ARR). Again, in six of seven evaluating rituximab, it was superior to other therapies. Time to relapse (TTR) was reported in five studies. The three studies that included Rituximab revealed a longer time to relapse in this arm of treatment. Finding were consistent in randomized and non-randomized studies. The new molecules Satralizumab, Eculizumab and Tocilizumab were evaluated in one study each, proving to be highly effective and safe. The safety profile analysis showed a higher number of adverse events for Azathioprine. This systematic review demonstrates a superiority tendency of Rituximab upon the other treatments strengthening the available evidence about NMOSD management. Superiority in EDSS outcomes, annual relapse rate, time to first relapse and relapses during treatment time was evidenced in the Rituximab group compared to other medications, with lower rates of adverse events. New molecules Tocilizumab, Eculizumab and Satralizumab also showed superiority in the evaluated results, especially in the relapses during treatment time outcome, although with subtle differences in EDSS and ARR outcomes. Our results suggest that monoclonal antibodies are highly effective and safe for the treatment of NMOSD; Rituximab showed better performance on multiple outcomes and has more evidence available. New molecules: Eculizumab, Tocilizumab, Satralizumab are good options for treatment. Drugs like Azathioprine and Mycophenolate are effective, but with a worse risk-benefit ratio, therefore, they are useful alternatives in places that do not have access to monoclonal antibodies.

Paraneoplastic neuromyelitis optica spectrum disorders: a case series.

Aquaporin-4 antibody (AQP4-IgG) neuromyelitis optica spectrum disorders (NMOSD) are rare idiopathic autoimmune diseases, presenting with optic neuritis (ON), longitudinally extensive transverse myelitis (LETM), and brainstem syndromes and a prevalence range between 0.5 and 4/100,000. Only 3% to 25% of NMOSD have been described as a paraneoplastic (PN) syndrome (PNNMOSD). Both idiopathic NMOSD (INMOSD) and PNNMOSD cases mostly affect females, but PNNMOSD usually presents with a spinal cord or brainstem involvement in elderly patients. Few cases of both malignancies (for the majority breast or lung cancer) and benign tumors (monoclonal gammopathy) were previously reported. Currently, there is no consensus on treatment approach for PNNMOSD (only surgical removal or surgery combined with chronic immunosuppression). Here, we present a series of three newly diagnosed PNNMOSD cases, who differ from each other for demographic and clinical features, tumor association, long-term treatment, and outcome. We propose that a PN etiology should be considered always whenever a new diagnosis of NMOSD is made, not only in patients over 50years old or in spinal cord/brainstem lesions presentations. Our findings add to existing evidence and raise awareness on PNNMOSD. We enhance the importance for the clinicians of recognizing tumor symptoms and signs whenever a NMOSD is newly diagnosed.

Characteristics and Predictors of Disease Course in Children Initially Presenting with Acute Disseminated Encephalomyelitis (ADEM)

Background: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system (CNS), characterized by new onset polyfocal neurologic symptoms with encephalopathy and multifocal demyelination, typically occurring in early childhood. The initial diagnosis of ADEM can be challenging, as up to 20% of children with multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD) are initially diagnosed with ADEM. Objective:To describe characteristics of patients with ADEM vs. recurrent demyelinating syndromes (MS, NMOSD, other) at the time of initial presentation and identify features at disease onset of presentations consistent with ADEM associated with monophasic demyelinating disease. Methods: This is a multicenter observational cohort study of children with a demyelinating disease diagnosis of ADEM, multiphasic ADEM, MS, and NMOSD who were followed at 12 regional pediatric MS referral centers in the US Network of Pediatric MS Centers. Descriptive statistics were used to report patient characteristics, clinical/imaging presenting features and subsequent outcomes. Logistic regression was used to predict features associated with a monophasic course. Results: As of July 2019, 872 pediatric patients with a final diagnosis of ADEM (n=89), MS (n= 664) or NMOSD (n=119) were identified. The mean +/- standard deviation follow-up for all patients was 5·7 +/- 3·1 years. ADEM patients were the youngest with mean age at first event 5·4 +/- 3·7 years (p < 0.001) and male predominance (62%) (p < 0·001). Severe clinical symptoms at onset were more frequent in ADEM (55% vs. 35% NMOSD and 15% MS, p < 0·001). After 2 years of follow-up, 78% (93/119) of patients initially diagnosed with ADEM retained this diagnosis (ADEM to ADEM), while 9·2% were later reclassified as MS, 3·3% as NMOSD and 7·5% as demyelinating disease not otherwise specified (DDNOS). In univariable logistic regression, younger age at first event was associated with retaining ADEM diagnosis, while presentation with optic neuritis and gadolinium enhancement on brain MRI were associated with ADEM reclassification to MS, NMOSD, or DDNOS at 2 years of follow-up. In multivariable analysis, older age at first event (OR 1·16 [95% CI 1·00 – 1·33] for 1-year increase, p = 0·04), presenting with optic neuritis (OR 15·31 [95% CI 3·27 – 71·66], p <0·001) and presence of gadolinium enhancement on brain MRI at onset (OR 6·80 [95% CI 2·03 – 22·78], p = 0·0019) were associated with reclassification of ADEM to MS, NMOSD or DDNOS within 2 years. Conclusion: Children who remain classified as ADEM vs. those who are reclassified as other demyelinating disorders are younger at onset, and less likely to have optic neuritis or gadolinium-enhancing lesions at disease onset. Funding: None to declare. Declaration of Interest: None to declare. Ethical Approval: The ethics committees of participating institutions approved this study. Parents and participants signed consent forms and assent forms when required by each center’s institutional review board prior to enrollment.

A window into the future? MRI for evaluation of neuromyelitis optica spectrum disorder throughout the disease course.

Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing, inflammatory disease of the central nervous system marked by relapses often associated with poor recovery and long-term disability. Magnetic resonance imaging (MRI) is recognized as an important tool for timely diagnosis of NMOSD as, in combination with serologic testing, it aids in distinguishing NMOSD from possible mimics. Although the role of MRI for disease monitoring after diagnosis is not as well established, MRI may provide important prognostic information and help differentiate between relapses and pseudorelapses. Increasing evidence of subclinical disease activity and the emergence of newly approved, highly effective immunotherapies for NMOSD adjure us to re-evaluate MRI as a tool to guide optimal treatment selection and escalation throughout the disease course. In this article we review the role of MRI in NMOSD diagnosis, prognostication, disease monitoring, and treatment selection.

Identification and temporal trends of patients with neuromyelitis optica spectrum disorder in a US insurance claims database

Background This US claims-based study aimed to identify and characterize temporal trends in diagnostic pathways for patients likely to have neuromyelitis optica spectrum disorder (NMOSD). Methods Patients were identified from IBM MarketScan Commercial Databases if, within 1 year, they had two NMOSD claims separated by ≥ 60 days; two transverse myelitis (TM) or optic neuritis (ON) claims separated by ≥ 60 days, and one additional symptom (TM, ON, or area postrema syndrome); or one NMOSD claim and one additional symptom. The first NMOSD or TM/ON claim was the index date, and the second claim was the diagnosis date. Similar methodology was used in temporal trend and incidence and prevalence analyses. Results Among 1,901 patients with NMOSD, 34.2% were identified by two NMO claims, 53.2% by ON or TM +1 symptom, and 12.6% by one NMOSD claim +1 symptom. Anti–aquaporin-4 immunoglobin G (AQP4-IgG) autoantibody tests and magnetic resonance imaging was used for 23.0% and 71.9% of cases, respectively. Across cohorts, 21.4–49.1% had multiple sclerosis (MS) diagnosis claims prior to index date, and 37.3–60.6% had an MS diagnosis, 14.9–31.0% had MS disease-modifying therapy (DMT) claims and 6.3–44.8% had immunosuppressive therapy (IST) claims <1 year after diagnosis. Over time, there were slight changes in MS diagnosis claims, AQP4-IgG autoantibody testing, and DMT and IST use before and after NMOSD diagnosis. Limitations This study is limited by the information available in US claims databases, which included the potential for misclassification of NMOSD based solely on claims codes and lack of reimbursement for AQP4-IgG testing by insurance companies. Conclusions Among patients likely to have NMOSD, low AQP4-IgG testing rates, IST use, frequent MS diagnosis claims, and DMT use highlight the need for a diagnostic algorithm and timely treatment of NMOSD.

The impact of the COVID-19 pandemic on hospitalizations and plasmapheresis therapy in multiple sclerosis and neuromyelitis optica spectrum disorder: a nationwide analysis from Germany.

Background:Many countries worldwide reported side effects of the coronavirus disease 2019 (COVID-19) pandemic that have influenced the care of patients with other diseases in both acute and elective settings. Patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) represent the major patient population suffering from an autoimmune inflammatory demyelinating disease of the central nervous system. We aimed to analyze MS and NMOSD hospitalizations, the application of plasmapheresis therapy, and the dynamic during different periods of the COVID-19 pandemic in Germany.Methods:We conducted a nationwide retrospective cross-sectional study using the administrative database of all hospitalized patients with the main diagnosis of MS and NMOSD, including the information on the application of plasmapheresis therapy. We included full-year data from 1463 hospitals of all MS and NMOSD patients hospitalized in 2019 and 2020 in Germany (n = 87,453). We compared case numbers and plasmapheresis therapy rates of the different pandemic periods in 2020 with the corresponding periods in 2019.Results:We observed a substantial decline of MS and NMOSD patients’ hospitalizations during the different pandemic periods, with the most remarkable decline during the first wave of the pandemic (First diagnosis of MS: −16.8%; relapsing-remitting MS: −34.0%; secondary progressive MS: −48.9%; primary progressive MS: −43.8%; NMOSD: −19.2%). Treatment rates with plasmapheresis increased for MS and NMOSD patients in 2020 compared to 2019 (1.8% versus 1.6%, p = 0.003; 14.0% versus 9.3%, p < 0.001), with a substantial increase during the first wave of the pandemic, especially in NMOSD patients (19.7% versus 8.4%, p < 0.001).Conclusion:There was a marked decline of MS and NMOSD patients’ hospitalizations during the different pandemic periods in 2020, with the most substantial reduction during the pandemic’s first wave and in progressive MS patients. MS and NMOSD patients who needed rescue relapse treatment continued to receive plasmapheresis therapy in Germany.

Recurrent bilateral optic neuritis associated with neuromyelitis optica spectrum disorder in a child

Reports of bilateral optic neuritis in children associated with viral infection or postimmunization are relatively common. Neuromyelitis optica spectrum disorders (NMOSD) is a rare syndrome of severe inflammatory immune-mediated demyelination of central nervous system, with median age of presentation being 37.5 years reported in south Indian population. NMOSD associated with optic neuritis in children is very rare. We present a case of 10-year-old girl with bilateral recurrent optic neuritis, seropositive for NMO-IgG (anti-AQP4) supporting the diagnosis of NMOSD. With prompt treatment, patient made a remarkable visual recovery without neurologic deficit. This report highlights prompt diagnosis, leading to better visual outcomes in NMOSD.

High serum neurofilament levels among Chinese patients with aquaporin-4-IgG-seropositive neuromyelitis optica spectrum disorders

BackgroundSerum neurofilament light chain (sNfL) is a promising biomarker for neuromyelitis optica spectrum disorders (NMOSD) and multiple sclerosis (MS), but there is limited validation data in specific ethnic and disease groups. ObjectiveTo investigate the levels of sNfL in a cohort of Chinese patients with NMOSD and compare sNfL levels in patients with different disease courses and treatments. MethodsWe analysed sNfL levels in 153 Chinese patients with NMOSD (n = 51) and MS (n = 102) using single-molecule array (Simoa) technology. The sNfL levels were compared with those of 71 healthy controls from two centres in southern China. For each disease, we assessed correlations between sNfL and disease phases and treatments. ResultsHigher levels of sNfL were found in the patients with NMOSD [17.97 (10.55–27.94) pg/mL] and MS [15.83 (8.92–25.67) pg/mL] compared to healthy controls [10.09 (7.19–13.29) pg/mL, p < 0.001]. No significant differences were found between the AQP4-IgG-positive NMOSD group and OCB-positive MS group. ConclusionssNfL measured by Simoa technology is a potential candidate blood biomarker for the diagnosis and disease monitoring of NMOSD in Chinese patients, warranting further prospective and multicentre studies.

Paroxysmal symptoms in neuromyelitis optica spectrum disorder: Results from an online patient survey

BackgroundParoxysmal symptoms (PS), defined as short-lasting, recurrent, and stereotyped neurological symptoms, are frequently reported by patients with Neuromyelitis Optica Spectrum Disorder (NMOSD). Their prevalence and spectrum of presentations in NMOSD have not been fully characterized. MethodsPatients with NMOSD, who were members of a closed international Facebook Group, were recruited to complete an anonymous survey on REDCap. Participants were queried regarding demographic and NMOSD-related characteristics and PS history. ResultsThe sample consisted of 219 responders with self-reported NMOSD, of whom 134 (63.8%) reported testing positive for AQP4 Antibody. 156 responders (71.9%) reported ≥1 type of PS during the disease course. The most common PS were intermittent tingling/numbness sensation (N=106, 67.9%), followed by involuntary muscle contractions/abnormal posture (N=95, 60.9%), hot/cold/burning sensations (N=87, 55.8%), and shock-like sensations along the spine or limbs (N=77, 49.4%). 150 responders (96% of those with PS) reported that PS were painful; in 82 responders (54.6%), the pain intensity reached ≥ 8/10 and in 40 responders (26.0%) - 10/10 level. PS were most commonly aggravated by fatigue (105 responders, 70.0%), physical activity (N=86, 57.3%), and neck flexion (N=39 responders, 26.0%). 82 patients (52.5% of those with PS) reported having been prescribed one or more medications for PS. Less than 50% reported them to be 'very helpful.' ConclusionsThis survey highlights that PS occurs commonly in NMOSD patients. The symptomatology of PS is diverse. PS are often painful and not adequately treated. Our study represents a novel method to learn more about a rare disease from the patient's perspective. Given the fact that the study was conducted using an anonymous questionnaire and the diagnosis of NMOSD was self-reported by the survey participants, its’ results should be regarded as a first step towards the understanding of PS in NMOSD, which should be further validated in a larger, controlled study.