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The term 'neuromyelitis optica spectrum disorders' (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and its formes frustes and to a number of closely related clinical syndromes without AQP4-IgG. NMOSD were originally considered subvariants of multiple sclerosis (MS) but are now widely recognized as disorders in their own right that are distinct from MS with regard to immunopathogenesis, clinical presentation, optimum treatment, and prognosis. In part 1 of this two-part article series, which ties in with our 2014 recommendations, the neuromyelitis optica study group (NEMOS) gives updated recommendations on the diagnosis and differential diagnosis of NMOSD. A key focus is on differentiating NMOSD from MS and from myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD), which shares significant similarity with NMOSD with regard to clinical and, partly, radiological presentation, but is a pathogenetically distinct disease. In part 2, we provide updated recommendations on the treatment of NMOSD, covering all newly approved drugs as well as established treatment options.

OBJECTIVE: To evaluate costs and health-related quality of life (HRQoL) of neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein-antibody associated disease (MOGAD).MethodsIn this multicenter cross-sectional study, data on consumption of medical and non-medical resources and work ability were assessed via patient questionnaires. Costs were analyzed in EUR for 2018 from the societal perspective. HRQoL was captured by the EuroQoL EQ-5D-5L questionnaire. Clinical data were retrieved from the Neuromyelitis Optica Study Group (NEMOS) database. RESULTS: Two hundred twelve patients (80% women; median age 50 [19-83] years; median disease duration 7 [0-43] years; median Expanded Disability Status Scale [EDSS] 3.5 [0-8.5]; 66% Aquaporin-4 IgG, 22% MOG IgG positive, 12% double seronegative) were analyzed. The mean total annual per capita cost of illness accounted for EUR (Euro) 59 574 (95% CI 51 225 to 68 293; USD [United States dollars] 70 297, 95% CI 60 445 to 80 586), and the mean index value of the EQ-5D-5L was 0.693 (95% CI 0.65 to 0.73). The most important cost drivers were informal care costs (28% of total costs), indirect costs (23%) and drugs (16%), especially immunotherapeutics. Costs showed a positive correlation with disease severity (ρ=0.56, 95% CI 0.45 to 0.65); in the EDSS 6.5-8.5 subgroup the mean annual costs were EUR 129 687 (95% CI 101 946 to 160 336; USD 153 031, 95% CI 120 296 to 189 196). The HRQoL revealed a negative correlation to disease severity (ρ=-0.69, 95% CI -0.76 to -0.61); in the EDSS 6.5-8.5 subgroup the EQ-5D-5L mean index value was 0.195 (95% CI 0.13 to 0.28). Neither antibody status nor disease duration influenced the total annual costs or HRQoL. CONCLUSION: These German data from the era without approved preventive immunotherapies show enormous effects of the diseases on costs and quality of life. An early and cost-effective therapy should be provided to prevent long-term disability and preserve quality of life.

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Objective: To analyze whether 1 of the 2 apheresis techniques, therapeutic plasma exchange (PE) or immunoadsorption (IA), is superior in treating neuromyelitis optica spectrum disorder (NMOSD) attacks and to identify predictive factors for complete remission (CR). Methods: This retrospective cohort study was based on the registry of the German Neuromyelitis Optica Study Group, a nationwide network established in 2008. It recruited patients with neuromyelitis optica diagnosed according to the 2006 Wingerchuk criteria or with aquaporin-4 (AQP4-ab)-antibody–seropositive NMOSD treated at 6 regional hospitals and 16 tertiary referral centers until March 2013. Besides descriptive data analysis of patient and attack characteristics, generalized estimation equation (GEE) analyses were applied to compare the effectiveness of the 2 apheresis techniques. A GEE model was generated to assess predictors of outcome. Results: Two hundred and seven attacks in 105 patients (87% AQP4-ab-antibody seropositive) were treated with at least 1 apheresis therapy. Neither PE nor IA was proven superior in the therapy of NMOSD attacks. CR was only achieved with early apheresis therapy. Strong predictors for CR were the use of apheresis therapy as first-line therapy (OR 12.27, 95% CI: 1.04–144.91, p = 0.047), time from onset of attack to start of therapy in days (OR 0.94, 95% CI: 0.89–0.99, p = 0.014), the presence of AQP4-ab-antibodies (OR 33.34, 95% CI: 1.76–631.17, p = 0.019), and monofocal attack manifestation (OR 4.71, 95% CI: 1.03–21.62, p = 0.046). Conclusions: Our findings suggest early use of an apheresis therapy in NMOSD attacks, particularly in AQP4-ab-seropositive patients. No superiority was shown for one of the 2 apheresis techniques. Classification of evidence: This study provides Class IV evidence that for patients with NMOSD, neither PE nor IA is superior in the treatment of attacks.

BACKGROUND: Antibodies to human full-length myelin oligodendrocyte glycoprotein (MOG-IgG) as detected by new-generation cell-based assays have recently been described in patients presenting with acute demyelinating disease of the central nervous system, including patients previously diagnosed with multiple sclerosis (MS). However, only limited data are available on the relevance of MOG-IgG testing in patients with chronic progressive demyelinating disease. It is unclear if patients with primary progressive MS (PPMS) or secondary progressive MS (SPMS) should routinely be tested for MOG-IgG. OBJECTIVE: To evaluate the frequency of MOG-IgG among patients classified as having PPMS or SPMS based on current diagnostic criteria. METHODS: For this purpose, we retrospectively tested serum samples of 200 patients with PPMS or SPMS for MOG-IgG using cell-based assays. In addition, we performed a review of the entire English language literature on MOG-IgG published between 2011 and 2017. RESULTS: None of 139 PPMS and 61 SPMS patients tested was positive for MOG-IgG. Based on a review of the literature, we identified 35 further MOG-IgG tests in patients with PPMS and 55 in patients with SPMS; the only reportedly positive sample was positive just at threshold level and was tested in a non-IgG-specific assay. In total, a single borderline positive result was observed among 290 tests. CONCLUSION: Our data suggest that MOG-IgG is absent or extremely rare among patients with PPMS or SPMS. Routine screening of patients with typical PPMS/SPMS for MOG-IgG seems not to be justified.

Background: Impaired low-contrast visual acuity (LCVA) is common in multiple sclerosis (MS) and other neurological diseases. Its assessment is often limited to selected contrasts, for example, 2.5% or 1.25%. Computerized adaptive testing with the quick contrast-sensitivity function (qCSF) method allows assessment across expanded contrast and spatial frequency ranges. Objective: The objective of this article is to compare qCSF with high- and low-contrast charts and patient-reported visual function. Methods: We enrolled 131 consecutive MS patients (mean age 39.6 years) to assess high-contrast visual acuity (HCVA) at 30 cm and 5 m, low-contrast vision with Sloan charts at 2.5% and 1.25%, qCSF and the National Eye Institute Visual Functioning Questionnaire (NEIVFQ). Associations between the different measures were estimated with linear regression models corrected for age, gender and multiple testing. Results: The association between qCSF and Sloan charts (R2¼0.68) was higher than with HCVA (5 m: R2¼0.5; 30 cm: R2¼0.41). The highest association with NEIVFQ subscales was observed for qCSF (R2 0.20 0.57), while Sloan charts were not associated with any NEIVFQ subscale after correction for multiple testing. Conclusion: The qCSF is a promising new outcome for low-contrast vision in MS and other neurological diseases. Here we show a closer link to patient-reported visual function than standard low- and high-contrast charts.