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Background: Between 29% and 67% of neuromyelitis optica spectrum disorder patients have cognitive alterations. Objective: To assess the frequency of cognitive impairment in patients with neuromyelitis optica spectrum disorder in Mexico using the Brief International Cognitive Assessment for Multiple Sclerosis. Methods: We evaluated 40 neuromyelitis optica spectrum disorder patients and 40 healthy controls from Mexico. Results: 28 (70.0%) patients with neuromyelitis optica spectrum disorder had cognitive impairment in two or more cognitive domains. Student´s T test showed statistically poor performance by neuromyelitis optica spectrum disorder patients compared to healthy controls on all three neuropsychological test scores. This significant difference was observed on the Symbols Digit Modalities Test (t = 8.875; p ≤ 0.001); California Verbal Learning Test-II memory (t = 10.418; p ≤ 0.001); and Brief Visuospatial Memory Test Revised (t = 6.123; p ≤ 0.001). Conclusions: This study showed that 70% of neuromyelitis optica spectrum disorder patients exhibited cognitive impairment in two or more cognitive domains. Determining the frequency of cognitive impairment will guide the decision of Neuropsychologists in planning cognitive rehabilitation across various domains.

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BACKGROUND: Multiple sclerosis misdiagnosis remains a problem despite the well-validated McDonald 2017. For proper evaluation of errors in the diagnostic process that lead to misdiagnosis, it is adequate to incorporate patients who are already under regular follow-up at reference centers of demyelinating diseases. OBJECTIVES: To evaluate multiple sclerosis misdiagnosis in patients who are on follow-up at a reference center of demyelinating diseases in Brazil. METHODS: We designed an observational study including patients in regular follow-up, who were diagnosed with multiple sclerosis at our specialized outpatient clinic in the Hospital of Clinics in the University of Sao Paulo, from 1996 to 2021, and were reassessed for misdiagnosis in 2022. We evaluated demographic information, clinical profile, and complementary exams and classified participants as "established multiple sclerosis," "non-multiple sclerosis, diagnosed," and "non-multiple sclerosis, undiagnosed." Failures in the diagnostic process were assessed by the modified Diagnostic Error Evaluation and Research tool. RESULTS: A total of 201 patients were included. After analysis, 191/201 (95.02%) participants were confirmed as "established multiple sclerosis," 5/201 (2.49%) were defined as "non-multiple sclerosis, diagnosed," and 5/201 (2.49%) were defined as "non-multiple sclerosis, undiagnosed." CONCLUSIONS: Multiple sclerosis misdiagnosis persists in reference centers, emphasizing the need for careful interpretation of clinical findings to prevent errors.

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Demyelinating diseases alter myelin or the coating surrounding most nerve fibers in the central and peripheral nervous systems. The grouping of human central nervous system demyelinating disorders today includes multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) as distinct disease categories. Each disease is caused by a complex combination of genetic and environmental variables, many involving an autoimmune response. Even though these conditions are fundamentally similar, research into genetic factors, their unique clinical manifestations, and lesion pathology has helped with differential diagnosis and disease pathogenesis knowledge. This review aims to synthesize the genetic approaches that explain the differential susceptibility between these diseases, explore the overlapping clinical features, and pathological findings, discuss existing and emerging hypotheses on the etiology of demyelination, and assess recent pathogenicity studies and their implications for human demyelination. This review presents critical information from previous studies on the disease, which asks several questions to understand the gaps in research in this field.

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BACKGROUND: Neuromyelitis Optica Spectrum Disorders (NMOSD) is an antibody-mediated disorder of the Central Nervous System where a leading role of the complement system has been demonstrated. OBJECTIVE: To measure the levels of complement factors C3, C4 and C5a in serum and plasma of clinical remission patients with AQP4-IgG + NMOSD. METHODS: Twelve patients with NMOSD AQP4 + according to 2015 criteria from a General Hospital in Buenos Aires, Argentina, were included in the study, and 19 age- and sex-matched healthy volunteers as a control group (HC). AQP4 antibodies were measured in serum by CBA analysis. Fresh blood samples were centrifuged to obtain serum and plasma. C3, C4, and AQP4 antibodies were measured in the serum, whereas C5a was measured in the plasma, which was obtained using Futhan (BD FUT-175®, BD Biosciences, San Jose, CA, USA). RESULTS: The complement factors, C3, C4, and C5a were measured in all samples. The mean concentration of C3 was 130.7 mg/dl (SD 16.1 mg/dl), and the mean concentration of C4 was 21.6 mg/dl (SD 4.8 mg/dl); both values were within the normal reference range (C3: 84-193 mg/dl; C4: 20-40 mg/dl) and were not significantly different (p > 0.05) from the mean levels in healthy controls (C3: 116.9 mg/dl; C4: 21.9 mg/dl). When analyzing the mean plasma level of C5a, we found a statistically significant difference (p = 0.0444) between the mean concentration of C5a in NMOSD patients (43.1 ng/ml; SD 48.7 ng/ml) and the HC group (17.7 ng/ml; SD 16.7 ng/ ml). CONCLUSIONS: In conclusion, the present study demonstrates that plasma C5a may be interesting to investigate as a potential biomarker of disease activity in NMOSD, in a larger and prospective cohort.

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BACKGROUND: Optic neuritis (ON) causes several sequela. Aggressive treatment with plasma exchange (TPE) is an option. This study describes improvement and safety outcomes with TPE. METHODS: We recruited adults with ON in neuromyelitis optica spectrum disorders (NMOSD) patients treated with TPE. The primary outcome was an improvement in the visual acuity scale (VOS). We described the data and used multivariate logistic regression to identify factors associated with response. RESULTS: Eighty-three patients received 558 TPE sessions. Mean age was 40.9 years (±13.7 years); 73.5% were women, 50.1% were first attack, and 10.7% were bilateral. Median VOS: 5 (range [R], 2-7). Median time between onset and TPE was 8 days (R, 1-32). By Keegan's criteria, 82.4% experience improvement and 78.3% improve in at least 1 point in VOS. Age and pre-TPE VOS were related to improvement. Low fibrinogen occurs in 26% sessions. CONCLUSION: TPE is effective and safety for ON in NMOSD patients. There is a need for a clinical trial using a therapeutic equivalent.

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INTRODUCTION: Neuromyelitis optica spectrum disorders (NMOSD) is a rare inflammatory and demyelinating disease of the central nervous system (CNS) more frequent in women and Afro-descendants. No previous epidemiological or prognostic study has been conducted in the region of the state of Bahia, Brazilian Northeast. OBJECTIVE: To evaluate clinical and prognostic aspects in patients with NMOSD from a cohort in northeastern Brazil. MATERIAL AND METHODS: A single-center retrospective study was conducted with consecutive patients diagnosed with NMOSD. Clinical and epidemiological characteristics were described. The degree of disability was expressed by the Expanded Disability Status Scale (EDSS). Worsening disability were analyzed through negative binomial regression adjusted for disease duration. RESULTS: Ninety-one patients were included, 72 (79.1%) female and 67 (73.6%) afro descendants. Mean age at onset was 36 (± 14) years and 73.3% were anti-aquaporin-4 antibody positive. Isolated transverse myelitis (32.9%) and isolated optic neuritis (22.4%) were the most frequent initial clinical syndromes. After multivariate analysis, optic neuritis (RR = 0.45; 95% CI = 0.23 - 0.88; p = 0.020) and dyslipidemia (RR = 0.40; 95% CI = 0.20 - 0.83; p = 0.014) were associated with slower disease progression. Area postrema involvement (RR = 6.70; 95% CI = 3.31 - 13.54; p < 0.001) and age at onset (RR = 1.03; 95% CI = 1.01 - 1.05; p = 0.003) were associated with faster disease progression. CONCLUSIONS: In the first clinical and prognostic study in northeastern Brazil, we identified area postrema involvement, age at onset, optic neuritis at fist syndrome and dyslipidemia as the main prognostic factors associated with disease progression.

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INTRODUCTION: Neuromyelitis optica spectrum disorders (NMOSD) are rare but often devastating neuroinflammatory autoimmune diseases of the central nervous system. Acute treatment is critically important and it should be initiated early and aggressively, as relapses result in severe residual disability. Acute treatments are still based on clinical experience and observational studies. The most commonly used treatments are steroids and plasmapheresis. Several new treatments to improve management and recovery after relapses in NMOSD are currently under investigation. AREAS COVERED: This review discusses current and the most recent advances in active development of phase II/III clinical trials for acute treatment options and therapeutic strategies that can help management improvement of NMOSD during a relapse. These treatments include bevacizumab, ublituximab and HBM9161. EXPERT OPINION: NMOSD relapses require prompt evaluation and timely treatment to restore function and mitigate disability. Timing is critical. Plasmapheresis showed better outcomes in terms of recovery when compared to high-dose intravenous methylprednisolone alone. Some groups suggest that plasmapheresis could be considered as an initial treatment approach in different clinical scenarios due to its higher effectiveness. Future research and/or real-world data will establish the advantages and disadvantages of these new treatments and define the appropriate patient profile.

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BACKGROUND: Neuromyelitis Optica Spectrum Disorders (NMOSD) are a group of inflammatory diseases of the Central Nervous System (CNS) that primarily affect the optic nerve and spinal cord, usually with a severe and relapsing course. Due to the scarce information in non-Caucasian populations, we aimed to describe incidence, prevalence, and main clinical characteristics of NMOSD in a defined region in Mexico. MATERIALS AND METHODS: Descriptive, retrospective analysis of all reported cases of NMOSD attended in the neurology department of the UMAE-HE, CMNO, IMSS, the biggest third level hospital in Western Mexico. We searched the electronic medical records of the hospital for patients with a diagnosis of NMO, and reviewed all cases to confirm if they fulfilled NMOSD 2015 diagnostic criteria. Data were collected through a structured form. We described adjusted incidence and prevalence according to the WHO method, for the IMSS affiliated total population in Jalisco state in 2019. RESULTS: 67 NMOSD patients were included in the analysis of clinical data, with a mean age at onset of symptoms of 36 years ((Rivera et al., 2008-65). Most patients were female (74.6%). 53 patients living in Jalisco by the end of 2019 were included in the analysis of prevalence and incidence. Adjusted prevalence was 0.71/100,000 (95% CI 0.55-0.92), while adjusted incidence was 1.87/1,000,000 person-years (95% CI 1.11-3.16). In the full cohort, the first symptom of NMOSD was optic neuritis in 49.3% of the patients, followed by transverse myelitis (23.9%) and area postrema syndrome (10.4%). 62 patients relapsed in a mean follow-up of 2 years (0-7). 5 patients with less than 6 months of follow up had not relapsed. 55.2% of the patients were AQP4-IgG +, 14.9% AQP4-IgG -, and 29.9% unknown status. CONCLUSIONS: Although NMOSD prevalence is similar to other reports around the world, incidence is higher than in Caucasian populations. We believe that this high incidence is related to an increased awareness of the disease in the era of new NMOSD treatments. Recurrent disease is very frequent in our cohort.

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Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory and autoimmune disease whose biomarker is the anti-AQP4-IgG autoantibody that binds to aquaporin-4 (AQP4) protein. We introduced a nanosensor with a sensitivity of 84.6%, higher than the CBA's 76.5%. Using silver nanoparticles (AgNPs), we detected not only seropositive but also some false-negative patients previously classified with CBA. It consisted of AgNPs coated with one of a panel of 5 AQP4 epitopes. The ability in detecting the anti-AQP4-IgG in NMOSD patients depended on the epitope and synergy could be obtained by combining different epitopes. We demonstrated that NMOSD patients could easily be distinguished from healthy subjects and patients with multiple sclerosis. Using the most sensitive AQP461-70 peptide, we established a calibration curve to estimate the concentration of anti-AQP4-IgG in seropositive NMOSD patients. The ability to enhance the accuracy of the diagnosis may improve the prognosis of 10-27% of anti-AQP4-IgG seronegative patients worldwide.

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BACKGROUND: In children, an acute demyelinating disease may evolve as a multiphasic disease with multiple relapses, such as multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). The Brainstem Auditory Evoked Potentials (BAEP) and Long-Latency Auditory Evoked Potentials (LLAEP) contribute to the identification of either retrocochlear changes or other central auditory nervous system (CANS) changes. OBJECTIVES: To characterize BAEP and LLAEP in children and adolescents with MS and NMOSD and verify the diagnostic values of these potentials in each of the demyelinating diseases. METHODS: The 40 participants were divided into two study groups (SG1 - MS, SG2 - NMOSD) and two comparison groups (CG1 and CG2), matched for age (9 years-17 years and 11 months) and sex. Electrophysiological hearing assessment was performed with BAEP and LLAEP. RESULTS: When SG1 and SG2 were compared with CG1 and CG2 regarding BAEP and LLAEP, both SG1 and SG2 presented a higher occurrence of changes. Also, individuals with MS had higher occurrences of BAEP changes, whereas individuals with NMOSD had a higher occurrence of LLAEP changes. CONCLUSIONS: BAEP and LLAEP in children and adolescents with MS or NMOSD showed higher latencies and lower amplitudes of some components when these individuals were compared with their peers. These procedures were highly accurate to identify demyelinating diseases. BAEP results were more abnormal in individuals with MS, while LLAEP was so with NMOSD. These findings indicate that the auditory evoked potentials are important instruments for the differential diagnosis of MS and NMOSD, and valuable to monitor disease evolution.

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