OBJECTIVE: Characterizing the neuropathological features of neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) is crucial for understanding its mechanisms. Given the important role of dynamic features in the brain\'s functional architecture, we aim to investigate the dynamic features of spontaneous brain activity and their concordance using resting-state functional magnetic resonance imaging (rs-fMRI) in NMOSD-ON.
METHODS: Fourteen NMOSD-ON patients and 21 healthy controls (HCs) underwent rs-fMRI and ophthalmological examinations. Five dynamic indices depicting different aspects of functional characteristics were calculated using a sliding window method based on rs-fMRI data. Kendall\'s coefficient was utilized to measure concordance among these indices at each time point. The differences of dynamic features between two groups were evaluated using two-sample t-tests, with correlations explored between altered dynamics and clinical parameters.
RESULTS: Compared to HCs, NMOSD-ON patients exhibited significant decreases in dynamic regional homogeneity (dReHo) and dynamic degree centrality (dDC) in visual regions, including bilateral cuneus, lingual gyrus, calcarine sulcus, and occipital gyrus. Conversely, increases were observed in left insula, left thalamus, and bilateral caudate. The concordance of NMOSD-ON patients was significantly lower than HCs. The dReHo of right cuneus negatively correlated with mean deviation of visual field (r = -0.591, p = 0.026) and the dReHo of left cuneus negatively correlated with disease duration (r = -0.588, p = 0.030).
CONCLUSIONS: The evidence suggests that regional dynamic functional alterations involving vision, emotional processing, and cognitive control may provide a new understanding of brain changes in the progression of NMOSD-ON.

Comorbidities in patients with multiple sclerosis (MS) and antibody-mediated diseases of the central nervous system (CNS) including neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disease (MOGAD) are common and may influence the course of their neurological disease. Comorbidity may contribute to neuronal injury and therefore limit recovery from attacks, accelerate disease progression, and increase disability. This study aims to explore the impact of comorbidity, particularly vascular comorbidity, and related risk factors on clinical and paraclinical parameters of MS, NMOSD and MOGAD. We propose COMMIT, a prospective multicenter study with longitudinal follow-up of patients with MS, NMOSD, and MOGAD, with or without comorbidities, as well as healthy subjects as controls. Subjects will be stratified by age, sex and ethnicity. In consecutive samples we will analyze levels of inflammation and neurodegeneration markers in both fluid and cellular compartments of the peripheral blood and cerebrospinal fluid (CSF) using multiple state-of-the-art technologies, including untargeted proteomics and targeted ultrasensitive ELISA assays and quantitative reverse transcription polymerase chain reaction (RT-qPCR) as well as high-dimensional single-cell technologies i.e., mass cytometry and single-cell RNA sequencing. Algorithm-based data analyses will be used to unravel the relationship between these markers, optical coherence tomography (OCT) and magnetic resonance imaging (MRI), and clinical outcomes including frequency and severity of relapses, long-term disability, and quality of life. The goal is to evaluate the impact of comorbidities on MS, NMOSD, and MOGAD which may lead to development of treatment approaches to improve outcomes of inflammatory demyelinating diseases of the CNS.

UNASSIGNED: Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory autoimmune disease affecting the central nervous system (CNS). NMOSD pathogenesis involves systemic inflammation. However, a causal relationship between circulating cytokine levels and NMOSD remains unclear.
UNASSIGNED: Mendelian randomization (MR) approaches were used to investigate the potential association between genetically determined circulating 19 inflammatory cytokines and 12 chemokines levels and the risk of developing NMOSD.
UNASSIGNED: After Bonferroni correction, the risk of aquaporin 4-antibody (AQP4-ab)-positive NMOSD was suggested to be causally associated with the circulating levels of three cytokines, including interleukin (IL)-4 [odds ratio (OR): 11.01, 95% confidence interval (CI): 1.16-104.56, P = 0.037], IL-24 (OR: 161.37; 95% CI: 2.46-10569.21, P = 0.017), and C-C motif chemokine 19 (CCL19) (OR: 6.87, 95% CI: 1.78-26.93, P = 0.006).
UNASSIGNED: These findings suggest that a genetic predisposition to higher levels of IL-4, IL-24, and CCL19 may exert a causal effect on the risk of AQP4-ab-positive NMOSD. Further studies are warranted to clarify how these cytokines affect the development of AQP4-ab-positive NMOSD.

OBJECTIVE: Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are two major demyelinating diseases affecting the central nervous system (CNS). The objective of this study is to evaluate the prevalence of pontine trigeminal nerve lesions in patients diagnosed with MS and NMOSD using MRI.
METHODS: This retrospective study included patients diagnosed with MS or NMOSD between July 2018 and July 2023. MS patients were clinically diagnosed using the 2017 McDonald criteria, while NMOSD patients were those who met the 2015 International Panel for NMO Diagnosis (IPND) criteria and were positive for Aquaporin-4 Antibody (AQP4-Ab).
RESULTS: The study included a total of 90 patients, with 45 diagnosed with MS and another 45 with NMOSD. Pontine trigeminal nerve lesions were observed in both MS and NMOSD, but were more prevalent in MS patients (20 % vs. 2 %, p = 0.008). Root entry zone (REZ) lesions were found in 4 of 45 MS patients, accounting for 9 % (95 % CI: 3 %-17 %), and were absent in the NMOSD group; however, there was no significant difference between the two groups (p = 0.12). Of the MS patients with pontine trigeminal nerve lesions, 6 out of 9 (63 %; 95 % CI, 36 %-98 %) exhibited bilateral lesions, which was significantly more prevalent compared to the NMOSD group (13 % vs. 0 %, p = 0.03).
CONCLUSIONS: The presence of pontine trigeminal nerve lesions, particularly when bilateral, are significantly more prevalent in MS patients than in those with NMOSD, suggesting their utility as a distinctive marker and potential diagnostic indicator specifically for MS.

Aims/Background Although electromyography has been extensively used in the diagnosis of neurological diseases, there is no comprehensive understanding of the electromyography manifestations of spinal dural arteriovenous fistula. Given the widespread use of electromyography in the diagnosis of neurological conditions, it is worthwhile to holistically analyse the electromyography findings of spinal dural arteriovenous fistula to differentiate it from neurological diseases that share similar clinical manifestations. The aim of this study is to evaluate whether electromyography can distinguish spinal dural arteriovenous fistula from longitudinally extensive transverse myelitis. Methods We holistically reviewed files of all patients who were diagnosed with spinal dural arteriovenous fistula or longitudinally extensive transverse myelitis at The First Medical Centre of PLA General Hospital from 1 January 2010 to 31 December 2020. We compared the symptomology, epidemiology, and imaging results of patients with spinal dural arteriovenous fistula and longitudinally extensive transverse myelitis, placing emphasis on their electromyography manifestations. Student\'s t test was used to analyse normally distributed data, while Chi-square test was used to compare classification statistics. Results Lesions of spinal dural arteriovenous fistula shown on images tend to appear at lower lumbar and sacral segments, whereas lesions of the cervical and upper thoracic segments are more characteristic of longitudinally extensive transverse myelitis. Spinal dural arteriovenous fistula patients and longitudinally extensive transverse myelitis patients overlap in terms of clinical manifestations. After comparison, the two groups of patients had different demographics (age, sex), onset mode, predisposing factors before onset, and electromyographic features. The electromyographic features of patients with spinal dural arteriovenous fistula were associated with neurogenic damage (p < 0.001). Conclusions In patients with spinal dural arteriovenous fistula, electromyography can help clinicians to identify early disease, avoid patient treatment delay, and eliminate unnecessary treatment.

BACKGROUND: Although the relationship between migraine and multiple sclerosis (MS) has been reported, the risk of migraine in MS and neuromyelitis optica spectrum disorder (NMOSD) is unclear. Therefore, this study investigated the risk of migraine in the Korean MS and NMOSD populations.
METHODS: This study analyzed claims data from 1,492 patients with MS and 1,551 patients with NMOSD based on diagnostic codes in the Korean National Health Insurance Service. Migraine risk was compared with a control group (matched 1:5 for age, sex, and comorbidities) using Cox proportional hazards analysis. Patients aged <20 years and with previous migraine were excluded.
RESULTS: Migraine risk was higher in patients with MS (adjusted hazard ratio [aHR] 1.37; 95% confidence interval [CI]: 1.15-1.62) but did not differ significantly in patients with NMOSD (aHR 1.05; 95% CI: 0.87-1.27) compared to controls. No significant sex-based differences in migraine risk were observed. Patients with NMOSD showed decreasing risk with age (p for interaction = 0.040). Comorbidities like hypertension, diabetes, or dyslipidemia did not significantly alter migraine risk in either group.
CONCLUSIONS: The study results revealed an increased risk of migraines in patients with MS but not in patients with NMSOD compared with matched controls.

UNASSIGNED: Neuromyelitis optica spectrum disorder (NMOSD) is a severe and rare inflammatory disease affecting the central nervous system through optic neuritis and transverse myelitis. Present study aimed to investigate the association between dietary inflammatory index (DII) and risk of NMOSD.
UNASSIGNED: In this case-control study, 30 NMOSD cases and 90 aged matched healthy individuals were recruited. Habitual dietary intakes were assessed by a validated 168-item food frequency questionnaire to calculate the DII score. A multiple adjusted regression was used to determine the odd ratio (OR) of NMOSD across DII tertiles. The Residual method was applied to adjust the energy intake.
UNASSIGNED: Participants in the top of DII tertile were more likely to have NMOSD in the crude model compared to those with the lowest one (OR: 4.18; 95%CI: 1.43-12.21). It was the case when multivariable confounders were considered in adjustment model I (OR: 3.98; 95%CI: 1.34-11.82) and II (OR: 4.43; 95%CI: 1.36-14.38), such that, individuals with a greater DII score had 3.98 and 4.43-time higher risk of NMOSD in model I and II, respectively.
UNASSIGNED: The Present study suggests that greater adherence to a pro-inflammatory diet may be associated with an increased risk of NMOSD.

UNASSIGNED: Complementary and alternative medications (CAM) are common among patients with multiple sclerosis (MS) for physical and psychological support. However, there is insufficient data regarding the application of CAM in the different cultures and beliefs of each community as well as patient\'s status.
UNASSIGNED: To evaluate the prevalence and modalities of the use of CAM among patients with central nervous system idiopathic inflammatory demyelinating diseases (CNS-IIDD) in a tertiary care hospital.
UNASSIGNED: A cross-sectional study was conducted at Siriraj Hospital from June to December 2021 involving patients with MS, neuromyelitis optic spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), idiopathic transverse myelitis (iTM), and optic neuritis (ON) to examine the prevalence and mode of CAM use and its correlation with patient characteristics.
UNASSIGNED: There were 107 patients. The diagnoses were MS (38), NMOSD (55), MOGAD (5), iTM (7), and ON (2). Most of the patients were female (89.7%), and 61.7% were diagnosed over 5 years. The mean Expanded Disability Status Scale was 2.63 (S.D., 2.38), and the median ambulation index was 0 (range 0-8.5). There were 68 patients (63.6%) with a history of CAM use for at least 3 months, while those with current use decreased to 62 (58.5%). Vitamins and minerals were the most commonly used, particularly vitamin D (97.1%) and calcium (47.7%). Both treatments were primarily prescribed (95.3%) rather than self-administered (24.3%). The main reasons for the use of CAM were to strengthen their health (48.6%) and relieve existing symptoms (28.0%).
UNASSIGNED: The use of CAM is common among patients with Thai CNS-IIDD. Further exploration of patient perspectives and preferences regarding CAM usage may contribute to a more comprehensive management approach for patients with CNS-IIDD.

BACKGROUND: Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory autoimmune disease with high risk of recurrence and disability, the treatment goal is a recurrence free state. Area postrema (AP) is one of the most common involved area of NMOSD, which may have a particular significance in the pathogenesis of NMOSD and clinical heterogeneity. Our study is to investigate the clinical and recurrent characteristics AP onset NMOSD patients.
METHODS: A retrospective study was done in a cohort of 166 AQP4-IgG seropositive NMOSD patients which were identified by the 2015 IPND criteria. The patients were divided into AP onset (APO-NMOSD) group and non-AP onset (NAPO-NMOSD) group based on the initial episode location. Clinical features and recurrence differences of two groups were compared.
RESULTS: The APO-NMOSD group and NAPO-NMOSD group had a population ratio of 24:142. APO-NMOSD patients were younger (34.6y VS 42.3y, P = 0.013), had lower EDSS at first episode (0.7 VS 4.2, p = 0.028) and last follow up (1.9 VS 3.3, p = 0.001), more likely to have multi-core lesions at the first attack (33.3% VS 9.2%, P = 0.001). Also, they had a higher annual recurrence rate (0.4 ± 0.28 VS 0.19 ± 0.25, P = 0.012). In natural course NMOSD patients without immunotherapy, APO-NMSOD had a shorter time of first relapse (P < 0.001) and higher annual recurrence rate (0.31 ± 0.22 VS 0.16 ± 0.26, P = 0.038) than NAPO-NMOSD. APO-NMOSD group also have a higher risk of having the first relapsing compared to optic neuritis onset-NMOSD (HR 2.641, 95% CI 1.427-4.887, p = 0.002) and myelitis onset-NMOSD group (HR 3.593, 95% CI 1.736-7.438, p = 0.001). Compared to NAPO-NMOSD, APO-NMOSD has a higher likelihood of brainstem recurrence (28.6% vs. 4.7%, p<0.001) during the first recurrence, while NAPO-NMOSD is more susceptible to optic nerve involvement (10.7% vs. 41.1%, p = 0.01).
CONCLUSIONS: AQP4-IgG seropositive NMOSD patients with AP onset are youngers and have higher risk of recurrence. Clinicians should pay attention to AP damage in NMOSD, as it indicates a potential risk of recurrence.
BACKGROUND: Retrospectively registered.

UNASSIGNED: An increasing body of research has demonstrated a robust correlation between circulating inflammatory proteins and neuromyelitis optica spectrum disorders (NMOSD). However, whether this association is causal or whether immune cells act as mediators currently remains unclear.
UNASSIGNED: We employed bidirectional two-sample Mendelian randomization (TSMR) analysis to examine the potential causal association between circulating inflammatory proteins, immune cells, and NMOSD using data from genome-wide association studies (GWAS). Five different methods for Mendelian randomization analyses were applied, with the inverse variance-weighted (IVW) method being the primary approach. Sensitivity analyses were further performed to assess the presence of horizontal pleiotropy and heterogeneity in the results. Finally, a two-step Mendelian randomization (MR) design was employed to examine the potential mediating effects of immune cells.
UNASSIGNED: A notable causal relationship was observed between three circulating inflammatory proteins (CSF-1, IL-24, and TNFRSF9) and genetically predicted NMOSD. Furthermore, two immune cell phenotypes, genetically predicted CD8 on naive CD8+ T cells, and Hematopoietic Stem Cell Absolute Count were negatively and positively associated with genetically predicted NMOSD, respectively, although they did not appear to function as mediators.
UNASSIGNED: Circulating inflammatory proteins and immune cells are causally associated with NMOSD. Immune cells do not appear to mediate the pathway linking circulating inflammatory proteins to NMOSD.