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.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is the central nervous system demyelinating disease differentiated from multiple sclerosis by the presence of anti-aquaporin 4-antibody (AQP4-ab), which is sometimes accompanied by non-organ-specific autoantibodies.
METHODS: We prospectively collected clinical information and profiles of non-organ-specific autoantibodies such as fluorescent antinuclear (FANA), anti-Sjögren\'s syndrome A (SSA)/Ro, anti-SS B (SSB)/La, anti-neutrophil cytoplasmatic (ANCA), lupus anticoagulant (LA), anti-cardiolipin (ACA), anti-double-stranded DNA (dsDNA), rheumatoid factor (RF), anti-thyroperoxidase, and anti-thyroglobulin antibodies in patients with NMOSD. Clinical characteristics and laboratory findings of patients with NMOSD with or without autoantibodies were analyzed. Cox proportional hazard models were used to identify independent risk factors predicting high disability in patients with NMOSD.
RESULTS: A total of 158 patients with NMOSD (Female: Male = 146:12; age, 36.11 ± 14.7) were included. FANA was observed most frequently (33.3 %), followed by anti-SSA (28.6 %), anti-SSB (10.0 %), RF (8.5 %), anti-dsDNA (7.0 %), LA (4.7 %), ACA (4.8 %), and ANCA (2.4 %). High disability (Expanded Disability Status Scale (EDSS) score ≥ 6) was observed more frequently in patients with RF (45.5 %) than in those without RF (14.5 %) (p = 0.02). RF was a significant predictive factor for the high disability (hazard ratio [HR], 3.763; 95 % confidence interval [CI], 1.086-13.038; p = 0.037), age at onset (HR, 1.093; 95 % CI, 1.05-1.14; p ≤0.001), and annual relapse rate (ARR) (HR, 4.212; 95 % CI, 1.867-9.503; p = 0.001).
CONCLUSIONS: Organ-specific and non-organ-specific autoantibodies are frequently observed in Korean patients with AQP4-ab-positive NMOSD. RF may be an independent predictor of high disability, along with age at onset and ARR.

UNASSIGNED: To explore ocular changes in patients with MS and NMOSD via SD-OCT and PVEP analysis.
UNASSIGNED: From August 2020 to July 2021, 82 patients (164 eyes) diagnosed with MS, 59 patients (118 eyes) diagnosed with NMOSD and 50 healthy controls (100 eyes) were retrospectively selected. SD-OCT and PVEP were performed to compare retinal nerve fibre layer (RNFL) thickness around the optic disc, ganglion cell inner plexiform layer (GCIPL) thickness in the macula and P100 latency and amplitude between the disease groups and the control group.
UNASSIGNED: In the NMOSD and MS groups, the thickness of the GCIPL quadrants in eyes with optic neuritis was thinner than that in eyes without optic neuritis, and the amplitude of the P100 wave decreased. In addition, in eyes with optic neuritis, patients with NMOSD have thinner RNFL thicknesses in the temporal and superior quadrants than patients with MS, and the thickness of the GCIPL is thinner in each region. In eyes without optic neuritis, patients with MS have thinner nasal RNFL than do those with NMOSD.
UNASSIGNED: SD-OCT and VEP may be useful for monitoring and distinguishing pathological changes in MS and NMOSD patients.

UNASSIGNED: In patients with SLE, concurrent NMOSD can manifest with optic neuritis and transverse myelitis. AQP-4 antibody positivity confirms the diagnosis. Prompt treatment is critical to manage the acute symptoms and prevent relapses, as highlighted by a young patient\'s case with optic neuritis and extensive spinal cord lesions.
UNASSIGNED: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system that affects the optic nerve and spinal cord. It is associated with autoantibodies against aquaporin-4 (AQP-4) and/or myelin oligodendrocytes glycoproteins. It is diagnosed based on clinical, radiological, and serological criteria, and treated with immunosuppressants in the acute phase. Long-term immunosuppression is essential to prevent potential relapses. In this case report, we present the case of a 19-year-old female patient with systemic lupus erythematosus (SLE), who presented with blurriness and loss of vision in her left eye. Optical coherence tomography was normal, but a gadolinium-enhanced cervico-dorsal MRI showed multiple lesions extending from the brainstem to the C7-T1 junction suggestive of longitudinally extensive transverse myelitis (LETM), the largest of which was a cystic lesion at the cervico-spinal junction. A contrast injection also revealed left optic neuritis. Cerebrospinal fluid analysis showed elevated IgG and red blood cell count, but no oligoclonal bands. The patient tested positive for AQP-4 autoantibodies, confirming the diagnosis of NMOSD. Treatment with intravenous methylprednisolone led to partial improvement, but the patient experienced a relapse with severe neurological symptoms, including tetraplegia and bladder and bowel dysfunction. This case illustrates the importance of considering NMOSD in the differential diagnosis of patients with SLE who present with optic neuritis and/or myelitis, especially when MRI findings are suggestive of LETM. Early diagnosis and adherence to treatment are crucial to prevent further relapses and deleterious sequelae.

This study aimed to identify the prevalence, clinical and radiographic characteristics, and risk factors for cognitive dysfunction in patients with Neuromyelitis optica spectrum disorder (NMOSD). Eighty-three participants who were diagnosed with NMOSD were recruited. Cognitive function was assessed using the Montreal Cognitive Assessment (MoCA) and Frontal Assessment Battery (FAB). The mean age of the patients was 47.78 ± 13.14 years, with an average of 12.05 ± 4.62 years of formal education. The majority (54%) exhibited cognitive impairment, defined by a MoCA score < 25 (mean: 22.96 ± 3.82). Disease severity (evaluated by the Expanded Disability Status Scale) and lower formal education levels were associated with cognitive impairment (p = 0.011 and < 0.001, respectively). The annualized relapse rate, disease duration, and AQP4 antibody status were not associated with cognitive impairment. Interestingly, informant-reported cognitive decline was associated with poorer cognitive performance (p = 0.027). Radiographic findings of lesion location and severity were associated with MoCA-assessed cognitive performance, particularly for lesions in the right parietal lobes (p = 0.023). Hippocampal atrophy was negatively correlated with FAB scores. In conclusion, approximately half of the Thai patients with NMOSD exhibited cognitive impairment, which was associated with age, formal education level, disease severity, relative perception, and specific radiological findings. Further studies incorporating comprehensive neuropsychological tests and subjective cognitive complaints are warranted.

Acute transverse myelitis (TM) is a rare, acquired neuro-immune spinal cord disorder that can be idiopathic or related to a secondary disease. Clinical signs and symptoms include motor weakness, sensory alterations, and bowel or bladder dysfunction. Often TM occurs in the younger population or middle-aged adults. This patient\'s presentation is unique in the fact that he does not fall into either of these age categories. In this case, a 72-year-old male with a past medical history of hypertension and type 2 diabetes mellitus presented to the emergency department due to a five-day history of worsening weakness of the upper extremities bilaterally. In addition, the patient reported a new onset of abdominal wall numbness. The patient reported being at a theme park a few days prior, denying any injuries and only complaining of neck discomfort during the car ride home. Labs and imaging were quickly ordered for diagnostic purposes. The patient was diagnosed with TM using magnetic resonance imaging (MRI), lumbar puncture, and clinical signs. The etiology was later discovered to be due to a new diagnosis of Sjögren\'s autoimmune disease. The patient was treated with high-dose intravenous steroids for five days while being monitored for any neurologic changes. The plan was to continue steroids by mouth once discharged from the hospital. Due to poor adherence to discharge instructions, the patient was readmitted after presenting to the emergency department with worsening symptoms. Physicians need to recognize and diagnose TM quickly, as some etiologies are treatable and can prevent further damage to the spinal cord.

Symmetrical lesions in the temporal poles and external capsules on brain MRI are known as radiological markers of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL); however, similar imaging findings have also been reported in neuromyelitis optica spectrum disorder (NMOSD), and this study investigated the frequency of such findings. The study included 55 NMOSD patients who met the 2015 international NMO diagnosis panel (IPND) criteria and were positive for aquaporin-4 antibodies (AQP4-Ab). Images were evaluated based on the consensus of two neuroradiologists, and brain lesions were detected in 33 patients, of whom 2 (6%) had symmetrical lesions in both the temporal poles and external capsules, and 1 (3%) had symmetrical lesions confined to the external capsules. Therefore, when symmetrical lesions in the temporal poles and external capsules are observed on MRI, NMOSD should be considered in the differential diagnosis.

BACKGROUND: The previous Japanese clinical practice guidelines for multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) were published in 2017. Recently, for the first time in 6 years, the MS and NMOSD guideline development committee revised the Japanese guidelines for MS, NMOSD, and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
METHODS: The committee utilized the Grading of Recommendations Assessment, Development, and Evaluation system based on the \"Minds Handbook for Clinical Practice Guideline Development 2020 Ver. 3.0″ with a focus on clinical questions (CQs). The committee also discussed clinical issues other than CQs, categorizing them as a question-and-answer (Q&A) section, including \"issues on which experts\' opinions agree to a certain extent\" and \"issues that are important but not included in the CQ\".
RESULTS: The committee identified 3, 1, and 1 key CQs related to MS, NMOSD, and MOGAD, respectively, and presented recommendations. A Q&A session regarding disease-modifying therapies and relapse prevention therapies for MS, NMOSD, and MOGAD was conducted. The revised guidelines were published in September 2023.
CONCLUSIONS: The Japanese guidelines for clinical practice on MS, NMOSD, and MOGAD were updated. Treatment strategies for MS, NMOSD, and MOGAD are changing, and these updated guidelines may assist with treatment decisions for these diseases in clinical practice.

Intestinal microbes play a crucial role in gut health and the immune-mediated central nervous system through the \"gut-brain\" axis. However, probiotic safety and efficacy in Neuromyelitis optica spectrum disorder (NMOSD) are not well-explored. A pilot clinic trial for NMOSD with probiotic intervention revealed alterations in the microbiota (increased Anaerostipes, Bacteroides; decreased Granulicatella, Streptococcus, Rothia). Metabolite analysis showed elevated 2-methylbutyric and isobutyric acids, reduced lithocholic acid (LCA), and glycodeoxycholic acid (GDCA). Immune markers Interleukin (IL-7), vascular endothelial growth factor (VEGF-A), and B lymphocyte chemoattractant (BLC) decreased, while plasma cells and transitional B cells increased post-probiotics, suggesting potential immunomodulatory effects on NMOSD.

Primary Sjögren\'s syndrome is an autoimmune disorder that is characterized by lymphocytic infiltration of salivary and lacrimal glands. The extra-glandular manifestations might be arthritis, myalgia, glomerulonephritis, skin rashes, and neurologic involvement. One of the uncommon neurologic manifestations is neuromyelitis optica spectrum disorder (NMOSD). In the present case, an older woman is reported that was diagnosed with NMOSD secondary to keratoconjunctivitis sicca, which is rare in geriatric practice.