Neuromyelitis

BACKGROUND: Optic neuritis (ON) is a common feature of inflammatory demyelinating diseases (IDDs) such as multiple sclerosis (MS), aquaporin 4-antibody neuromyelitis optica spectrum disorder (AQP4 + NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, the involvement of the optic chiasm (OC) in IDD has not been fully investigated. AIMS: To examine OC differences in non-acute IDD patients with (ON+) and without ON (ON-) using magnetisation transfer ratio (MTR), to compare differences between MS, AQP4 + NMOSD and MOGAD and understand their associations with other neuro-ophthalmological markers...

Subclavian artery injuries during internal jugular vein puncture when attempting central venous catheter insertion are rare. A 60-year-old man undergoing treatment for neuromyelitis optica with paralysis and sensory loss developed a complication during catheter placement into his right internal jugular vein for plasmapheresis. His previous physician felt resistance and discontinued the procedure. The patient later developed mild dyspnea and dysphagia. Computed tomography scans indicated thrombus formation and tracheal deviation...

BACKGROUND: The diagnosis and treatment of autoimmune optic neuritis (ON) has improved with the accessibility and reliability of aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody testing, yet autoantibody-negative ON remains common. This study describes the demographic, clinical, and outcome data in patients with isolated ON across the pediatric and adult cohort. METHODS: A retrospective chart review of University of Utah Health patients with the International Classification of Diseases (ICD) code of ICD-9 377...

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UNLABELLED: Paediatric acquired demyelinating syndrome(ADS) constitute group of treatable disorders with acute neurologic dysfunction. Neuroimaging has played significant role in diagnosis of ADS. We describe clinico-radiologic spectrum, outcome and compare the groups Acute disseminated encephalomyelitis (ADEM), Neuromyelitis-optica-spectrum disorder (NMOSD), clinically-isolated-syndrome (CIS), Multiple sclerosis (MS) and Myelin-oligodendrocyte-glycoprotein antibody associated disorders (MOGAD)...

BACKGROUND: Lycium barbarum glycopeptide (LbGp), extracted from the traditional Chinese medicine (TCM) of Lycium barbarum (LB), provides a neuroprotective effect against neurodegenerative and neuroimmune disorders contributing to its immunomodulatory and anti-inflammatory roles. Neuromyelitis optica spectrum disorders (NMOSD) is an autoimmune-mediated central nervous system (CNS) demyelinating disease, clinically manifested as transverse myelitis (TM) and optic neuritis. However, no drug has been demonstrated to be effective in relieving limb weakness and visual impairment of NMOSD patients...

BACKGROUND: Recent evidence shows that immunosuppressive agents can affect the gut microbiota in autoimmune diseases. However, the relationship between the gut microbiome and B-cell depletion immunotherapy in neuromyelitis optica spectrum disorder (NMOSD) remains poorly understood. OBJECTIVES: To evaluate the distinct intestinal microbial patterns and serum cytokine levels after short-term rituximab treatment (three months) in patients with NMOSD. METHODS: Firstly, we conducted a cross-sectional study involving 46 treatment-naïve NMOSD patients and 48 matched healthy controls...

INTRODUCTION: We aimed to assess the frequency, duration, and severity of area postrema syndrome (APS) during follow-up in neuromyelitis optica spectrum disorder (NMOSD) patients, as well as its association with inflammatory activity and prognostic factors of APS severity in a real-world setting. METHODS: We conducted a retrospective study on a cohort of Latin American (LATAM) NMOSD patients who had experienced APS during their follow-up. Patients from Mexico, Peru, Brazil, Colombia, Panama, Chile and Argentina patients who met 2015 NMOSD criteria were included...

Monoclonal antibody therapies mark the new era of targeted treatment for relapse prevention in aquaporin-4 (AQP4)-immunoglobulin G (IgG)-positive neuromyelitis optica spectrum disorder (AQP4-IgG+NMOSD). For over a decade, rituximab, an anti-CD20 B-cell-depleting agent, had been the most effectiveness treatment for AQP4-IgG+NMOSD. Tocilizumab, an anti-interleukin-6 receptor, was also observed to be effective. In 2019, several randomized, placebo-controlled trials were completed that demonstrated the remarkable efficacy of eculizumab (anti-C5 complement inhibitor), inebilizumab (anti-CD19 B-cell-depleting agent), and satralizumab (anti-interleukin-6 receptor), leading to the Food and Drug Administration (FDA) approval of specific treatments for AQP4-IgG+NMOSD for the first time...

Neuro-ophthalmologic diseases include a broad range of disorders affecting the afferent and efferent visual pathways. Recently, monoclonal antibody (mAb) therapies have emerged as a promising targeted approach in the management of several of these complex conditions. Here, we describe the mechanism-specific applications and advancements in neuro-ophthalmologic mAb therapies. The application of mAbs in neuro-ophthalmologic diseases highlights our increasing understanding of disease-specific mechanisms in autoimmune conditions such as neuromyelitis optica, thyroid eye disease, and myasthenia gravis...

Optic neuritis (ON) is a debilitating condition that through various mechanisms, including inflammation or demyelination of the optic nerve, can result in partial or total permanent vision loss if left untreated. Accurate diagnosis and promptly initiated treatment are imperative related to the potential of permanent loss of vision if left untreated, which can lead to a significant reduction in the quality of life in affected patients. ON is subtyped as "typical" or "atypical" based on underlying causative etiology...

OBJECTIVES: Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE's safety and feasibility data in these conditions. MATERIALS & METHODS: The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON)...

Cognitive impairment (CI) has been reported in 29-70% of patients with neuromyelitis optica spectrum disorder (NMOSD). Abnormal white matter (WM) functional networks that correlate with cognitive functions have not been studied well in patients with NMOSD. The aim of the current study was to investigate functional connectivity (FC), spontaneous activity, and functional covariance connectivity (FCC) abnormalities of WM functional networks in patients with NMOSD and their correlation with cognitive performance...

INTRODUCTION: Cognitive impairment has a substantial impact on the daily function of people living with demyelinating diseases. However, the study of cognitive failures and their association with clinical variables in people suffering from neuromyelitis optica spectrum disorder (NMOSD) has been scarce, especially in the latin american (Mexican) population at early and middle stages of the disease. METHOD: We applied the Rao's Brief Repeatable Battery of Neuropsychological tests and obtained data of lesion burden through magnetic resonance imaging (MRI), expression of AQPQ4-IgG antibodies, and degree of disability in 30 patients with NMOSD and 30 healthy participants as a control group...

We report a rare case of paraneoplastic neurological syndrome with dual seropositivity of anti-aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies in a 40 year-old woman with metastatic triple-negative breast cancer. She received multiple lines of anti-neoplastic treatment, including immunotherapy with pembrolizumab, as well as cytotoxic chemotherapy. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab...

Neuromyelitis optica is an inflammatory demyelinating disease of the central nervous system that differs from multiple sclerosis. Over the past 20 years, the search for biomarkers for neuromyelitis optica has been ongoing. Here, we used a bibliometric approach to analyze the main research focus in the field of biomarkers for neuromyelitis optica. Research in this area is consistently increasing, with China and the United States leading the way on the number of studies conducted. The Mayo Clinic is a highly reputable institution in the United States, and was identified as the most authoritative institution in this field...

OBJECTIVE: The glymphatic system is a glial-based perivascular network that promotes brain metabolic waste clearance. Glymphatic system dysfunction has been observed in both multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD), indicating the role of neuroinflammation in the glymphatic system. However, little is known about how the two diseases differently affect the human glymphatic system. The present study aims to evaluate the diffusion MRI-based measures of the glymphatic system by contrasting MS and NMOSD...

Herein, the author summarize the basic findings on the neuropathology of inflammatory and autoimmune central nervous system (CNS) diseases. Current knowledge on infectious, demyelinating, and autoimmune diseases have also been reported. Further, I emphasize the importance of considering the neuropathology of meningitis, encephalitis, and abscesses as infectious diseases; multiple sclerosis and neuromyelitis optica as demyelinating diseases; and vasculitis, paraneoplastic neurological syndrome, and collagen diseases as autoimmune diseases...

This is a report on remarkable visual recovery from blindness in a case of isolated optic perineuritis (OPN). A 68-year-old Chinese lady presented with a two-week history of progressive painless bilateral vision loss. Her vision was 6/18 on the right eye and no perception of light (NPL) on the left eye with positive relative afferent pupillary defect (RAPD). Fundus showed hyperaemic and swollen optic disc bilaterally. MRI of the brain and orbit revealed hyperintense periventricular white matter lesions, possibly early changes of multiple sclerosis (MS), and perineural enhancement of optic nerve bilaterally, consistent with OPN...

BACKGROUND: Forkhead box P3 ( FOXP3 ) plays a critical role in the pathogenesis of autoimmune disorders. In the present study, we genotyped three single-nucleotide polymorphisms, namely, rs2232365, rs3761548, and rs3761549, to determine the relationship between FOXP3 polymorphisms and neuromyelitis optica spectrum disorder (NMOSD) susceptibility among the Northern Chinese Han population. MATERIALS AND METHODS: We genotyped single nucleotide polymorphisms at loci of the FOXP3 gene (rs2232365, rs3761548, and rs3761549136) in 136 NMOSD patients and 224 healthy subjects using the multiplex SNaPshot technique...