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Myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease is a neuroinflammatory disorder (MOGAD) with heterogeneous phenotype including paroxysms of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, brainstem demyelination, and encephalitis. Fluid-attenuated inversion recovery hyperintense cortical lesions in MOG-associated encephalitis with seizures, or FLAMES, is a manifestation of cerebral cortical encephalitis seen less frequently than other typical MOG antibody–associated disease presentations. Cases of FLAMES are rarer in children, and frequently initially misdiagnosed with infectious meningoencephalitis. Other meningocortical manifestations of MOG antibody–associated disease have been described and likely exist along a continuum. In this retrospective single-center case series, we describe the demographic, clinical, radiographic, laboratory, and electroencephalographic features of 5 children with clinicoradiographic features consistent with the spectrum of MOG-IgG–positive meningocortical syndromes. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to investigate the clinical characteristics and predictors of relapse in double negative atypical inflammatory demyelinating disease (IDD) and to explore potential antigenic targets by tissue‐based assays (TBA) using rat brain indirect immunofluorescence. Methods: We compared the clinical, laboratory, and MRI data of double negative atypical IDD with other IDD patients. Serum samples were collected for TBA. The predictors of relapse were examined over a minimum of 24 months follow‐up. Results: In our cohort of 98 patients with double negative atypical IDD, there was no significant female predominance (58.2%, 57/98). The lesions primarily affected the spinal cord and brain stem, with fewer cases of involvement in the area postrema (5.1%, 5/98) and longitudinally extensive transverse myelitis (43.9%, 43/98). A total of 62.5% (50/80) patients tested positive for anti‐astrocyte antibodies based on rat brain TBA. Over a median duration of 39.5 months, 80 patients completed the entire follow‐up, and 47.5% (38/80) patients exhibited monophasic course. A total of 36% (18/50) patients positively for anti‐astrocyte antibodies had a monophasic course, which is significantly lower than patients negatively for anti‐astrocyte antibodies (66.7%, 20/30) (p = 0.008). The presence of anti‐astrocyte antibodies (hazard ratio (HR), 2.243; 95% CI, 1.087–4.627; p = 0.029) and ≥4 cerebrum lesions at first attack (HR, 2.494; 95% CI, 1.224–5.078; p = 0.012) were risk factors for disease relapse, while maintenance immunotherapy during remission (HR, 0.361; 95% CI, 0.150–0.869; p = 0.023) was protective factor. Interpretation: Double negative atypical IDD are unique demyelinating diseases with a high relapse rate. Maintenance immunotherapy is helpful to the prevention of relapse, particularly in patients with anti‐astrocyte antibodies or ≥4 cerebrum lesions at first attack. [ABSTRACT FROM AUTHOR]

Background and objectives: Neuromyelitis optica spectrum disorder (NMOSD) is a group of demyelinating diseases of the nervous system with high relapse rate and high disability rate without treatment, and we aimed to explore the influencing factors related to the recurrence of NMOSD and provide basis for clinical treatment in this study. Methods: Referring to the diagnostic criteria for NMOSD issued in 2015, 259 patients were enrolled. Clinical information, cerebrospinal fluid (CSF) and serum analysis results, brain and spinal cord magnetic resonance imaging (MRI) findings, treatment details, and prognosis were all recorded. Results: 176 (68.00%) participants were found to be AQP4 Ab-positive in serum or CSF, and the relapse rate was 36.67% (95/259). These 259 individuals were separated into two groups: non-release (n = 164) and relapse (n = 95). In terms of EDSS scores at onset, EDSS score after treatment, lesion location, serum creatinine (Cr) and treatment strategy, there were statistical differences between the two groups. Multivariable logistic regression analyses revealed five predictors for recurrence of NMOSD patients within two years: EDSS scores at onset, transverse myelitis, brain/brainstem, Cr, and Rituximab/immunosuppressants. Conclusion: It is essential to explore the risk factors related to recurrence and prevent them to reduce the risk of disability and improve the prognosis, and the recurrence rate of NMOSD may be affected by several factors. [ABSTRACT FROM AUTHOR]

Background: Idiopathic transverse myelitis (iTM) is defined as an inflammatory myelopathy of undetermined etiology, even after a comprehensive workup to identify other possible causes. Generally, the characteristics of recurrent iTM are not clearly defined. This study aimed to identify the clinical characteristics and predictive factors of recurrence in patients with iTM. Methods: We retrospectively recruited patients with transverse myelitis (TM) who visited Chungnam National University Hospital between January 2011 and December 2021. We included patients who were followed up for at least 2 years and excluded those diagnosed with multiple sclerosis or neuromyelitis optica spectrum disorder (NMOSD) during the initial episode or follow-up period. Patients with iTM were categorized into two groups: monophasic idiopathic TM (mTM) and recurrent idiopathic TM (rTM). We compared the clinical characteristics and spinal magnetic resonance imaging findings between the two groups. Results: In total, 167 patients were reviewed, of whom 112 were excluded. Finally, we included 55 patients with iTM. In 55 patients, 11 (20.0%) and 44 (80%) were classified into the rTM and mTM groups, respectively. Male predominance was observed in the iTM, rTM, and mTM groups. The percentage of patients with low vitamin D levels was significantly higher in the rTM group (100.0%) compared with the mTM group (70%) (p = 0.049). In addition, longitudinally extensive transverse myelitis (LETM) was observed more frequently in the rTM group, in 8 of 11 (72.7%) patients, compared with 15 of 44 (34.1%) patients in the mTM group, with the difference being statistically significant (p = 0.020). In multivariate regression analysis, female sex, younger age at onset, low serum vitamin D level (<30 ng/mL), and LETM were risk factors for recurrence. LETM was a significant predictor of relapse in iTM (p = 0.043, odds ratio = 13.408). Conclusion: In this study, the clinical features of mTM and rTM are nearly indistinguishable. In conclusion, >20% of the patients with iTM experience recurrence, and LETM is the most significant risk factor for recurrence. In cases of recurrence, there is a favorable response to immunotherapy, and the prognosis is generally good. Although LETM may be the initial symptom of NMOSD, it may be manifestation of iTM, and in cases of idiopathic LETM, it is important to be mindful of the elevated risk of recurrence. Based on these results, idiopathic rTM has good clinical prognosis and response to immunosuppressive treatment. [ABSTRACT FROM AUTHOR]

Background and Purpose Methods Results Conclusions The diagnostic criteria for myelin oligodendrocyte glycoprotein antibody (MOG‐IgG)‐associated disease (MOGAD) were published in 2023. We aimed to determine the performance of the new criteria in Latin American (LATAM) patients compared with the 2018 criteria and explore the significance of MOG–IgG titers in diagnosis.We retrospectively reviewed the medical records of LATAM (Argentina, Chile, Brazil, Peru, Ecuador, and Colombia) adult patients with one clinical MOGAD event and MOG‐IgG positivity confirmed by cell‐based assay. Both 2018 and 2023 MOGAD criteria were applied, calculating diagnostic performance indicators.Among 171 patients (predominantly females, mean age at first attack = 34.1 years, mean disease duration = 4.5 years), 98.2% patients met the 2018 criteria, and of those who did not fulfill diagnostic criteria (n = 3), all tested positive for MOG‐IgG (one low‐positive and two without reported titer). Additionally, 144 (84.2%) patients met the 2023 criteria, of whom 57 (39.5%) had MOG‐IgG+ titer information (19 clearly positive and 38 low‐positive), whereas 87 (60.5%) patients had no MOG‐IgG titer. All 144 patients met diagnostic supporting criteria. The remaining 27 patients did not meet the 2023 MOGAD criteria due to low MOG‐IgG (n = 12) or lack of titer antibody access (n = 15), associated with the absence of supporting criteria. The 2023 MOGAD criteria showed a sensitivity of 86% (95% confidence interval = 0.80–0.91) and specificity of 100% compared to the 2018 criteria.These findings support the diagnostic utility of the 2023 MOGAD criteria in an LATAM cohort in real‐world practice, despite limited access to MOG‐IgG titration. [ABSTRACT FROM AUTHOR]

Key Clinical Message: 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. 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. [ABSTRACT FROM AUTHOR]

Background: Retrospective studies suggest that spinal movement disorders, especially tonic spasms, are prevalent in NMOSD. However, there have been no prospective studies evaluating spinal movement disorders in NMOSD, MOGAD, and idiopathic transverse myelitis (ITM). Methods: Patients referred to a tertiary neuroimmunology clinic for spinal cord demyelination (excluding MS) were evaluated. All patients answered a movement disorders survey and underwent a movement disorder-focused exam. Movement disorders were compared among patients with NMOSD with and without AQP4-IgG, MOGAD, and ITM. Patients with and without involuntary movements were also compared to identify predictors of spinal movement disorders. Results: Sixty-three patients were evaluated from 2017 to 2021 (71% females, median age 49 years, range 18–72 years, median disease duration 12 months, range 1–408). Of the total, 49% had ITM, 21% had NMOSD without AQP4-IgG, 19% had NMOSD with AQP4-IgG, and 11% had MOGAD. Movement disorders were present in 73% of the total patients and were most frequent in NMOSD with AQP4-IgG (92%) and least frequent in MOGAD (57%). The most frequent spinal movement disorders were tonic spasms (57%), focal dystonia (25%), spinal tremor (16%), spontaneous clonus (9.5%), secondary restless limb syndrome (9.5%), and spinal myoclonus (8%). Multivariate analysis showed that longitudinally extensive myelitis and AQP4-IgG are independent risk factors for the development of spinal movement disorders, while MOG-IgG and African American race were associated with a lower risk of developing these movement disorders. Conclusions: Spinal movement disorders are highly prevalent in non-MS demyelinating disorders of the spinal cord. Prevalence rates exceed those reported in MS and retrospective NMOSD studies. [ABSTRACT FROM AUTHOR]

We aimed to study the risks of relapse and long term disability in children with non-MS acquired demyelinating syndromes (ADS). In this prospective, multi-centre study, from the 14 UK pediatric neurology centres, children (<16 years) experiencing a first episode of ADS were recruited from 2010 to 2014. Case report forms were collected prospectively. A total of 269 children were recruited and followed up for a median of 7.2 years. Median age at onset was 9y (IQR 9.5–14.5, 126 females). At last follow-up, 46 (18 %) had MS, 4 AQP4-Ab NMOSD and 206 (80 %) had other ADS, of which 27 (13 %) relapsed. Relapsing MOGAD was the diagnosis in 12/27, 6 were seronegative and 9 did not have antibodies tested. Frequency of relapse differed according to first presentation in non-MS ADS, being least likely in transverse myelitis (p = 0.025). In the non-MS group, MOG-Ab was predictive of relapse (HR = 8.42; p < 0.001) occurring 8 times as often decreasing over time. Long-term difficulties did not differ between children with monophasic vs relapsing diseases. The risk of relapse in non-MS ADS depends on initial diagnosis, and MOG-Ab positivity. Long-term difficulties are observed regardless of relapses and are determined by presenting phenotype. [ABSTRACT FROM AUTHOR]

Abstract Background Experimental studies have shown that repetitive trans-spinal magnetic stimulation (TsMS) decreases demyelination and enables recovery after spinal cord injury (SCI). However, the usefulness of TsMS in humans with SCI remains unclear. Therefore, the main objective of this study is to evaluate the effects of TsMS combined with kinesiotherapy on SCI symptoms. We describe a protocol treatment with TsMS and kinesiotherapy in a patient with SCI due to neuromyelitis optica (NMO)-associated transverse myelitis. Case presentation A 23-year-old white male with NMO spectrum disorders started symptoms in 2014 and included lumbar pain evolving into a mild loss of strength and sensitivity in both lower limbs. Five months later, the symptoms improved spontaneously, and there were no sensorimotor deficits. Two years later, in 2016, the symptoms recurred with a total loss of strength and sensitivity in both lower limbs. Initially, physiotherapy was provided in 15 sessions with goals of motor-sensory recovery and improving balance and functional mobility. Subsequently, TsMS (10 Hz, 600 pulses, 20-seconds inter-trains interval, at 90% of resting motor threshold of the paravertebral muscle) was applied at the 10th thoracic vertebral spinous process before physiotherapy in 12 sessions. Outcomes were assessed at three time points: prior to physiotherapy alone (T-1), before the first session of TsMS combined with kinesiotherapy (T0), and after 12 sessions of TsMS combined with kinesiotherapy (T1). The patient showed a 25% improvement in walking independence, a 125% improvement in balance, and an 18.8% improvement in functional mobility. The Patient Global Impression of Change Scale assessed the patient’s global impression of change as ‘much improved’. Conclusion TsMS combined with kinesiotherapy may safely and effectively improve balance, walking independence, and functional mobility of patients with SCI due to NMO-associated transverse myelitis.

Acute disseminated encephalomyelitis (ADEM), or postinfectious encephalomyelitis, is a demyelinating central nervous system disease that typically presents with multifocal neurologic symptoms and encephalopathy. Numerous pathogens have been associated with ADEM, and the implicated viruses include coronavirus, coxsackie, cytomegalovirus, Epstein-Barr, herpes simplex, hepatitis A, HIV, influenza, measles, rubella, varicella zoster, and adenovirus. Although severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection has been associated with ADEM, the incidence is quite low. We present the case of a 9-year-old boy with ADEM plus longitudinal extensive myelitis who had a SARS-CoV-2 infection history and acute adenovirus infection. We evaluated the diagnosis and treatment challenges. Although our patient had severe neurological respiratory failure requiring intubation and tetraplegic flaccid paralysis, he had a total recovery before hospital discharge.

Acute transverse myelitis (ATM) is an inflammatory disorder caused by many etiologies, from postinfectious to autoimmune. Rarely, ATM cases have been reported after both COVID-19 infection and COVID-19 vaccination. We described our experience with ATM after COVID-19 infection and conducted a literature review. Case finding Methods We reported a case of longitudinally extensive ATM after COVID 19 infection, who also received convalescent plasma therapy, and present a comprehensive literature review of ATM cases reported after COVID-19 infection and COVID-19 vaccination. The literature search was done using PubMed and Google scholar with keywords and selected peer-reviewed articles. The search included all cases from Jan 2020 to Sept 2022. Results A total of 60 ATM cases reported association with post COVID 19 infection, and 23 ATM cases reported association with post COVID 19 vaccinations. Among post COVID 19 ATM cases, the mean age was 49 years and the youngest reported was 7-month-old. A total of 55% (33) were longitudinally extensive ATM. The most common symptom was lower extremity weakness. One case was reported as necrotizing myelitis on biopsy, and another case overlapped with syndrome of GBS and longitudinal ATM. No cases reported using convalescent plasma therapy after infection. Almost all the ATM cases were treated with steroids, but some cases needed additional treatment since not all responded adequately. Six cases (10%) responded with steroids plus plasmapheresis, and 5 cases (8%) responded with steroids + IVIG, especially in the pediatric age group. One case reported a positive response after treatment with eculizumab, and another with infliximab. Two cases (3%) remained paraparetic. Among post covid-19 vaccine ATM cases, 4 cases (17%) were reported as longitudinally extensive ATM. Five cases (21%) had symptom onset within a week after vaccination. Almost all reported a response to steroids except for one case which reported fatality after the 58th day after vaccination. Conclusion ATM, in the setting of acute COVID-19 infection, has been described in multiple cases and is a rare complication of COVID-19 vaccination.

We report the case of a 55-year-old man with multi-symptomatic transverse myelitis after vaccination against coronavirus disease 2019 (COVID-19). The patient was diagnosed based on the course of the disease and the results of imaging and laboratory tests. We excluded other most probable causes of the disease. The quick start of diagnosis allowed for early treatment with intravenous steroids and then plasmapheresis and the implementation of modern rehabilitation methods using biofeedback platforms, among others, and an exoskeleton. The patient returned to work, but the rehabilitation process continues to this day due to persistent symptoms that impair the patient’s quality of life.

In order to discuss the clinical and MRI features, diagnosis, and prevention of myelin oligodendrocyte glycoprotein antibody‐associated disease (MOGAD), we reported an adult case of MOG antibody‐related disease misdiagnosed as cerebral infarction. This patient's first clinical symptom was limb weakness, that different from previous reports of MOG antibody‐related diseases, such as brainstem encephalitis, neuromyelitis optical, and transverse myelitis. The main treatment plan is high‐dose corticosteroid therapy combined with immunoglobulin therapy. This case indicated that some MOGAD patients lack of specificity in the clinical manifestations and imaging perhaps would be misdiagnosed as cerebral infarction, encephalitis, immune peripheral neuropathy, MS, NMOSD, and other diseases. For patients with atypical clinical manifestations or imaging, it is especially important to take antibody detection as early as possible to make correct diagnosis and active treatment in time to avoid disability. [ABSTRACT FROM AUTHOR]

Background: Experimental studies have shown that repetitive trans-spinal magnetic stimulation (TsMS) decreases demyelination and enables recovery after spinal cord injury (SCI). However, the usefulness of TsMS in humans with SCI remains unclear. Therefore, the main objective of this study is to evaluate the effects of TsMS combined with kinesiotherapy on SCI symptoms. We describe a protocol treatment with TsMS and kinesiotherapy in a patient with SCI due to neuromyelitis optica (NMO)-associated transverse myelitis. Case presentation: A 23-year-old white male with NMO spectrum disorders started symptoms in 2014 and included lumbar pain evolving into a mild loss of strength and sensitivity in both lower limbs. Five months later, the symptoms improved spontaneously, and there were no sensorimotor deficits. Two years later, in 2016, the symptoms recurred with a total loss of strength and sensitivity in both lower limbs. Initially, physiotherapy was provided in 15 sessions with goals of motor-sensory recovery and improving balance and functional mobility. Subsequently, TsMS (10 Hz, 600 pulses, 20-seconds inter-trains interval, at 90% of resting motor threshold of the paravertebral muscle) was applied at the 10th thoracic vertebral spinous process before physiotherapy in 12 sessions. Outcomes were assessed at three time points: prior to physiotherapy alone (T-1), before the first session of TsMS combined with kinesiotherapy (T0), and after 12 sessions of TsMS combined with kinesiotherapy (T1). The patient showed a 25% improvement in walking independence, a 125% improvement in balance, and an 18.8% improvement in functional mobility. The Patient Global Impression of Change Scale assessed the patient's global impression of change as 'much improved'. Conclusion: TsMS combined with kinesiotherapy may safely and effectively improve balance, walking independence, and functional mobility of patients with SCI due to NMO-associated transverse myelitis. [ABSTRACT FROM AUTHOR]

This document is a correction notice for an article titled "Patterns and utility of myelin oligodendrocyte glycoprotein (MOG) antibody testing in cerebrospinal fluid" published in the Journal of Neurology. The correction addresses errors in the original article, including misinformed information about CSF MOG antibody testing and a small error in reporting CSF MOG values. The corrections do not change the meaning, data, or interpretation of the results. The given text appears to be a table showing different neurological conditions and their corresponding number of cases. The conditions listed include primary psychiatric, movement disorder, peripheral nerve, neurosarcoidosis, CNS vasculitis, brain cancer/tumor, vision other, inflammatory brain disorder, idiopathic intracranial hypertension, seizure disorder, systemic lupus Erythematosus, PRES, normal, and other/unknown. The table also includes information about CSF MOG staining and immunotherapy timing for certain patients. The article discusses the use of various immunotherapies for the treatment of MOG antibody-associated disorders (MOGAD). It highlights the challenges in interpreting weakly positive titers as a reliable diagnostic tool for MOGAD. The study found that in their current research, 4 out of 5 cerebrospinal fluid (CSF) MOG staining results were weak, suggesting the need for further investigation into the diagnostic criteria for MOGAD. The authors provide valuable insights into the limitations and [Extracted from the article]

Background: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder characterized by inflammatory assaults on the central nervous system (CNS), particularly on the optic nerves and spinal cord. In recent years, a wider range of clinical manifestations of this complex disease have been observed, emphasizing the importance of gaining a more profound understanding beyond optic neuritis (ON) and transverse myelitis (TM). Current knowledge: This study explores the many clinical symptoms of NMOSD, including common and uncommon presentations. Distinctive aspects of ON, TM, and diencephalic/brainstem syndromes are examined, highlighting their unique characteristics in contrast to conditions such as multiple sclerosis. We also discuss extra‐CNS involvement, such as unusual signs, including muscle involvement, retinal injury, auditory impairment, and rhinological symptoms. Aims and objectives: Our study intends to highlight the wide range and complexity of NMOSD presentations, emphasizing the significance of identifying unusual symptoms for precise diagnosis and prompt management. The specific processes that contribute to the varied clinical presentation of NMOSD are not well understood despite existing information. This emphasizes the necessity for more study to clarify the mechanisms that cause different symptoms and discover new treatment targets for this complex autoimmune disorder. Conclusion: It is essential to acknowledge the complex and varied clinical manifestations of NMOSD to enhance diagnosis, treatment, and patient results. By enhancing our comprehension of the fundamental processes and investigating innovative therapeutic approaches, we may aim to enhance the quality of life for persons impacted by this illness. [ABSTRACT FROM AUTHOR]

Purpose of Review: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a distinct neuroinflammatory condition characterized by attacks of optic neuritis, transverse myelitis, and other demyelinating events. Though it can mimic multiple sclerosis and neuromyelitis optica spectrum disorder, distinct clinical and radiologic features which can discriminate these conditions are now recognized. This review highlights recent advances in our understanding of clinical manifestations, diagnosis, and treatment of MOGAD. Recent Findings: Studies have identified subtleties of common clinical attacks and identified more rare phenotypes, including cerebral cortical encephalitis, which have broadened our understanding of the clinicoradiologic spectrum of MOGAD and culminated in the recent publication of proposed diagnostic criteria with a familiar construction to those diagnosing other neuroinflammatory conditions. These criteria, in combination with advances in antibody testing, should simultaneously lead to wider recognition and reduced incidence of misdiagnosis. In addition, recent observational studies have raised new questions about when to treat MOGAD chronically, and with which agent. Summary: MOGAD pathophysiology informs some of the relatively unique clinical and radiologic features which have come to define this condition, and similarly has implications for diagnosis and management. Further prospective studies and the first clinical trials of therapeutic options will answer several remaining questions about the peculiarities of this condition. [ABSTRACT FROM AUTHOR]

Myelin oligodendrocyte glycoprotein antibody‐associated disease (MOGAD) is diagnosed by serum MOG‐immunoglobulin G (MOG‐IgG) in association with typical demyelination. 111/1127 patients with paired CSF/serum samples were seropositive for MOG‐IgG. Only 7/1016 (0.7%) seronegative patients had CSF‐restricted MOG‐IgG. While 3/7 patients had longitudinally extensive transverse myelitis, four had a confirmed alternate diagnosis (three multiple sclerosis, one CNS vasculitis). In a national referral setting, CSF‐restricted MOG‐IgG had a low sensitivity (2.63%, 95%CI 0.55–7.50%) and low positive predictive value (1.97%, 95%CI 0.45–8.13%). We strongly recommend serum as the preferred diagnostic biospecimen, and urge caution in the interpretation of CSF‐restricted MOG‐IgG in patients without clinico‐radiological features consistent with MOGAD. [ABSTRACT FROM AUTHOR]

The treatment of primary Sjögren’s syndrome (pSS) coexisting with neuromyelitis optica spectrum disorder (NMOSD) using protein-A immunoadsorption combined with immunosuppressive therapy has rarely been reported. Herein, we present the case of a 35-year-old female diagnosed with pSS concomitant with NMOSD (pSS-NMOSD) who demonstrated a positive response to protein-A immunoadsorption after failing to respond to therapy comprising high-dose intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG). Within one week of receiving three sessions of immunoadsorption combined with immunosuppressive treatment, the patient’s clinical symptoms (blurred vision, paraparesis, and dysfunctional proprioception) significantly improved. Additionally, a rapid decrease in the circulating levels of Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), immunoglobulin (Ig) A, IgG, IgM, erythrocyte sedimentation rate (ESR), and rheumatoid factor (RF) were observed. Magnetic resonance imaging (MRI) further revealed a significant reduction in the lesions associated with longitudinal extensive transverse myelitis. During the follow-up period, prednisolone was gradually tapered to a maintenance dose of 5-10 mg/day, whereas mycophenolate mofetil (MMF) was maintained at 1.0-1.5 g/day. The patient’s condition has remained stable for four years, with no signs of recurrence or progression observed on imaging examination. Therefore, this case suggests that protein A immunoadsorption may represent a potentially effective therapeutic option for patients with pSSNMOSD who are refractory to conventional treatments. [ABSTRACT FROM AUTHOR]

Introduction. Longitudinal extensive transverse myelitis (LETM) has four main causes: inflammatory, malnutrition, vascular, and infectious causes. Among the commonly described viral causes leading to LETM are the Herpesviridae family, HIV, and HTLV‐1. Case Presentation. A 43‐year‐old man presented with asymmetric weakness of the lower limbs (the left side was weaker), urinary retention, and flank pain. The symptoms began five days after shingle eruption and progressed over twelve days. He was diagnosed with longitudinal extensive transvers myelitis extending from T4 to T6, which corresponded to the same dermatome involved in shingles. The PCR result of cerebrospinal fluid was positive for varicella‐zoster virus with a viral load of 500 copies/ml. Additionally, the initial HIV enzyme‐linked immunosorbent assay (ELISA) test was positive, and his CD4 count was 72 cells/mm3. Other lab results were normal. Based on the appearance of LETM in the thoracic MRI at T4‐T6, VZV myelitis was diagnosed, and treatment was initiated with acyclovir (30 mg/kg divided daily for twenty‐one days), methylprednisolone (1 g/day for three days), prophylactic antibiotics (trimethoprim/sulfamethoxazole, rifampin, and isoniazid), and antiretroviral therapy (dolutegravir and Truvada). After 2‐month follow‐up, he was nearly free of symptoms. Conclusion. Infection is one of the critical causes of transverse myelitis. When a patient presents with skin shingles along with myelopathy, varicella‐zoster myelitis should be considered, and the patient should be evaluated in terms of immune system dysfunction. Treatment with acyclovir has been shown to be effective in reducing clinical symptoms in such cases. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the clinical spectrum and immediate outcomes of acute flaccid paralysis (AFP) in children presenting at National Institute of Child Health, Karachi, Pakistan. Study Design: Cross-sectional study. Setting: Department of Pediatrics, NICH, Karachi, Pakistan. Period: August 2023 to January 2024. Methods: A total of 121 children of either gender, aged 1-15 years, and presenting with AFP were analyzed. AFP was diagnosed by nerve conduction study. Causes of AFP, like Guillian Barre Syndrome (GBS), transverse myelitis, traumatic neuritis were noted. Outcomes were noted in terms of discharged from hospital, left against medical advice, and mortality. Results: In a total of 121 children, 75 (62.0%) were boys. The mean age was 6.00±2.88 years, ranging between 1-12 years. There were 74 (61.2%) children who were fully vaccinated as per age. The most common cause of AFP were GBS, transverse myelitis, and hypokalemic paralysis, noted in 49 (40.5%), 19 (15.7%), and 15 (12.4%) children, respectively. Ninety (74.4%) children were discharged after the treatment, 10 (8.3%) left against medical advice, whereas mortality was noted in 21 (17.4%) children. Children leaving against medical advice were left out from the analysis to compared final outcomes with respect to various study variables. Incomplete vaccination status (p=0.0006), and presentation with sensory loss (p=0.0003) were found to have significant association with mortality. Conclusion: Guillian Barre Syndrome was found to be the most common cause behind acute flaccid paralysis in children. Incomplete vaccination history, and presenting with sensory loss were associated with poor outcomes. [ABSTRACT FROM AUTHOR]

Background: Idiopathic acute transverse myelitis (IATM) is a focal inflammatory disorder of the spinal cord that results in motor, sensory, and autonomic dysfunction. However, the comparative analysis of MRI-negative and MRI-positive in IATM patients were rarely reported. Objectives: The purpose of this study was to compare MRI-negative with MRI-positive groups in IATM patients, analyze the predictors for a poor prognosis, thus explore the relationship between MRI-negative and prognosis. Methods: We selected 132 patients with first-attack IATM at the First Affiliated Hospital of Nanchang University from May 2018 to May 2022. Patients were divided into MRI-positive and MRI-negative group according to whether there were responsible spinal MRI lesions, and good prognosis and poor prognosis based on whether the EDSS score ≥ 4 at follow-up. The predictive factors of poor prognosis in IATM patients was analyzed by logistic regression models. Results: Of the 132 patients, 107 first-attack patients who fulfilled the criteria for IATM were included in the study. We showed that 43 (40%) patients had a negative spinal cord MRI, while 27 (25%) patients were identified as having a poor prognosis (EDSS score at follow-up ≥ 4). Compared with MRI-negative patients, the MRI-positive group was more likely to have back/neck pain, spinal cord shock and poor prognosis, and the EDSS score at follow-up was higher. We also identified three risk factors for a poor outcome: absence of second-line therapies, high EDSS score at nadir and a positive MRI result. Conclusions: Compared with MRI-negative group, MRI-positive patients were more likely to have back/neck pain, spinal cord shock and poor prognosis, with a higher EDSS score at follow-up. The absence of second-line therapies, high EDSS score at nadir, and a positive MRI were risk factors for poor outcomes in patients with first-attack IATM. MRI-negative patients may have better prognosis, an active second-line immunotherapy for IATM patients may improve clinical outcome. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Sarcoidosis‐induced LETM represents a rare but life‐threatening neurological manifestation of sarcoidosis, characterized by spinal cord inflammation, and associated neurological deficits. Sarcoidosis should be included in the differential diagnosis of LETM, particularly in patients with no lung involvement. Prompt recognition and management are obligatory to optimize outcomes and prevent long‐term disability. Sarcoidosis is a multisystem inflammatory granulomatous disorder characterized by the formation of noncaseating granulomas. Although sarcoidosis commonly affects the skin, lymph nodes, and lungs, neurological involvement of sarcoidosis has also been reported. Longitudinally extensive transverse myelitis (LETM) is a rare but well‐documented serious manifestation of neuroscoidosis. We report a case of LETM caused by sarcoidosis in a 53‐year‐old male who presented with progressive bilateral lower extremity weakness, urinary retention, and paresthesia. Laboratory evaluations revealed elevated inflammatory markers. Magnetic resonance imaging of the spine showed hyperintense signals consistent with transverse myelitis. Cerebrospinal fluid analysis revealed lymphocytic pleocytosis and elevated protein levels. Chest computed tomography showed hilar lymphadenopathy. A biopsy of the intrathoracic lymph node showed noncaseating granulomas consistent with sarcoidosis. A diagnosis of sarcoidosis‐induced LETM was made after ruling out all other possible etiologies. His condition improved gradually after starting high‐dose prednisone, mycophenolate, and rehabilitation strategies. Our case underscores the importance of prompt diagnosis and management of sarcoidosis‐induced LETM and highlights that sarcoidosis must be included among differential diagnoses of LETM, especially in cases with no lung involvement. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica is an autoimmune disease with nonspecific onset, with symptoms that may be similar and confused with multiple sclerosis. Devic's disease (neuromyelitis optica) mainly manifests as optic neuritis and concomitant myelitis. The patient's first manifestation of Devic's disease was reported in April 2020. Two months later the patient was admitted to the neurology ward where serological analysis, MRI and other diagnostics were performed, leading to an initial diagnosis of transverse myelitis. After another two months, significant deterioration of vision in the right eye occurred. The presence of AQP4-Ab and differential diagnostics performed established the diagnosis of neuromyelitis optica. The patient was treated with rituximab and plasmapheresis. Despite effective immunosuppressive treatment quadriplegia persists, and the patient moves using a wheelchair. This report presents features of rare astrocytopathy, which is underdiagnosed due to its similarity to multiple sclerosis. This work aims to remind us which symptoms suggest the proper diagnosis of neuromyelitis optica. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica spectrum disorder (NMOSD) is a clinical syndrome characterized by attacks of acute optic neuritis and transverse myelitis. We report a casewith paraneoplastic NMOSD that improved after immunosuppressive therapy, surgical resection, and chemotherapy. A 48-year-old woman initially presented with gradual binocular visual loss over the course of one week. The patient was evaluated usingmagnetic resonance imaging (MRI), computed tomography (CT), visual evoked potential (VEP), pathological biopsy, immunohistochemistry, and autoimmune antibody testing. The brain MRI findings were normal. The VEP revealed prolonged P100 latencies in the right eye and an absence of significant waves in the left eye. Positive serum AQP4-IgG antibodies were found. The patient was diagnosed as NMOSD. Then the patient responded well to treatment with methylprednisolone. An ovarian tumor was found in the patient using abdominal MRI and CT. The tumor was surgically resected, and a pathological biopsy revealed that it was ovarian dysgerminoma. The patient received four rounds of chemotherapy after surgery. Onemonth after the final chemotherapy treatment, a positron emission tomography (PET) scan revealed no tumor. The vision of the patient gradually recovered and serum AQP4 was negative. Furthermore, we summarized the characteristics of patients diagnosed with paraneoplastic NMOSD associated with ovarian neoplasms in previous studies. This is a characteristic case of overlapping NMOSD and ovarian dysgerminoma, demonstrating the importance of tumor therapy in cases of paraneoplastic NMOSD. [ABSTRACT FROM AUTHOR]

Background: Pediatric acute transverse myelitis (ATM) accounts for 20–30% of children presenting with a first acquired demyelinating syndrome (ADS) and may be the first clinical presentation of a relapsing ADS such as multiple sclerosis (MS). B cells have been strongly implicated in the pathogenesis of adult MS. However, little is known about B cells in pediatric MS, and even less so in pediatric ATM. Our lab previously showed that plasmablasts (PB), the earliest B cell subtype producing antibody, are expanded in adult ATM, and that these PBs produce self-reactive antibodies that target neurons. The goal of this study was to examine PB frequency and phenotype, immunoglobulin selection, and B cell receptor reactivity in pediatric patients presenting with ATM to gain insight to B cell involvement in disease. Methods: We compared the PB frequency and phenotype of 5 pediatric ATM patients and 10 pediatric healthy controls (HC) and compared them to previously reported adult ATM patients using cytometric data. We purified bulk IgG from the plasma samples and cloned 20 recombinant human antibodies (rhAbs) from individual PBs isolated from the blood. Plasma-derived IgG and rhAb autoreactivity was measured by mean fluorescence intensity (MFI) in neurons and astrocytes of murine brain or spinal cord and primary human astrocytes. We determined the potential impact of these rhAbs on astrocyte health by measuring stress and apoptotic response. Results: We found that pediatric ATM patients had a reduced frequency of peripheral blood PB. Serum IgG autoreactivity to neurons in EAE spinal cord was similar in the pediatric ATM patients and HC. However, serum IgG autoreactivity to astrocytes in EAE spinal cord was reduced in pediatric ATM patients compared to pediatric HC. Astrocyte-binding strength of rhAbs cloned from PBs was dependent on somatic hypermutation accumulation in the pediatric ATM cohort, but not HC. A similar observation in predilection for astrocyte binding over neuron binding of individual antibodies cloned from PBs was made in EAE brain tissue. Finally, exposure of human primary astrocytes to these astrocyte-binding antibodies increased astrocytic stress but did not lead to apoptosis. Conclusions: Discordance in humoral immune responses to astrocytes may distinguish pediatric ATM from HC. [ABSTRACT FROM AUTHOR]

Aquaporin-4 antibodies (AQP4-Abs) are a diagnostic marker for patients with a demyelinating disease called neuromyelitis optica spectrum disorder (NMOSD). Anti-Argonaute antibodies (AGO-Abs) present as potential biomarkers of the overlap syndrome between NMOSD and other autoimmune diseases. In this paper, we present the case of an adult woman with numbness, tingling, and burning sensations in her arms and subsequent bilateral internuclear ophthalmoplegia. Brain–cervical–thoracic magnetic resonance imaging (MRI) showed T2 hyperintensities in the dorsal brainstem and around the midbrain aqueduct and longitudinally transverse myelitis with homogeneous enhancement on gadolinium-enhanced MRI. The contemporaneous detection of AQP4- and AGO-Abs led to a definite diagnosis of overlap syndrome of NMOSD with AGO-Abs. The patient was treated with immunosuppressive agents, including corticosteroids and immunoglobulins, and achieved remission. This case highlights a novel phenotype of NMOSD with AGO-Abs overlap syndrome, which presents with relapsing brainstem syndrome and longitudinally extensive myelitis with acute severe neurological involvement. The promising prognosis of the disease could serve as a distinct clinical profile. Broad screening for antibodies against central nervous system autoimmune antigens is recommended in suspected patients with limited or atypical clinical manifestations. [ABSTRACT FROM AUTHOR]

Background: Longitudinally extensive transverse myelitis (LETM) is characterized by spinal cord lesions that affect at least three spinal cord segments. It can be associated with various inflammatory conditions. While imaging characteristics can aid in diagnosis, relying solely on them may lead to misinterpretation. Case presentation: We describe a 35-year-old woman who presented with subacute myelitis. Her cervical MRI (magnetic resonance imaging) revealed an extensive lesion from the area postrema down to the second thoracic level, with a trident sign observed in axial T1-weighted post-gadolinium imaging. The presence of a trident sign in MRI of patients with myelopathy is more commonly associated with sarcoidosis than other conditions. But our patient had positive (rechecked) AQP4 antibody and negative FDG-PET (fluorodeoxyglucose positron emission tomography) scan that shows trident sign could be seen in other inflammatory disorders such as NMO (neuromyelitis optica). Conclusion: Trident sign is not pathognomonic for sarcoidosis, additional investigations are necessary to identify the diagnoses related to the trident sign. [ABSTRACT FROM AUTHOR]

We report the case of a 55-year-old man with multi-symptomatic transverse myelitis after vaccination against coronavirus disease 2019 (COVID-19). The patient was diagnosed based on the course of the disease and the results of imaging and laboratory tests. We excluded other most probable causes of the disease. The quick start of diagnosis allowed for early treatment with intravenous steroids and then plasmapheresis and the implementation of modern rehabilitation methods using biofeedback platforms, among others, and an exoskeleton. The patient returned to work, but the rehabilitation process continues to this day due to persistent symptoms that impair the patient's quality of life. [ABSTRACT FROM AUTHOR]

Clinical syndromes associated with antibodies against myelin oligodendrocyte glycoprotein (MOG) are now recognized as a distinct neurological disease entity, and are gaining increasing attention. The pathogenic mechanisms underlying MOG-antibody disease (MOGAD) remain incompletely understood. Case series, facilitated by registries, and observational studies over the past few years have shed increasing light on the clinical aspects and therapeutic approaches of MOGAD. MOGAD may manifest with a variety of clinical syndromes, including acute disseminated encephalomyelitis (ADEM), autoimmune encephalitis, optic neuritis (ON) and transverse myelitis (TM). MOGAD can be either monophasic or relapsing. This review aims to provide a comprehensive updated description of the clinical spectrum, paraclinical features, and prognosis of MOG-antibody disease, as well as summarize its therapeutic considerations. Randomized clinical trials, standardized diagnostic criteria and treatment guidelines are the steps forward. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID‐19) caused by the severe acute respiratory syndrome coronavirus 2n first appeared in Wuhan, China in 2019. Soon after, it was declared a pandemic by the World Health Organization. The health crisis imposed by a new virus and its rapid spread worldwide prompted the fast development of vaccines. For the first time in human history, two vaccines based on recombinant genetic material technology were approved for human use. These mRNA vaccines were applied in massive immunization programs around the world, followed by other vaccines based on more traditional approaches. Even though all vaccines were tested in clinical trials prior to their general administration, serious adverse events, usually of very low incidence, were mostly identified after application of millions of doses. Establishing a direct correlation (the cause‐effect paradigm) between vaccination and the appearance of adverse effects has proven challenging. This review focuses on the main adverse effects observed after vaccination, including anaphylaxis, myocarditis, vaccine‐induced thrombotic thrombocytopenia, Guillain–Barré syndrome, and transverse myelitis reported in the context of COVID‐19 vaccination. We highlight the symptoms, laboratory tests required for an adequate diagnosis, and briefly outline the recommended treatments for these adverse effects. The aim of this work is to increase awareness among healthcare personnel about the serious adverse events that may arise post‐vaccination. Regardless of the ongoing discussion about the safety of COVID‐19 vaccination, these adverse effects must be identified promptly and treated effectively to reduce the risk of complications. [ABSTRACT FROM AUTHOR]

Key Clinical Message 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. Abstract 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.

Spinal cord inflammation is a rare presentation of schistosomiasis infection. The present report describes the case of a young patient presenting subacute medullary symptoms revealing extensive longitudinal myelitis related to schistosomiasis, also known as bilharzia. The diagnosis was based on detection of parasite eggs in stool. The patient was treated with Praziquantel, corticosteroids and plasma exchanges, leading to a favorable clinical course. [ABSTRACT FROM AUTHOR]

This document is an editorial published in the journal Frontiers in Neurology. It discusses the advances and controversies in the pathophysiology, diagnosis, and treatment of myelopathies and spinal cord injuries. The editorial highlights the high burden of spinal cord injuries on population health and the lack of universally effective treatment. It also explores various topics related to spinal cord injuries, including transverse myelitis, neuroprotective agents, regenerative neurology, degenerative cervical myelopathy, and central cord syndrome. The articles in this research topic provide a wide perspective on spinal cord injuries and contribute to our understanding of their pathophysiology and clinical management. [Extracted from the article]

Purpose: We aimed to present the clinical, imaging and laboratory findings of patients which diagnosed Neuromyelitis Optica Spectrum Disease (NMOSD) and accompanied by antibody positivity. Materials ve Metods: This retrospective observational study included 15 patients diagnosed with NMOSH. Results: Eleven of our patients were female and four were male. The mean age at onset was 51.27±12.26 years. The first clinical attack was simultaneous optic neuritis (ON) and myelitis in 3 patients, myelitis in 6 patients and ON in 5 patients. Ten patients had positive aquaporin-4 (AQP-4) IgG antibody and 5 patients had positive myelin oligodentrocyte glycoprotein (MOG) IgG antibody. Six patients had elevated cerebrospinal fluid (CSF) protein. CSF oligoclonal band of eight patients was Type 1 negative, 1 patient was Type 2 positive, and 1 patient was Type 3 positive. Eight patients had normal cranial magnetic resonance imaging, while 1 patient had lesions similar to multiple sclerosis (MS). Two patients had a previous diagnosis of Sjögren's Syndrome (SS) and 3 patients were diagnosed with SS in our clinic. Intravenous methylprednisolone (1000 mg/day) and thoropathic plasma exchange were given as treatment for the attack, and oral steroids, azathioprine, rituximab and cyclophosphamide were given as prophylactic treatment. Conclusion: NMOSH has a more severe prognosis than MS. It is most frequently associated with SS among autoimmune diseases. It has been observed that patients with MOG IgG antibody positivity have better response to attack and prophylactic treatment than patients with AQP-4 IgG antibody positivity.

BACKGROUND: Gadolinium enhancement of spinal nerve roots on magnetic resonance imaging (MRI) has rarely been reported in spinal dural arteriovenous fistula (SDAVF). Nerve root enhancement and cerebrospinal fluid (CSF) pleocytosis can be deceptive and lead to a misdiagnosis of myeloradiculitis. We report a patient who was initially diagnosed with neurosarcoid myeloradiculitis due to spinal nerve root enhancement, mildly inflammatory cerebrospinal fluid, and pulmonary granulomas, who ultimately was found to have an extensive symptomatic SDAVF. CASE PRESENTATION: A 52-year-old woman presented with a longitudinally extensive spinal cord lesion with associated gadolinium enhancement of the cord and cauda equina nerve roots, and mild lymphocytic pleocytosis. Pulmonary lymph node biopsy revealed non-caseating granulomas and neurosarcoid myeloradiculitis was suspected. She had rapid and profound clinical deterioration after a single dose of steroids. Further work-up with spinal angiography revealed a thoracic SDAVF, which was surgically ligated leading to clinical improvement. CONCLUSIONS: This case highlights an unexpected presentation of SDAVF with nerve root enhancement and concurrent pulmonary non-caseating granulomas, leading to an initial misdiagnosis with neurosarcoidosis. Nerve root enhancement has only rarely been described in cases of SDAVF; however, as this case highlights, it is an important consideration in the differential diagnosis of non-inflammatory causes of longitudinally extensive myeloradiculopathy with nerve root enhancement. This point is highly salient due to the importance of avoiding misdiagnosis of SDAVF, as interventions such as steroids or epidural injections used to treat inflammatory or infiltrative mimics may worsen symptoms in SDAVF. We review the presentation, diagnosis, and management of SDAVF as well as a proposed diagnostic approach to differentiating SDAVF from inflammatory myeloradiculitis.

• Study assessed four adverse events following 2022–2023 seasonal influenza vaccines. • Study population was 12.7 million Medicare enrollees 65 years and older. • There was no increase in post-vaccination incidence rates for three adverse events. • Anaphylaxis rates were elevated and possibly modified by concomitant vaccination. While safety of influenza vaccines is well-established, some studies have suggested potential associations between influenza vaccines and certain adverse events (AEs). This study examined the safety of the 2022–2023 influenza vaccines among U.S. adults ≥ 65 years. A self-controlled case series compared incidence rates of anaphylaxis, encephalitis/encephalomyelitis, Guillain-Barré Syndrome (GBS), and transverse myelitis following 2022–2023 seasonal influenza vaccinations (i.e., any, high-dose or adjuvanted) in risk and control intervals among Medicare beneficiaries ≥ 65 years. We used conditional Poisson regression to estimate incidence rate ratios (IRRs) and 95 % confidence intervals (CIs) adjusted for event-dependent observation time and seasonality. Analyses also accounted for uncertainty from outcome misclassification where feasible. For AEs with any statistically significant associations, we stratified results by concomitant vaccination status. Among 12.7 million vaccine recipients, we observed 76 anaphylaxis, 276 encephalitis/encephalomyelitis, 134 GBS and 75 transverse myelitis cases. Only rates of anaphylaxis were elevated in risk compared to control intervals. With all adjustments, an elevated, but non-statistically significant, anaphylaxis rate was observed following any (IRR: 2.40, 95% CI: 0.96–6.03), high-dose (IRR: 2.31, 95% CI: 0.67–7.91), and adjuvanted (IRR: 3.28, 95% CI: 0.71–15.08) influenza vaccination; anaphylaxis IRRs were 2.54 (95% CI: 0.49–13.05) and 1.64 (95% CI: 0.38–7.05) for persons with and without concomitant vaccination, respectively. Rates of encephalitis/encephalomyelitis, GBS, or transverse myelitis were not elevated following 2022–2023 seasonal influenza vaccinations among U.S. adults ≥ 65 years. There was an increased rate of anaphylaxis following influenza vaccination that may have been influenced by concomitant vaccination. [ABSTRACT FROM AUTHOR]

The Neuromyelitis Optica Spectrum Disorders (NMOSD) constitute a spectrum of rare autoimmune diseases of the central nervous system characterized by episodes of transverse myelitis, optic neuritis, and other demyelinating attacks. Previously thought to be a subtype of multiple sclerosis, NMOSD is now known to be a distinct disease with unique pathophysiology, clinical course, and treatment options. Although there have been significant recent advances in the diagnosis and treatment of NMOSD, the field still lacks clinically validated biomarkers that can be used to stratify disease severity, monitor disease activity, and inform treatment decisions. Here we review many emerging NMOSD biomarkers including markers of cellular damage, neutrophil-to-lymphocyte ratio, complement, and cytokines, with a focus on how each biomarker can potentially be used for initial diagnosis, relapse surveillance, disability prediction, and treatment monitoring. [ABSTRACT FROM AUTHOR]

This article discusses the occurrence of myelitis, a neurological condition, related to SARS-CoV-2 infection. The authors present two cases of SARS-CoV-2-related myelitis that occurred in Italy and review other reported cases in the literature. The article highlights the variability in symptoms and lesion locations among these cases. The exact mechanism by which the virus causes myelitis is still unclear, but it may involve direct damage to the spinal cord or an autoimmune response triggered by viral antigens. The article concludes that myelitis is a rare manifestation of SARS-CoV-2 infection and further research is needed to understand its underlying mechanisms. [Extracted from the article]

Background: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an idiopathic central nervous system (CNS) demyelinating disease gaining recognition with wider availability of cell-based assay (CBA) testing and recently published diagnostic criteria. However, uncertainty remains regarding the interpretation of antibody titers, particularly cerebrospinal fluid (CSF) MOG antibody titers. Methods: All MOG IgG CBA results performed by the provincial MitogenDx laboratory in Alberta from July 2017 to July 2023 were retrieved. Chart review was performed in patients with both serum and CSF testing and ≥ 1 positive MOG antibody result. Demographics, antibody titers, clinical and imaging features, treatment, and diagnosis were analyzed based on serum/CSF status. Results: Among 4494 MOG CBA assays, there were 413 CSF samples in 402 patients, and 268 patients had at least one associated serum sample. Mean time between CSF and serum testing was 20.9 days (range 0–870 days), most with testing within 30 days. Five of the 268 patients had serum positive/CSF positive MOG antibodies, 4 with acute disseminated encephalomyelitis and 1 with longitudinally extensive transverse myelitis. Twenty-three patients had serum positive/CSF negative MOG and 13/23 with optic neuritis. CSF MOG antibody positive patients were younger, and more likely to remain MOG seropositive versus CSF negative patients. No seronegative patient had MOG antibodies in CSF. Conclusions: In province-wide testing, CSF MOG antibodies were rare, only in MOG seropositive patients and none with optic neuritis. Our study does not support a clear role for CSF MOG antibody testing in the majority of patients, although further study is required. [ABSTRACT FROM AUTHOR]

Background: Live cell-based assay (LCBA) is the gold standard for MOG-IgG detection, and fixed CBA (FCBA) is a widely used commercial alternative. Recent criteria attributed a diagnostic value to MOG-IgG titration with both LCBA and FCBA, with low-titre samples requiring additional supporting features for MOGAD diagnosis. However, FCBA titration is not validated. We aimed to assess the impact of the criteria-based MOG-IgG testing in MOGAD diagnosis. Methods: Thirty-eight serum samples of LCBA MOG-IgG1-positive MOGAD patients were titred on MOG-IgG LCBA and FCBA, and the presence of supporting features for MOGAD assessed. MOGAD criteria were evaluated in four testing scenarios: (a) FCBA without titration; (b) FCBA with titration; c) LCBA without titration; (d) LCBA with titration. Results: FCBA without titration failed to reach MOGAD diagnosis in 11/38 patients (28.9%, negative results in 5, lack of supporting features in 6). Patients with unconfirmed diagnosis had optic neuritis (ON, n = 8), or transverse myelitis (TM, n = 3). FCBA with titration allowed MOGAD diagnosis in 4 additional patients. Correlation between LCBA and FCBA titres was moderate (Spearman's rho 0.6, p < 0.001). Conclusions: FCBA yields high rate of misdiagnosis mainly due a lower analytical sensitivity. FCBA titration provides a moderate diagnostic advantage in FCBA positive patients. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a granulomatous disorder with multi‑organ involvement, and etiology still remains unknown. Neurosarcoidosis is the involvement of the nervous system in sarcoidosis. Spinal cord involvement is usually intra‑dural, but extra‑dural involvement can also occur. Here, we report a case of 30 years old lady presenting with subacute onset paraparesis with bladder and bowel involvement, which was finally diagnosed as sarcoidosis‑associated myelopathy with the longitudinally extensive transverse myelitis (LETM) phenotype. [ABSTRACT FROM AUTHOR]

Objective: Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune-mediated central nervous system disorders distinguished by the presence of serum aquaporine-4 IgG antibody (AQP4-Ab). The clinical panel comprises severe optic neuritis (ON) and transverse myelitis, which can result in incomplete recovery and a high risk of recurrence. Methods: This study aimed to evaluate the visual outcomes of three patients with severe acute ON in NMOSD that was non-responsive to intravenous methylprednisolone (IVMP), who received plasma exchange therapy (PLEX). We included three patients (P1, P2 and P3) with severe acute ON who had no improvement after IVMP treatment and were admitted to the ophthalmology department at the Emergency University Hospital Bucharest from January 2022 to September 2023. All three patients with ON were diagnosed in accordance with the criteria described by the Optic Neuritis Treatment Trial. All the subjects were experiencing their first attack. Results: The mean recruitment age was 35.3 ± 7.71. All patients were seropositive for the AQP4 antibody. All patients were tested for serum myelin oligodendrocyte glycoprotein (MOG) antibody but only one showed a positive test (P3). Lesions visible in orbital MRI indicated the involvement of retrobulbar, canalicular and/or intracranial segments. All three subjects had no response or incomplete remission after an IVMP protocol (5 days of 1000 mg intravenous methylprednisolone in sodium chloride 0.9%). The mean time from onset of optic neuritis to PLEX was 37.6 days. The PLEX treatment protocol comprised five cycles of plasma exchange treatment over 10 days, with a plasma exchange session every other day. An amount of 1 to 1.5 volumes of circulating plasma were dialyzed for 2–4 h. At 1 month after the completion of PLEX therapy, BCVA and VF parameters were improved in all three patients. Conclusion: The treatment of ON remains subject to debate and is somewhat controversial. Plasma exchange must be considered as a rescue therapy when IVMP is insufficient for AQP4-ON patients. This study revealed that PLEX treatment effectively improves the visual outcomes of patients experiencing their first attack of severe acute isolated ON after high-dose IVMP treatment. This study suggests that PLEX may be associated with improved visual outcomes in NMOSD acute optic neuritis. [ABSTRACT FROM AUTHOR]

Background: Transverse myelitis (TM) is a demyelinating inflammatory disease that presents with motor, sensory, and autonomic dysfunction, which may be acute or subacute. COVID-19-associated TM has been described in a scarce number of patients. Clinical case: A 15-year-old previously healthy male patient with respiratory disease before his neurological deterioration presented to the emergency room after developing a complete medullary syndrome located at the cervical-dorsal level, with ascending and symmetric paraparesis that rapidly progressed to paraplegia, with sensory dysfunction from the T3 level, sphincter dysfunction and sudden ventilatory deterioration that required mechanical ventilation. Magnetic resonance imaging was compatible with acute TM. Inflammatory and non-inflammatory etiologies were discarded. In addition, a positive severe acute respiratory syndrome coronavirus 2 test was obtained. Treatment included steroid pulses and plasmapheresis, with an insidious evolution. Conclusion: COVID-19 is an infrequent cause of TM and should be suspected when other etiologies have been ruled out. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease in which the majority of patients have autoantibodies against the aquaporin‐4 (AQP4) water channel. In the central nervous system (CNS), AQP4 is expressed on the cell membrane of astrocytes, and anti‐AQP4 antibodies harm AQP4 expressing astrocytes, resulting in astrocytopathy. The majority of patients are female and have acute visual disturbances and transverse myelitis, as well as brainstem and other cerebral lesions. These symptoms are frequently severe and develop quickly, resulting in substantial impairment in daily life. Patients are treated with high‐dose methylprednisolone infusions, plasma exchange, and intravenous immunoglobulin administration during the acute stage. Based on the pathogenesis of NMOSD, various effective relapse‐preventing therapies have emerged in recent years. The majority of them have been proved to have a positive impact on the patients who have been treated. They increased the number of therapy options available, albeit long‐term efficacy and safety would need to be monitored for a longer period. Through its unique fluid transport function in the CNS, knowledge of the functions of AQP4 has grown in the field of not only inflammatory disorders like NMOSD, but also other neurodegenerative disorders like Alzheimer's disease. [ABSTRACT FROM AUTHOR]

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• We have identified an association between ChAdOx1 nCoV-19 and an increased risk of Acute Disseminated Encephalomyelitis incident admissions. • Dose-specific analysis suggests that dose 1 of ChAdOx1 nCoV-19 may be associated with an increased risk of both Acute Disseminated Encephalomyelitis and Transverse Myelitis incident admissions. • These findings build upon the recently published Global Vaccine Data Network study that suggested an excess of Acute Disseminated Encephalomyelitis and Transverse Myelitis following adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines compared to historically expected background rates. • This study demonstrates the value of the Global Vaccine Data Network collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare potential associations, as well as the value of linked population level datasets such as VSHL able to rapidly explore associations that are identified. Acute Disseminated Encephalomyelitis (ADEM) and Transverse Myelitis (TM) are within the group of immune mediated disorders of acquired demyelinating syndromes. Both have been described in temporal association following various vaccinations in case reports and case series and have been evaluated in observational studies. A recent analysis conducted by The Global Vaccine Data Network (GVDN) observed an excess of ADEM and TM cases following the adenoviral vectored ChAdOx1 nCoV-19 (AZD1222) and mRNA-1273 vaccines, compared with historically expected background rates from prior to the pandemic. Further epidemiologic studies were recommended to explore these potential associations. We utilized an Australian vaccine datalink, Vaccine Safety Health-Link (VSHL), to perform a self-controlled case series analysis for this purpose. VSHL was selected for this analysis as while VSHL data are utilised for GVDN association studies, they were not included in the GVDN observed expected analyses. The VSHL dataset contains vaccination records sourced from the Australian Immunisation Register, and hospital admission records from the Victorian Admitted Episodes Dataset for 6.7 million people. These datasets were used to determine the relative incidence (RI) of G040 (ADEM) and G373 (TM) ICD-10-AM coded admissions in the 42-day risk window following COVID-19 vaccinations as compared to control periods either side of the risk window. We observed associations between ChAdOx1 adenovirus vector COVID-19 vaccination and ADEM (all dose RI: 3.74 [95 %CI 1.02,13.70]) and TM (dose 1 RI: 2.49 [95 %CI: 1.07,5.79]) incident admissions. No associations were observed between mRNA COVID-19 vaccines and ADEM or TM. These findings translate to an extremely small absolute risk of ADEM (0.78 per million doses) and TM (1.82 per million doses) following vaccination; any potential risk of ADEM or TM should be weighed against the well-established protective benefits of vaccination against COVID-19 disease and its complications. This study demonstrates the value of the GVDN collaboration leveraging large population sizes to examine important vaccine safety questions regarding rare outcomes, as well as the value of linked population level datasets, such as VSHL, to rapidly explore associations that are identified. [ABSTRACT FROM AUTHOR]

Myelin oligodendrocyte glycoprotein-immunoglobulin G associated disease (MOGAD) is an autoimmune demyelinating disorder of the central nervous system (CNS) which usually occurs with recurrent optic neuritis, transverse myelitis, acute disseminating encephalomyelitis, or brainstem encephalitis. To date, the anti-CD 20 drug rituximab (RTX) is employed in MOGAD although some authors reported the efficacy of Tocilizumab (TCZ) in refractory patients. We present the case of a woman affected by refractory MOGAD who was treated with TCZ after therapy with RTX had failed to prevent relapses. We also conducted a current literature review on TCZ use in MOGAD. A 57-year-old Caucasian woman affected by MOGAD with severe motor impairment and cognitive dysfunction was treated from 2020 to February 2022 with RTX. However, she experienced progressive clinical and cognitive worsening associated with white matter lesions mimicking leukodystrophy. In February 2022, the patient started therapy with TCZ administered with improvement of cognitive performance, walking ability, and brainstem functions. During TCZ, our patient reached the condition of NEDA-3 (no relapse, no increase in disability, no MRI activity on neuroimaging follow-up performed in September 2023). Moreover, the patient experienced paucisymptomatic SARS-CoV-2 infection that did not modify TCZ schedule. To date, there are few evidence on the efficacy and safety of TCZ in MOGAD. However, all the reviewed cases showed that TCZ represents an effective therapy in drug-resistant MOGAD. Our case highlights the efficacy of TCZ in drug resistant MOGAD and strengthens previous reports of TCZ safety and efficacy in MOGAD. [ABSTRACT FROM AUTHOR]