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Multiple Sclerosis and Related Disorders Sep 2023Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a CNS demyelinating disease that targets myelin oligodendrocyte glycoprotein and recurs in...
BACKGROUND
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a CNS demyelinating disease that targets myelin oligodendrocyte glycoprotein and recurs in approximately 50% of patients after the initial episode. Multiple relapses may have adverse consequences, but the factors influencing relapse are unclear. This study analyzed the clinical risk factors for relapse in patients with MOGAD.
METHODS
Twenty-four MOGAD patients diagnosed at the Department of Neurology, First Hospital of Shanxi Medical University from March 2018 to November 2020 were retrospectively analyzed in this study. The patients were divided into a monophasic course and a relapsing course according to their disease process. The patients' epidemiological characteristics, clinical symptoms, laboratory tests, imaging features, and regression were summarized. Comparisons were made between the monophasic and relapsing course to identify the possible factors associated with the clinical features and recurrence.
RESULTS
At a mean follow-up of 15 months (range: 8 to 24 months), seventeen of the 24 patients (70.8%) had monophasic disease, and 7 (29.2%) had relapsing disease. Among the 24 patients, 17 patients (70.9%) had low Myelin oligodendrocyte glycoprotein antibody (MOG-IgG) serum titers (<1:100), and 7 patients (29.1%) had high MOG-IgG serum titers (≥1:100). Compared to the monophasic course group, patients in the relapsing course group had higher serum antibody titers (71.4% vs. 11.7%, P = 0.035). Onset phenotypes included encephalitis (50%), myelitis (45.8%), and optic neuritis (45.8%), with 66.7% of patients starting with a single phenotype and 33.3% starting with two or more phenotypes. Optic neuritis was more common in the relapsing course group (85.7%) than the monophasic course group (29.4%) (P = 0.023). There was no significant difference between the two groups in the proportion of myelitis and encephalitis. A previous history or background of immunological disease was present in 33.3% of patients, with a significantly higher proportion in the relapsing course group than in the monophasic course group (71.4% vs. 17.6%, P = 0.021). Regarding ancillary examinations, the relapsing course group was more likely to have CSF leukocytes higher than 50/mm than the monophasic course group (60% vs. 0, P = 0.045), while there was no difference in the number and site distribution of the lesions on MRI.
CONCLUSIONS
Our study suggests that the most common clinical manifestations of MOGAD are diminished visual acuity, limb/facial numbness, and ocular/orbital pain. The onset phenotype consisting of optic neuritis, a history of immune disease, high antibody titers (≥1:100), and high cerebrospinal fluid leukocytes (above 50/mm) suggests a high likelihood of MOGAD recurrence.
Topics: Humans; Myelin-Oligodendrocyte Glycoprotein; Retrospective Studies; Optic Neuritis; Myelitis; Encephalitis; Chronic Disease; Immunoglobulin G; Recurrence; Autoantibodies
PubMed: 37442076
DOI: 10.1016/j.msard.2023.104879 -
Magnetic Resonance Imaging Clinics of... May 2024Multiple sclerosis (MS) is a chronic inflammatory disease of the nervous system. MR imaging findings play an integral part in establishing diagnostic hallmarks of the... (Review)
Review
Multiple sclerosis (MS) is a chronic inflammatory disease of the nervous system. MR imaging findings play an integral part in establishing diagnostic hallmarks of the disease during initial diagnosis and evaluating disease status. Multiple iterations of diagnostic criteria and consensus guidelines are put forth by various expert groups incorporating imaging of the brain and spine, and efforts have been made to standardize imaging protocols for MS. Emerging ancillary imaging findings have also attracted increasing interests and should be sought for on radiologic examination. In this paper, the authors review the clinical guidelines and approach to imaging of MS and related disorders, focusing on clinically impactful image interpretation and MR imaging reporting.
Topics: Humans; Multiple Sclerosis; Magnetic Resonance Imaging; Brain; Radiography
PubMed: 38555146
DOI: 10.1016/j.mric.2024.01.001 -
American Journal of Ophthalmology Aug 2023To evaluate the effectiveness of plasma exchange (PLEX) for optic neuritis (ON).
PURPOSE
To evaluate the effectiveness of plasma exchange (PLEX) for optic neuritis (ON).
METHODS
We conducted an international multicenter retrospective study evaluating the outcomes of ON following PLEX. Outcomes were compared to raw data from the Optic Neuritis Treatment Trial (ONTT) using a matched subset.
RESULTS
A total of 395 ON attack treated with PLEX from 317 patients were evaluated. The median age was 37 years (range 9-75), and 71% were female. Causes of ON included multiple sclerosis (108), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) (92), aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) (75), seronegative-NMOSD (34), idiopathic (83), and other (3). Median time from onset of vision loss to PLEX was 2.6 weeks (interquartile range [IQR], 1.4-4.0). Median visual acuity (VA) at the time of PLEX was count fingers (IQR, 20/200-hand motion), and median final VA was 20/25 (IQR, 20/20-20/60) with no differences among etiologies except MOGAD-ON, which had better outcomes. In 81 (20.5%) ON attacks, the final VA was 20/200 or worse. Patients with poor outcomes were older (P = .002), had worse VA at the time of PLEX (P < .001), and longer delay to PLEX (P < .001). In comparison with the ONTT subset with severe corticosteroid-unresponsive ON, a final VA of worse than 20/40 occurred in 6 of 50 (12%) PLEX-treated ON vs 7 of 19 (37%) from the ONTT treated with intravenous methylprednisolone without PLEX (P = .04).
CONCLUSION
Most ON attacks improved with PLEX, and outcomes were better than attacks with similar severity in the ONTT. The presence of severe vision loss at nadir, older age, and longer delay to PLEX predicted a worse outcome whereas MOGAD-ON had a more favorable prognosis. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.
Topics: Humans; Female; Male; Plasma Exchange; Retrospective Studies; Myelin-Oligodendrocyte Glycoprotein; Optic Neuritis; Neuromyelitis Optica; Vision Disorders; Autoantibodies
PubMed: 36822570
DOI: 10.1016/j.ajo.2023.02.013 -
Cureus Apr 2024Acute median nerve neuropathy is most often due to trauma; acute carpal tunnel syndrome is considered a surgical emergency and must be ruled out. A right-hand dominant...
Acute median nerve neuropathy is most often due to trauma; acute carpal tunnel syndrome is considered a surgical emergency and must be ruled out. A right-hand dominant male presented to the emergency department with progressive unilateral pain and numbness in the median nerve distribution after experiencing a pop while doing pushups. The evaluation was limited to pain, but there was no gross deformity, and the distal right upper extremity was neurovascularly intact. All imaging was unremarkable. The patient received adequate pain control and complete resolution of symptoms. Despite presenting with symptoms congruent with possible carpal tunnel syndrome, the patient's physical exam and imaging findings were inconsistent with the diagnosis. Acute median nerve neuritis is less commonly described, and no cases have been reported secondary to push-ups, but it should be considered in nontraumatic patients. With conservative management, patients can have complete resolution and no reoccurrence of symptoms.
PubMed: 38707043
DOI: 10.7759/cureus.57549 -
Archivos de La Sociedad Espanola de... Aug 2023The objective of this research is to identify and systematize the medical conditions generated by SARS-CoV-2 on the optic nerve and retina of young, adult, and elderly... (Review)
Review
The objective of this research is to identify and systematize the medical conditions generated by SARS-CoV-2 on the optic nerve and retina of young, adult, and elderly adults who suffered from COVID-19 in the period 2019-2022. A theoretical documentary review (TDR) was conducted within the framework of an investigation to determine the current state of knowledge of the subject under study. The TDR includes the analysis of publications in the scientific databases PubMed/Medline, Ebsco, Scielo and Google. A total of 167 articles were found, of which 56 were studied in depth, and these evidence the impact of COVID-19 infection on the retina and optic nerve of infected patients, both during the acute phase and in subsequent recovery. Among the reported findings, the following stand out: anterior and posterior non-arteritic ischemic optic neuropathy, optic neuritis, central or branch vascular occlusion, paracentral acute medial maculopathy, neuroretinitis, as well as concomitant diagnoses such as possible Vogt-Koyanagi-Harada disease, multiple evanescent white dot syndrome (MEWDS), Purtscher-like retinopathy, among others.
Topics: Adult; Humans; Aged; COVID-19; SARS-CoV-2; Retina; Optic Nerve; Chorioretinitis
PubMed: 37369321
DOI: 10.1016/j.oftale.2023.06.015 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Chordoma; Skull Base Neoplasms; Head and Neck Neoplasms; Optic Neuritis; Skull
PubMed: 36728058
DOI: 10.1097/WNO.0000000000001575 -
Frontiers in Immunology 2023Optic neuritis (ON) is often an early sign of multiple sclerosis (MS), and recent studies show a link between HIF-1 pathway activation and inflammation. This study aimed...
INTRODUCTION
Optic neuritis (ON) is often an early sign of multiple sclerosis (MS), and recent studies show a link between HIF-1 pathway activation and inflammation. This study aimed to determine if inhibition of the HIF-1 pathway using the HIF-1a antagonist acriflavine (ACF) can reduce clinical progression and rescue the ocular phenotype in an experimental autoimmune encephalomyelitis (EAE) ON model.
METHODS
EAE-related ON was induced in 60 female C57BL/6J mice by immunization with MOG33-55, and 20 EAE mice received daily systemic injections of ACF at 5 mg/kg. Changes in the visual function and structure of ACF-treated EAE mice were compared to those of placebo-injected EAE mice and naïve control mice.
RESULTS
ACF treatment improved motor-sensory impairment along with preserving visual acuity and optic nerve function. Analysis of retinal ganglion cell complex alsoshowed preserved thickness correlating with increased survival of retinal ganglion cells and their axons. Optic nerve cell infiltration and magnitude of demyelination were decreased in ACF-treated EAE mice. Subsequent in vitro studies revealed improvements not only attributed to the inhibition of HIF-1 butalso to previously unappreciated interaction with the eIF2a/ATF4 axis in the unfolded protein response pathway.
DISCUSSION
This study suggests that ACF treatment is effective in an animal model of MS via its pleiotropic effects on the inhibition of HIF-1 and UPR signaling, and it may be a viable approach to promote rehabilitation in MS.
Topics: Female; Animals; Mice; Encephalomyelitis, Autoimmune, Experimental; Acriflavine; Mice, Inbred C57BL; Optic Neuritis; Retinal Ganglion Cells; Multiple Sclerosis
PubMed: 37942317
DOI: 10.3389/fimmu.2023.1271118 -
Journal of Neuroimmunology Aug 2023To evaluate the factors determining the final clinical phenotype after an initial isolated attack of optic neuritis (ON). ON could be an isolated event or the initial...
BACKGROUND AND OBJECTIVES
To evaluate the factors determining the final clinical phenotype after an initial isolated attack of optic neuritis (ON). ON could be an isolated event or the initial presentation of a chronic neuroimmunological condition.
METHODS
This was a retrospective analysis of patients presenting to University Hospitals Cleveland Medical Center for an initial, isolated attack of ON. Final clinical phenotypes were idiopathic ON, multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein associated disease (MOGAD), or secondary ON (e.g. neurosarcoidosis). Several potential predictors at the time of initial presentation were compared among the different phenotypes to determine early predictors. Categorical variables were compared using Pearson χ2 or Fisher's exact test, and continuous variables were compared using independent t-test.
RESULTS
Sixty-four patients met criteria (average age 41.3 ± 13.3, 78.1% females). Average time to final diagnosis was 8.3 months, and average follow-up was 47 months. The final phenotypes were MS (22, 34%), idiopathic ON (14, 22%), MOGAD (11, 17%), NMOSD (10, 16%), and secondary ON (7, 11%). White race, unilateral ON, short segment hyperintensity on orbital MRI, classical demyelination on brain MRI, and not requiring PLEX were associated with MS. Older age, poor steroid responsiveness, and requiring PLEX were associated with NMOSD. African American race, bilateral ON, papillitis on fundoscopy, long segment hyperintensity on orbital MRI, and normal brain MRI were associated with MOGAD. Normal or thinned retinal nerve fiber layer on OCT, short segment hyperintensity on orbital MRI, and normal brain MRI were associated with idiopathic ON.
CONCLUSION
The final clinical phenotype may be predictable at the time of initial ON presentation. This requires a careful evaluation of patient demographics, treatment response, funduscopic findings, OCT, and orbital and brain MRIs. Utilizing early predictors in clinical practice could better inform prognosis and management decisions.
PubMed: 37343437
DOI: 10.1016/j.jneuroim.2023.578130 -
Journal of Clinical Medicine Sep 2023Acute optic neuritis (AON) is a common cause of sudden visual loss in young patients. Because of the risk of demyelinating disease, patients affected by unilateral or... (Review)
Review
Acute optic neuritis (AON) is a common cause of sudden visual loss in young patients. Because of the risk of demyelinating disease, patients affected by unilateral or bilateral optic neuritis should be evaluated and treated accordingly. Despite advancements in imaging of the brain and retina, misdiagnosis of AON is not uncommon. Indeed, some acute disorders of the retina have the potential to mimic AON and their prompt diagnosis may avoid unnecessary neurologic investigation, psychological stress to the patient, and delays in treatment. This review describes uncommon retinal disorders presenting with sudden-onset visual loss and absent or subtle funduscopic manifestation that can mimic AON. Multimodal retinal imaging is essential in detecting these conditions and in their differential diagnosis. It behooves neurologists and general ophthalmologists to be aware of these entities and be familiar with multimodal imaging of the retina.
PubMed: 37685787
DOI: 10.3390/jcm12175720 -
Neurology(R) Neuroimmunology &... Jan 2024Elevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) has been largely unexplored. The objectives of this...
BACKGROUND AND OBJECTIVES
Elevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) has been largely unexplored. The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP.
METHODS
In this retrospective, single-center cohort study, we examined the clinical and paraclinical data from the initial presentation and follow-up data of children diagnosed with MOGAD. In those with opening pressure (OP) measurements, univariate analyses were used to evaluate factors associated with increased ICP, which was defined as OP > 28 cm HO. We also present a case series of patients with or without OP measurement who required medical and/or surgical management of increased ICP.
RESULTS
Of 86 children with MOGAD, 43 (50.0%) had an OP recorded and 7 (8.1%) required ICP management. In those with OP recorded, the median (interquartile range) OP for the different MOGAD phenotypes were: 30.0 (22.8-41.6) (acute disseminated encephalomyelitis, ADEM), 20.5 (16.1-23.6) (optic neuritis), 17.0 (17.0-22.5) (myelitis), and 19.5 (16.5-29.3) (other) cm H0. Overall, 20.9% had increased ICP based on an OP > 28 cm HO, of whom 77.8% presented with ADEM. In a subgroup analysis of those presenting with ADEM, those with an elevated ICP had longer hospital stay ( = 0.007) and neurologic disability (defined as modified Rankin Scale >1) ( = 0.049). In those with or without OP recorded, 7 (6 with ADEM, one with cerebral cortical encephalitis) required ICP-directed therapies. Findings on brain MRI in these 7 children revealed extensive disease burden with bilateral cerebral involvement and evidence of restricted diffusion. While neuropsychological data in this small subset revealed significant variability, all sustained identifiable deficits after discharge, including attention-deficit hyperactivity disorders and language and learning disorders.
DISCUSSION
In pediatric MOGAD, increased OP and ADEM at initial presentation were associated with longer hospital stays and greater long-term morbidity. Although invasive ICP monitoring has not been specifically advocated in the management of MOGAD, it is important to recognize signs and symptoms of increased ICP in these patients and consider ICP monitoring and management strategies based on clinical and radiologic findings, especially in those presenting with ADEM and with OP > 28 cm HO.
Topics: Humans; Child; Myelin-Oligodendrocyte Glycoprotein; Cohort Studies; Retrospective Studies; Intracranial Pressure; Intracranial Hypertension
PubMed: 37918972
DOI: 10.1212/NXI.0000000000200174