A Phase Ib Clinical Study to Evaluate the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Preliminary Efficacy of B001 in Subjects With Aquaporin-4 Antibody (AQP4-IgG) Positive Neuromyelitis Optic Spectrum Disorder (NMOSD)

The objectives of this phase Ib study are to evaluate the efficacy, safety, pharmacokinetics, pharmacodynamics and immunogenic profiles of B001 in subjects with aquaporin-4 antibody (AQP4-IgG) positive NMOSD.

Start Date07 Apr 2022

Sponsor / Collaborator

Shanghai Pharmaceuticals Holding Co., Ltd.

NCT05540262

/ RecruitingNot ApplicableIIT

Edaravone in the Treatment of Aquaporin-4 Antibody-positive Optic Neuritis

Edaravone has demonstrated a beneficial effect in promoting remyelination and protecting axons in animals with NMOSD. The researchers posit that edaravone may enhance visual outcomes in patients with aquaporin-4 antibody-positive optic neuritis.

Start Date15 Jan 2022

Sponsor / Collaborator

First Affiliated Hospital of Guangxi Medical University

NCT03586557

/ Unknown statusNot ApplicableIIT

Effectiveness of Plasma Exchange in Treating With Severe Acute AQP4-Ab Positive Optic Neuritis

Patients aged between 18 and 70 with acute aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) positive optic neuritis, irrespective of prior using of corticosteroids in this episode of disease, are chosen by the physician. Patients will then be randomized to receive high dose of intravenous corticosteroids combined with plasma exchange (PE), or merely high dose of intravenous corticosteroids followed subsequent taper. The main outcome of visual acuity and OCT parameters will be compared at baseline, one, three and six months after treatments, and other assessments will also be recorded and compared. This will allow for determination on whether additional PE plays a role in better prognosis in acute AQP4-IgG positive optic neuritis.

Start Date01 Sep 2018

Sponsor / Collaborator

Chinese People's Liberation Army General Hospital

100 Clinical Results associated with AQP4-IgG positive Optic Neuritis

100 Translational Medicine associated with AQP4-IgG positive Optic Neuritis

0 Patents (Medical) associated with AQP4-IgG positive Optic Neuritis

139

Literatures (Medical) associated with AQP4-IgG positive Optic Neuritis

01 Jan 2025·Neurology Neuroimmunology & Neuroinflammation

A Retrospective Single-Center Study of Myelitis Associated With Rheumatologic Disease

Article

Author: Krett, Jonathan D. ; Barreras, Paula ; Filippatou, Angeliki G. ; Sotirchos, Elias S. ; Gelber, Allan C. ; Pardo, Carlos A.

BACKGROUND AND OBJECTIVESPrevious reports of patients with myelitis associated with rheumatologic disease may have had unrecognized aquaporin-4 (AQP4)-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD). We clinicoradiologically and serologically characterized patients with myelitis associated with rheumatologic disease evaluated in the era of availability of MOG-IgG and more sensitive AQP4-IgG cell-based assays.METHODSA retrospective cohort (2018-2023) at Johns Hopkins Medicine with diagnoses of myelopathy and rheumatologic comorbidity was identified by electronic medical record (EMR) query. All patients with myelitis unrelated to typical multiple sclerosis (MS) were included and analyzed by chart review.RESULTSOf 238 patients identified by EMR query, 197 were excluded (148 not meeting prespecified inclusion criteria, 49 had typical MS), resulting in 41 patients for review. The mean age at myelitis onset was 44 ± 15 years; 39 (95%) were female. Rheumatologic diagnoses included 17 (41.5%) with systemic lupus erythematosus (SLE), 10 (24.3%) Sjögren syndrome (SS), 6 (15%) undifferentiated connective tissue disease (UCTD), 5 (12%) combinations of SLE/SS/UCTD with antiphospholipid antibody syndrome, 1 (2.4%) rheumatoid arthritis, 1 (2.4%) psoriatic arthritis, and 1 (2.4%) Behçet disease. 20 patients (49%) were diagnosed with AQP4-IgG seropositive NMOSD, 3 (7%) with MOGAD, and 18 (44%) had "double-seronegative" myelitis. Of these 18, 3 were diagnosed with AQP4-IgG seronegative NMOSD, 1 neuro-Behçet disease, and 14 other (unclassifiable) myelitis. Excluding 1 patient with neuro-Behçet disease, 18 (90%) of 20 AQP4-IgG seropositive patients had longitudinally extensive cord lesions compared with 5 (29%; p < 0.001) of 17 "double-seronegative" patients and 2 (67%) of 3 with MOGAD. "Double-seronegative" patients more commonly had CSF-restricted oligoclonal bands. Functional outcomes did not differ by diagnosis, and most patients received acute immunotherapy at the time of initial myelitis diagnosis with at least partial recovery over a median follow-up of 38 (interquartile range: 9-74) months.DISCUSSIONApproximately half of our rheumatologic disease cohort with myelitis unrelated to MS had AQP4-IgG seropositive NMOSD while MOGAD accounted for a small but clinically relevant proportion of patients. Further research is needed to characterize myelitis etiology in patients who are seronegative for both AQP4-IgG and MOG-IgG.

01 Jan 2025·Multiple Sclerosis and Related Disorders

Children with MOG-IgG positive bilateral optic neuritis misdiagnosed as fulminant idiopathic intracranial hypertension

Article

Author: Wendel, Eva-Maria ; Reindl, Markus ; Tibussek, Daniel ; Sartori, Stefano ; Nosadini, Margherita ; Yilmaz, Deniz ; Panzer, Andreas ; Geis, Tobias ; Schoene-Bake, Jan-Christoph ; Barisic, Nina ; Bertolini, Annikki ; Pakeerathan, Thivya ; Knierim, Ellen ; Ayzenberg, Ilya ; Nikolaus, Marc ; Rostásy, Kevin ; Chang, Petrus

BACKGROUNDFulminant idiopathic intracranial hypertension (IIH) is characterized by headache, rapid decrease of vision and elevated CSF-opening pressure.OBJECTIVETo delineate a subgroup of MOGAD mimicking fulminant IIH.METHODSIn this case series children with MOGAD with vision loss, optic disc swelling and elevated CSF opening pressure, initially diagnosed with fulminant IIH, were included.RESULTS8 MOGAD patients (median age 12.5y, f:m = 7:1) with an initial diagnosis of fulminant IIH were included. Rapid bilateral visual loss and headache was present in all patients in addition to bilateral optic disc swelling and decreased visual acuity. In 2 patients cMRI showed prominent optic discs or an empty sella sign. In 7 patients the CSF opening pressure was markedly elevated. Patients were treated with Acetazolamide (n = 6), CSF external drainage (n = 2) or implantation of VP shunt (n = 1). Intravenous Methylprednisolone was administered in all patients because of worsening of symptoms, positive MOG-ab titers and/or new MRI white matter lesions. Visual recovery was good with residuals in 1/8 children. OCT revealed thickening of pRNFL in the acute stage in all patients with a rapid declining of pRNFL and retinal atrophy thereafter.CONCLUSIONChildren with bilateral MOGAD-ON presenting with loss of vision and elevated CSF opening pressure are at risk of being misdiagnosed as fulminant IIH. The role of elevated CSF opening pressure in MOGAD warrants further investigations. We recommend to include measurement of CSF opening pressure in the work up of neuroinflammatory diseases, in particular in patients with suspected MOGAD, to consider adapted pressure management.

01 Nov 2024·Multiple Sclerosis and Related Disorders

Preliminary findings of a ‘test bundle’ to accelerate the diagnosis of MS and NMOSD following optic neuritis

Article

Author: Lundgren, Karen B ; Anderson-Benge, Ellen ; McLouth, Christopher ; Das, Saurav ; Lundgren, Karen B. ; Khawla, Abusamra ; Anderson-benge, Ellen ; Avasarala, Jagannadha ; Wilkerson, Paul

No study has investigated the length of time it takes to diagnose multiple sclerosis (MS) or neuromyelitis optic spectrum disorder (NMOSD, aquaporin 4 antibody disease or myelin oligodendrocyte glycoprotein antibody disease, MOGAD) following the onset of de novo optic neuritis (ON). Minimizing the time between ON and downstream diagnoses needs urgency since early diagnosis equals early treatment. The time elapsed from ON to a subsequent diagnosis of MS/NMOSD was estimated through analysis of retrospective data collected from the Axon Registry (AR) of the American Academy of Neurology (AAN) and from the University of Kentucky (UK), Lexington. The time to diagnose MS/NMOSD was arbitrarily set as occurring < 6 months (early) or > 6 months (delayed) following ON. Data was collected between 2007 and 2021 (AR) and 2012 to 2022, for UK, respectively. Of the 4015 ON patients from the AR dataset, 1069 (26.6 %) were diagnosed with MS, with 857 (80.2 %) diagnosed < 6 months (early) and 212 (19.8 %) diagnosed after > 6 months (delayed). Secondly, 420/4015 (10.4 %) were diagnosed with NMOSD (either MOGAD or AQP4 antibody disease), of which 340/420 (80.9 %) were diagnosed < 6 months (early) and 80/420 (19 %) diagnosed > 6 months (delayed). In the UK dataset, a total of 90/1464 individuals (6.14 %) were diagnosed with MS; of these, 69 patients (76.7 %) were diagnosed at < 6 months (early) and included a sub-group of 25 (27.8 %) diagnosed < 4 weeks; 21 (23.3 %) were diagnosed > 6 months (delayed) following ON. In either dataset (AR or UK, between 20 % - 23 % of MS diagnoses occurred > 6 months (delayed) after a diagnosis of ON. An accelerated diagnosis (4 weeks or less) of MS/NMOSD following ON in the UK data suggests that it is possible to minimize the time to a downstream diagnosis if a 'test bundle' of MRI of orbits, brain, C-spine, cerebrospinal fluid (CSF) analysis, and serum testing for NMOSD is used. Additional studies using prospective, larger datasets are required to confirm our findings.

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