Related Experiment Video
Updated: Jun 7, 2025

Human Serum Anti-aquaporin-4 Immunoglobulin G Detection by Cell-based Assay
Published on: April 5, 2019
Prevalence of Progression Independent of Relapse Activity and Relapse-Associated Worsening in Patients With
Pakeeran Siriratnam1, Saif Huda1, Anneke Van Der Walt1
1From the Department of Neuroscience (P.S., A.V.D.W., P.G.S., Y.C.F., W.Z.Y., C.Z., V.G.J., H.B., M.M.), Central Clinical School, Monash University, Melbourne, Victoria; Department of Neurology (P.S., A.V.D.W., P.G.S., Y.C.F., W.Z.Y., V.G.J., H.B., M.M.), Alfred Health, Melbourne, Victoria, Australia; Department of Neurology (P.S., S.H.), Walton Centre NHS Foundation Trust, Liverpool, United Kingdom; Neuroimmunology Centre (S.S., I.R., T.K.), Department of Neurology, The Royal Melbourne Hospital, Parkville; CORe (S.S., I.R., T.K.), Department of Medicine, University of Melbourne, Victoria; Royal Hobart Hospital (Y.C.F.), Hobart, Tasmania, Australia; Nehme and Therese Tohme Multiple Sclerosis Center (S.J.K.), American University of Beirut Medical Center, Beirut, Lebanon; Department of Neurology (T.C.), Faculty of Medicine, University of Debrecen, Hungary; Department of Neurology (B.W.), Antwerp University Hospital, Edegem; Translational Neurosciences Research Group (B.W.), Faculty of Medicine and Health Sciences, University of Antwerp, Wilrijk, Belgium; Faculty of Medicine (M.E.), Isfahan University of Medical Sciences; Neurology (M.E.), Dr. Etemadifar MS Institute, Isfahan, Iran; Izmir University of Economics (S.O.), Medical Point Hospital; Multiple Sclerosis Research Association (S.O.), Izmir, Turkey; Department of Neurology and Center of Clinical Neuroscience (P.N., D.H.), First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic; Department of Neurology (A.A.), School of Medicine and Koc University Research Center for Translational Medicine (KUTTAM), Koc University, Istanbul, Turkey; College of Medicine & Health Sciences and Sultan Qaboos University Hospital (A.A.-A.), Sultan Qaboos University, Al-Khodh, Oman; Department of Neuroscience (C.M.R.-T.), Hospital Germans Trias I Pujol, Badalona, Spain; Department of Neurology (G.L.), University Hospital Ghent, Belgium; Department of Medical and Surgical Sciences and Advanced Technologies (F.P.), GF Ingrassia, Catania, Italy; Multiple Sclerosis Unit (F.P.), AOU Policlinico G Rodolico-San Marco, University of Catania; Department of Neuroscience (M.F.), MS Center, Neurology Unit, S. Maria delle Croci Hospital, AUSL Romagna, Ravenna, Italy; Department of Biotechnological and Applied Clinical Sciences (DISCAB) (M.F.), University of L'Aquila, Italy; Department of Neurology (C.B.), Karadeniz Technical University, Medical Faculty, Trabzon, Turkey; Department of Neurology (P.A.M.), Royal Brisbane Hospital; University of Queensland (P.A.M.), Australia; Department of Neurology (R.T.), Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey; Hunter Medical Research Institute (J.L.-S.), Neurology, University of Newcastle; and Hunter New England Health (J.L.-S.), John Hunter Hospital, New South Wales, Australia.
Objectives:
In aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), disability accrual is mostly attributed to relapses. This study aimed to assess the prevalence of progression independent of relapse activity (PIRA) and relapse-associated worsening (RAW) in AQP4-IgG NMOSD.
Methods:
This was a retrospective cohort study of patients with AQP4-IgG NMOSD enrolled in the MSBase international data registry. Patients required a minimum of 3 recorded Expanded Disability Status Scale (EDSS) scores: baseline, event, and a 6-month confirmation score. Presence and absence of relapses between the baseline and event EDSS scores determined RAW and PIRA, respectively. Descriptive statistics were used to present the results.
Results:
A total of 181 patients followed for a median of 4.5 years (Q1 1.7, Q3 7.8) were included. Most patients were female (88.4%), and the median age at disease onset was 38.1 years. Overall, 4 patients (2.2%) developed 5 incidences of PIRA and 13 patients developed RAW (7.2%).
Discussion:
This multicenter study highlights that PIRA is very rare in AQP4-IgG NMOSD. Limitations of this study include the sole focus of overall EDSS to measure disability, lack of requirement for a second EDSS score to confirm baseline EDSS, and the absence of magnetic resonance imaging information for all patients.
More Related Videos
09:29Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
Published on: August 21, 2017
10:46A Method of Trigonometric Modelling of Seasonal Variation Demonstrated with Multiple Sclerosis Relapse Data
Published on: December 9, 2015