Evidence map›Paper›PMID 40227344›Full record

ArticleJournal of neurology2025

Progression independent of relapse activity and relapse-associated worsening in seronegative NMOSD: an international cohort study.

Pakeeran Siriratnam, Saif Huda, Anneke Van der Walt, Paul Sanfilippo, Sifat Sharmin, Yi Chao Foong, Wei Zhen Yeh, Chao Zhu, Samia J Khoury, Tunde Csepany and 20 more

Abstract readMulticenter Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Trial
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

30 authors.

Pakeeran SiriratnamDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Saif HudaDepartment of Neurology, Walton Centre NHS Foundation Trust, Liverpool, UK.
Anneke Van der WaltDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Paul SanfilippoDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Sifat SharminDepartment of Neurology, Neuroimmunology Centre, The Royal Melbourne Hospital, Parkville, VIC, Australia.
Yi Chao FoongDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Wei Zhen YehDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Chao ZhuDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Samia J KhouryNehme and Therese Tohme Multiple Sclerosis Center, American University of Beirut Medical Center, Beirut, Lebanon.
Tunde CsepanyDepartment of Neurology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
Barbara WillekensDepartment of Neurology, Antwerp University Hospital, Drie Eikenstraat 655, 2650, Edegem, Belgium.
Masoud EtemadifarFaculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Serkan OzakbasIzmir University of Economics, Medical Point Hospital, Izmir, Turkey.
Petra NytrovaDepartment of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Ayse AltintasDepartment of Neurology, School of Medicine, Koc University Research Center for Translational Medicine (KUTTAM), Koc University, Istanbul, Turkey.
Abdullah Al-AsmiCollege of Medicine & Health Sciences and Sultan, Qaboos University Hospital, Sultan Qaboos University, Al-Khodh, Oman.
Cristina Ramo-TelloDepartment of Neuroscience, Hospital Germans Trias I Pujol, Badalona, Spain.
Guy LaureysDepartment of Neurology, Universitary Hospital Ghent, Ghent, Belgium.
Francesco PattiDepartment of Medical and Surgical Sciences and Advanced Technologies, GF Ingrassia, Catania, Italy.
Dana HorakovaDepartment of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague and General University Hospital, Prague, Czech Republic.
Matteo FoschiDepartment of Neurology, Medical Faculty, Karadeniz Technical University, Trabzon, Turkey.
Cavit BozDepartment of Neurology, Medical Faculty, Karadeniz Technical University, Trabzon, Turkey.
Pamela McCombeDepartment of Neurology, Royal Brisbane Hospital, Brisbane, Australia.
Recai TurkogluDepartment of Neurology, Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey.
Izanne RoosDepartment of Neurology, Neuroimmunology Centre, The Royal Melbourne Hospital, Parkville, VIC, Australia.
Jeannette Lechner-ScottHunter Medical Research Institute, University of Newcastle, NeurologyNewcastle, Australia.
Tomas KalincikDepartment of Neurology, Neuroimmunology Centre, The Royal Melbourne Hospital, Parkville, VIC, Australia.
Vilija JokubaitisDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Helmut ButzkuevenDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia.
Mastura MonifDepartment of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia. mastura.monif@monash.edu.ORCID http://orcid.org/0000-0001-6404-9768

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPrevious studies have indicated that progression independent of relapse activity (PIRA) is uncommon in patients with aquaporin- 4 antibody-positive (AQP4-IgG) neuromyelitis optica spectrum disorder (NMOSD). However, the patterns of disability accumulation in seronegative NMOSD are unknown. This study aimed to evaluate the prevalence of PIRA and relapse-associated worsening (RAW) in seronegative NMOSD.

methodsWe conducted a retrospective, multicentre cohort study of seronegative NMOSD patients from the MSBase registry. Inclusion criteria required at least three recorded expanded disability status scale (EDSS) scores: baseline, progression, and 6 months confirmed disability progression (CDP). For those with 6-month CDP, the presence or absence of relapse between baseline and progression determined the classification as RAW or PIRA, respectively. Descriptive statistics were employed to present the data.

resultsThis study included 93 patients, with a median follow-up duration of 5.0 years (Q1 2.8, Q3 8.4). The cohort predominantly consisted of female patients (77.4%), with a median age of onset of 33.9 years (Q1 26.1, Q3 41.2). PIRA was observed in 1 case (1.1%), whilst RAW was documented in 7 cases (7.5%).

conclusionThis international cohort study confirms that CDP is uncommon in seronegative NMOSD. Given more than three quarters of CDP occur due to RAW, therapeutic strategies should focus primarily on preventing relapses.

Indexed as

Disease ProgressionNeuromyelitis OpticaAdultAquaporin 4AutoantibodiesCohort StudiesDisability EvaluationFemaleFollow-Up StudiesHumansMaleMiddle AgedRecurrenceRegistriesRetrospective StudiesYoung AdultAQP4 protein, humanAquaporin 4AutoantibodiesDisabilityEDSSNMOSDProgression independent of relapsesRelapse-associated worseningSeronegative

Identifiers

PMID40227344
PMCPMC11996963

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.