Evidence map›Paper›PMID 37433584›Full record

SynthesisEuropean journal of neurology2023

Non-demyelinating disorders mimicking and misdiagnosed as NMOSD: a literature review.

Pietro Zara, Alessandro Dinoto, Sara Carta, Valentina Floris, Davide Turilli, Adrian Budhram, Sergio Ferrari, Stefania Milia, Paolo Solla, Sara Mariotto and 3 more

Open access · bronzeAbstract readSystematic Review
In one paragraph

Synthesis in European journal of neurology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed, 1 pooled it
5.1field-weighted citation impact, top 4% of its field
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

11 citing papers in PubMed, 1 synthesis or guideline pooled it, 20 citations in OpenAlex.

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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

13 authors at 5 institutions in 3 countries.

Pietro ZaraNeurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.
Alessandro DinotoNeurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.ORCID 0000-0002-3249-4792
Sara CartaNeurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.
Valentina FlorisNeurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.
Davide TurilliNeurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.
Adrian BudhramDepartment of Clinical Neurological Sciences, Western University, London, Ontario, Canada.
Sergio FerrariNeurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.ORCID 0000-0003-3855-5135
Stefania MiliaNeurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.
Paolo SollaNeurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.
Sara MariottoNeurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy.ORCID 0000-0002-7806-3103
Eoin P FlanaganDepartment of Neurology, Mayo Clinic, Rochester, Minnesota, USA.ORCID 0000-0002-6661-2910
A Sebastian Lopez ChiribogaDepartment of Neurology, Mayo Clinic, Jacksonville, Florida, USA.
Elia SechiNeurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.ORCID 0000-0003-4698-663X
University of Sassari · ITUniversity of Verona · ITJacksonville College · USMayo Clinic · USWestern University · CA

Funding

Utilizing epidemiology, imaging and fluid biomarkers in MOG antibody-associated disease to inform prognosis, pathogenesis and therapeuticsR01NS113828 · NINDS · MAYO CLINIC ROCHESTER · PI Eoin P Flanagan · 2020 to 2026
$2.4M
NINDS NIH HHS R01 NS113828
6 · The paper itself

Abstract

backgroundDifferentiating neuromyelitis optica spectrum disorder (NMOSD) from its mimics is crucial to avoid misdiagnosis, especially in the absence of aquaporin-4-IgG. While multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein-IgG associated disease (MOGAD) represent major and well-defined differential diagnoses, non-demyelinating NMOSD mimics remain poorly characterized.

methodsWe conducted a systematic review on PubMed/MEDLINE to identify reports of patients with non-demyelinating disorders that mimicked or were misdiagnosed as NMOSD. Three novel cases seen at the authors' institutions were also included. The characteristics of NMOSD mimics were analyzed and red flags associated with misdiagnosis identified.

resultsA total of 68 patients were included; 35 (52%) were female. Median age at symptoms onset was 44 (range, 1-78) years. Fifty-six (82%) patients did not fulfil the 2015 NMOSD diagnostic criteria. The clinical syndromes misinterpreted for NMOSD were myelopathy (41%), myelopathy + optic neuropathy (41%), optic neuropathy (6%), or other (12%). Alternative etiologies included genetic/metabolic disorders, neoplasms, infections, vascular disorders, spondylosis, and other immune-mediated disorders. Common red flags associated with misdiagnosis were lack of cerebrospinal fluid (CSF) pleocytosis (57%), lack of response to immunotherapy (55%), progressive disease course (54%), and lack of magnetic resonance imaging gadolinium enhancement (31%). Aquaporin-4-IgG positivity was detected in five patients by enzyme-linked immunosorbent assay (n = 2), cell-based assay (n = 2: serum, 1; CSF, 1), and non-specified assay (n = 1).

conclusionsThe spectrum of NMOSD mimics is broad. Misdiagnosis frequently results from incorrect application of diagnostic criteria, in patients with multiple identifiable red flags. False aquaporin-4-IgG positivity, generally from nonspecific testing assays, may rarely contribute to misdiagnosis.

Indexed as

Neuromyelitis OpticaSpinal Cord DiseasesAquaporin 4AutoantibodiesContrast MediaFemaleGadoliniumHumansImmunoglobulin GMaleMyelin-Oligodendrocyte GlycoproteinAquaporin 4AutoantibodiesContrast MediaGadoliniumImmunoglobulin GMyelin-Oligodendrocyte Glycoproteindifferential diagnosisfalse positivitymimickersmisdiagnosisneuromyelitis optica spectrum disorder

Identifiers

PMID37433584
PMCPMC10530555
OpenAlexW4383873970

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

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.