Evidence map›Paper›PMID 38506182›Full record

SynthesisEuropean journal of neurology2024

Clinical and neuroimaging phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy: A systematic review and meta-analysis.

Caroline Hagbohm, Russell Ouellette, Eoin P Flanagan, Dagur I Jonsson, Fredrik Piehl, Brenda Banwell, Ronny Wickström, Ellen Iacobaeus, Tobias Granberg, Benjamin V Ineichen

Abstract readSystematic ReviewMeta-Analysis
In one paragraph

Synthesis in European journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
42citing papers in PubMed, 2 pooled it
–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

42 citing papers in PubMed, 2 syntheses or guidelines pooled it.

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  5. EBV-associated autoimmune glial fibrillary acidic protein astrocytopathy with lumbosacral leptomeningeal enhancement: a case report and literature review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
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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

10 authors.

Caroline HagbohmDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Russell OuelletteDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Eoin P FlanaganDepartment of Neurology, Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.ORCID 0000-0002-6661-2910
Dagur I JonssonDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Fredrik PiehlDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Brenda BanwellDivision of Child Neurology, Children's Hospital of Philadelphia, Department of Neurology and Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Ronny WickströmDepartment of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.
Ellen IacobaeusDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Tobias GranbergDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Benjamin V IneichenDepartment of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.ORCID 0000-0003-1362-4819

Funding

ALF Medicine ALF 20200224CIMED FoUI-976444Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung P400PM_183884University of Zurich, UZH Alumni
6 · The paper itself

Abstract

objectiveThis study was undertaken to provide a comprehensive review of neuroimaging characteristics and corresponding clinical phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A), a rare but severe neuroinflammatory disorder, to facilitate early diagnosis and appropriate treatment.

methodsA PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis)-conforming systematic review and meta-analysis was performed on all available data from January 2016 to June 2023. Clinical and neuroimaging phenotypes were extracted for both adult and paediatric forms.

resultsA total of 93 studies with 681 cases (55% males; median age = 46, range = 1-103 years) were included. Of these, 13 studies with a total of 535 cases were eligible for the meta-analysis. Clinically, GFAP-A was often preceded by a viral prodromal state (45% of cases) and manifested as meningitis, encephalitis, and/or myelitis. The most common symptoms were headache, fever, and movement disturbances. Coexisting autoantibodies (45%) and neoplasms (18%) were relatively frequent. Corticosteroid treatment resulted in partial/complete remission in a majority of cases (83%). Neuroimaging often revealed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities (74%) as well as perivascular (45%) and/or leptomeningeal (30%) enhancement. Spinal cord abnormalities were also frequent (49%), most commonly manifesting as longitudinally extensive myelitis. There were 88 paediatric cases; they had less prominent neuroimaging findings with lower frequencies of both T2/FLAIR hyperintensities (38%) and contrast enhancement (19%).

conclusionsThis systematic review and meta-analysis provide high-level evidence for clinical and imaging phenotypes of GFAP-A, which will benefit the identification and clinical workup of suspected cases. Differential diagnostic cues to distinguish GFAP-A from common clinical and imaging mimics are provided as well as suitable magnetic resonance imaging protocol recommendations.

Indexed as

Glial Fibrillary Acidic ProteinNeuroimagingAstrocytesAutoantibodiesAutoimmune Diseases of the Nervous SystemHumansNeuroinflammatory DiseasesPhenotypeAutoantibodiesGFAP protein, humanGlial Fibrillary Acidic Proteincentral nervous system diseasesencephalopathyGFAP protein, humanmagnetic resonance imagingsystematic review

Identifiers

PMID38506182
PMCPMC11235751

What OpenQuestion holds

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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.