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

Autor: Hagbohm C; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Department of Neuroradiology, Karolinska University Hospital, Stockholm, Sweden., Ouellette R; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Department of Neuroradiology, Karolinska University Hospital, Stockholm, Sweden., Flanagan EP; Department of Neurology, Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA., Jonsson DI; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Department of Neurophysiology, Karolinska University Hospital, Stockholm, Sweden., Piehl F; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Centre for Neurology, Academic Specialist Centre, Karolinska University Hospital, Stockholm, Sweden., Banwell B; Division 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., Wickström R; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.; Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden., Iacobaeus E; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Department of Neurology, Karolinska University Hospital, Stockholm, Sweden., Granberg T; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Department of Neuroradiology, Karolinska University Hospital, Stockholm, Sweden., Ineichen BV; Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.; Center for Reproducible Science, University of Zürich, Zürich, Switzerland.
Jazyk: angličtina
Zdroj: European journal of neurology [Eur J Neurol] 2024 Jul; Vol. 31 (7), pp. e16284. Date of Electronic Publication: 2024 Mar 20.
DOI: 10.1111/ene.16284
Abstrakt: Objective: This 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.
Methods: A 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.
Results: A 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%).
Conclusions: This 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.
(© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)
Databáze: MEDLINE
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