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Clinical and neuroimaging phenotypes were extracted for both adult and paediatric forms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 93 studies with 681 cases (55% males; median age\u2009=\u200946, range\u2009=\u20091\u2013103\u2009years) were included. Of these, 13 studies with a total of 535 cases were eligible for the meta\u2010analysis. Clinically, GFAP\u2010A 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\u2010attenuated 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%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This systematic review and meta\u2010analysis provide high\u2010level evidence for clinical and imaging phenotypes of GFAP\u2010A, which will benefit the identification and clinical workup of suspected cases. Differential diagnostic cues to distinguish GFAP\u2010A from common clinical and imaging mimics are provided as well as suitable magnetic resonance imaging protocol recommendations.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.16284","type":"journal-article","created":{"date-parts":[[2024,3,20]],"date-time":"2024-03-20T06:07:22Z","timestamp":1710914842000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":79,"title":["Clinical and neuroimaging phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy: A systematic review and meta\u2010analysis"],"prefix":"10.1111","volume":"31","author":[{"given":"Caroline","family":"Hagbohm","sequence":"first","affiliation":[{"name":"Department of Clinical Neuroscience Karolinska Institutet  Stockholm Sweden"},{"name":"Department of Neuroradiology Karolinska University Hospital  Stockholm 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