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

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.