RT Journal Article
SR Electronic
T1 Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1394
OP 1402
DO 10.3174/ajnr.A8236
VO 45
IS 10
A1 Shetty, Dhruv
A1 Brahmbhatt, Sneh
A1 Desai, Amit
A1 Bathla, Girish
A1 Mohan, Suyash
A1 Gupta, Vivek
A1 Soni, Neetu
A1 Vibhute, Prasanna
A1 Agarwal, Amit
YR 2024
UL http://www.ajnr.org/content/45/10/1394.abstract
AB SUMMARY: Glial fibrillary acidic protein (GFAP) astrocytopathy is a recently described autoimmune inflammatory disorder of the CNS characterized by the presence of specific antibodies targeting the intracellular filament protein in mature astrocytes. The pathogenesis is heterogeneous and poorly understood, with around 20%–34% of cases occurring as a paraneoplastic syndrome, most frequently associated with ovarian teratomas. It presents clinically as acute or subacute encephalomyelitis, and the diagnosis relies on imaging and detection of GFAP-Immunoglobulin (GFAP-IgG) in the CSF. Characteristic imaging findings include linear perivascular enhancement in the white matter extending in a radial pattern. Other imaging findings include periependymal enhancement, longitudinally extensive cord signal changes, intramedullary enhancement, optic neuritis, and papillitis. There is significant imaging overlap with other neuroinflammatory diseases like neuromyelitis optica spectrum disorder and lymphoproliferative conditions. GFAP astrocytopathy is characteristically responsive to steroids with, however, a significant rate of relapse. Currently, literature on this novel entity is limited with no established diagnostic criteria or standard treatment regimen. This comprehensive review explores the clinical, radiographic, and histopathologic aspects of GFAP astrocytopathy, shedding light on its complex nature and potential diagnostic challenges. The paper highlights the neuroimaging findings with a focus on differentiating GFAP astrocytopathy from other neuroinflammatory disorders.ADEMacute disseminated encephalomyelitisAQP4aquaporin 4CLIPPERSchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroidsIFAimmunofluorescence assayIgGImmunoglobulin GGFAPglial fibrillary acidic proteinMOGADmyelin oligodendrocyte glycoprotein antibody-associated diseaseNMOSDneuromyelitis optica spectrum disorderOCToptical coherence tomographyPACNSprimary angiitis of the CNSSLIPPERSsupratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroidsWBCwhite blood cell