Skip to main content
Advertisement

Main menu

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home

User menu

  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

ASHNR American Society of Functional Neuroradiology ASHNR American Society of Pediatric Neuroradiology ASSR
  • Alerts
  • Log in

Advanced Search

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds

AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Review ArticleUltra-High-Field MRI/Imaging of Epilepsy/Demyelinating Diseases/Inflammation/Infection

Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics

Dhruv Shetty, Sneh Brahmbhatt, Amit Desai, Girish Bathla, Suyash Mohan, Vivek Gupta, Neetu Soni, Prasanna Vibhute and Amit Agarwal
American Journal of Neuroradiology October 2024, 45 (10) 1394-1402; DOI: https://doi.org/10.3174/ajnr.A8236
Dhruv Shetty
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sneh Brahmbhatt
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Amit Desai
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Amit Desai
Girish Bathla
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Girish Bathla
Suyash Mohan
bDepartment of Radiology, Perelman School of Medicine (S.M), University of Pennsylvania, Philadelphia, Pennsylvania
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Vivek Gupta
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Vivek Gupta
Neetu Soni
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Neetu Soni
Prasanna Vibhute
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Prasanna Vibhute
Amit Agarwal
aFrom the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Amit Agarwal
  • Article
  • Figures & Data
  • Supplemental
  • Info & Metrics
  • Responses
  • References
  • PDF
Loading

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Abstract

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.

ABBREVIATIONS:

ADEM
acute disseminated encephalomyelitis
AQP4
aquaporin 4
CLIPPERS
chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
IFA
immunofluorescence assay
IgG
Immunoglobulin G
GFAP
glial fibrillary acidic protein
MOGAD
myelin oligodendrocyte glycoprotein antibody-associated disease
NMOSD
neuromyelitis optica spectrum disorder
OCT
optical coherence tomography
PACNS
primary angiitis of the CNS
SLIPPERS
supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids
WBC
white blood cell
  • © 2024 by American Journal of Neuroradiology
View Full Text

Log in using your username and password

Forgot your user name or password?

Log in through your institution

You may be able to gain access using your login credentials for your institution. Contact your library if you do not have a username and password.
PreviousNext
Back to top

In this issue

American Journal of Neuroradiology: 45 (10)
American Journal of Neuroradiology
Vol. 45, Issue 10
1 Oct 2024
  • Table of Contents
  • Index by author
  • Complete Issue (PDF)
Advertisement
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on American Journal of Neuroradiology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics
(Your Name) has sent you a message from American Journal of Neuroradiology
(Your Name) thought you would like to see the American Journal of Neuroradiology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Cite this article
Dhruv Shetty, Sneh Brahmbhatt, Amit Desai, Girish Bathla, Suyash Mohan, Vivek Gupta, Neetu Soni, Prasanna Vibhute, Amit Agarwal
Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics
American Journal of Neuroradiology Oct 2024, 45 (10) 1394-1402; DOI: 10.3174/ajnr.A8236

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
0 Responses
Respond to this article
Share
Bookmark this article
Astrocytopathy: Imaging Features & Mimics
Dhruv Shetty, Sneh Brahmbhatt, Amit Desai, Girish Bathla, Suyash Mohan, Vivek Gupta, Neetu Soni, Prasanna Vibhute, Amit Agarwal
American Journal of Neuroradiology Oct 2024, 45 (10) 1394-1402; DOI: 10.3174/ajnr.A8236
del.icio.us logo Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Purchase

Jump to section

  • Article
    • Abstract
    • ABBREVIATIONS:
    • EPIDEMIOLOGY AND CLINICAL FEATURES
    • PATHOPHYSIOLOGY
    • MOLECULAR MARKERS AND HISTOPATHOLOGY
    • NEUROIMAGING PATTERN
    • RADIOGRAPHIC MIMICS
    • Conclusions
    • Footnotes
    • References
  • Figures & Data
  • Supplemental
  • Info & Metrics
  • Responses
  • References
  • PDF

Related Articles

  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Crossref (6)
  • Google Scholar

This article has been cited by the following articles in journals that are participating in Crossref Cited-by Linking.

  • Unveiling GFAP Astrocytopathy: Insights from Case Studies and a Comprehensive Review of the Literature
    Panagiotis Gklinos, Fotios Athanasopoulos, Vagia Giatrakou, Nikolaos-Achilleas Arkoudis, Dorothea Pournara, Eirini Giagkou, Argyro Tountopoulou, Sofia Vassilopoulou, Dimos-Dimitrios Mitsikostas
    Antibodies 2024 13 4
  • Intercellular Adhesion Molecule 1 (ICAM-1): An Inflammatory Regulator with Potential Implications in Ferroptosis and Parkinson’s Disease
    Matthew R. Miller, Harold E. Landis, Robert E. Miller, Yousef Tizabi
    Cells 2024 13 18
  • Autoimmune glial fibrillary acidic protein astrocytopathy misdiagnosed as intracranial infectious diseases: case reports and literature review
    Runhua Bai, Li An, Wei Du, Zhiwei Wang, Xiaokun Qi, Jianguo Liu, Ming Ren, Yingxin Yu
    Frontiers in Immunology 2025 16
  • Die Anti-GFAP-Astrozytopathie und ihre Differenzialdiagnosen
    Neuroradiologie Scan 2025 15 02
  • Impaired glymphatic function in autoimmune glial fibrillary acidic protein astrocytopathy: a prospective analysis
    Xiaofeng Xu, Xiaohong Su, Li Xu, Xiaodong Chen, Dongcheng Li, Junyu Liu, Kai Dai, Jia Liu, Ying Jiang, Fuhua Peng
    Multiple Sclerosis and Related Disorders 2025 99
  • Role of Glial Cells and Receptors in Schizophrenia Pathogenesis
    Yousef Tizabi, Marta C. Antonelli, Daniela Tizabi, Michael Aschner
    Neurochemical Research 2025 50 2

More in this TOC Section

  • 7T MRI vasculitis imaging
  • Synthetic MRI Links to MS Disability
  • 7T MRI of the Internal Auditory Canal
Show more ULTRA-HIGH-FIELD MRI/IMAGING OF EPILEPSY/DEMYELINATING DISEASES/INFLAMMATION/INFECTION

Similar Articles

Advertisement

Indexed Content

  • Current Issue
  • Accepted Manuscripts
  • Article Preview
  • Past Issues
  • Editorials
  • Editor's Choice
  • Fellows' Journal Club
  • Letters to the Editor
  • Video Articles

Cases

  • Case Collection
  • Archive - Case of the Week
  • Archive - Case of the Month
  • Archive - Classic Case

More from AJNR

  • Trainee Corner
  • Imaging Protocols
  • MRI Safety Corner
  • Book Reviews

Multimedia

  • AJNR Podcasts
  • AJNR Scantastics

Resources

  • Turnaround Time
  • Submit a Manuscript
  • Submit a Video Article
  • Submit an eLetter to the Editor/Response
  • Manuscript Submission Guidelines
  • Statistical Tips
  • Fast Publishing of Accepted Manuscripts
  • Graphical Abstract Preparation
  • Imaging Protocol Submission
  • Evidence-Based Medicine Level Guide
  • Publishing Checklists
  • Author Policies
  • Become a Reviewer/Academy of Reviewers
  • News and Updates

About Us

  • About AJNR
  • Editorial Board
  • Editorial Board Alumni
  • Alerts
  • Permissions
  • Not an AJNR Subscriber? Join Now
  • Advertise with Us
  • Librarian Resources
  • Feedback
  • Terms and Conditions
  • AJNR Editorial Board Alumni

American Society of Neuroradiology

  • Not an ASNR Member? Join Now

© 2025 by the American Society of Neuroradiology All rights, including for text and data mining, AI training, and similar technologies, are reserved.
Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire