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<meta name="DC.Title" content="Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
<meta name="DC.Identifier" content="10.3174/ajnr.A8236" />
<meta name="DC.Date" content="2024-10-01" />
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<meta name="DC.Description" content="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.

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" />
<meta name="DC.Contributor" content="Dhruv Shetty" />
<meta name="DC.Contributor" content="Sneh Brahmbhatt" />
<meta name="DC.Contributor" content="Amit Desai" />
<meta name="DC.Contributor" content="Girish Bathla" />
<meta name="DC.Contributor" content="Suyash Mohan" />
<meta name="DC.Contributor" content="Vivek Gupta" />
<meta name="DC.Contributor" content="Neetu Soni" />
<meta name="DC.Contributor" content="Prasanna Vibhute" />
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<meta name="article:published_time" content="2024-10-01" />
<meta name="article:section" content="Ultra-High-Field MRI/Imaging of Epilepsy/Demyelinating Diseases/Inflammation/Infection" />
<meta name="citation_title" content="Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;SUMMARY:&lt;/h3&gt;
&lt;p&gt;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.&lt;/p&gt;" />
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<meta name="twitter:title" content="Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
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<meta name="twitter:description" content="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.

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" />
<meta name="og-title" property="og:title" content="Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics" />
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<meta name="og-site-name" property="og:site_name" content="American Journal of Neuroradiology" />
<meta name="og-description" property="og:description" content="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.

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" />
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    <div class="highwire-article-citation highwire-citation-type-highwire-article node110802--5" data-node-nid="110802" id="top-node-110802--6488655029" data-pisa="ajnr;45/10/1394" data-pisa-master="ajnr;ajnr.A8236" data-apath="/ajnr/45/10/1394.atom" data-hw-author-tooltip-instance="highwire_author_tooltip"><div class="highwire-cite highwire-cite-highwire-article highwire-citation-jcore-article-title-complete clearfix has-author-tooltip highwire-citation-highwire-article-top-a">
  
  
      <div class="highwire-cite-overline"><span  class="highwire-cite-metadata-article-type highwire-cite-metadata">Review Article</span><span class="separator-pipe"></span><span  class="highwire-cite-metadata-art-cat highwire-cite-metadata"><span class="wrapper">Ultra-High-Field MRI/Imaging of Epilepsy/Demyelinating Diseases/Inflammation/Infection</span></span></div>
  
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      <h1 class="highwire-cite-title" id="page-title">Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics</h1>  
    	<div class="highwire-cite-authors"><span  class="highwire-citation-authors"><span class="highwire-citation-author first" data-delta="0">Dhruv Shetty</span>, <span class="highwire-citation-author" data-delta="1">Sneh Brahmbhatt</span>, <span class="highwire-citation-author" data-delta="2">Amit Desai</span>, <span class="highwire-citation-author" data-delta="3">Girish Bathla</span>, <span class="highwire-citation-author" data-delta="4">Suyash Mohan</span>, <span class="highwire-citation-author" data-delta="5">Vivek Gupta</span>, <span class="highwire-citation-author" data-delta="6">Neetu Soni</span>, <span class="highwire-citation-author" data-delta="7">Prasanna Vibhute</span> and <span class="highwire-citation-author" data-delta="8">Amit Agarwal</span></span></div>
  
    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">October 2024,  </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">45 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1394-1402; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata">DOI: https://doi.org/10.3174/ajnr.A8236 </span></div>
  
  
  
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<div id="hw-article-author-popups-top-node-110802--6488655029" style="display: none;"><div class="author-tooltip-0"><div class="author-tooltip-name">Dhruv Shetty </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Dhruv%2BShetty%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Shetty%20D&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li 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target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3ASneh%2BBrahmbhatt%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-2"><div class="author-tooltip-name">Amit Desai </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Amit%2BDesai%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Desai%20A&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AAmit%2BDesai%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0002-3073-6823" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Amit Desai</a></li></ul></div><div class="author-tooltip-3"><div class="author-tooltip-name">Girish Bathla </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Girish%2BBathla%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Bathla%20G&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AGirish%2BBathla%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0003-3170-242X" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Girish Bathla</a></li></ul></div><div class="author-tooltip-4"><div class="author-tooltip-name">Suyash Mohan </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>b</span>Department of Radiology, Perelman School of Medicine (S.M), University of Pennsylvania, Philadelphia, Pennsylvania</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Suyash%2BMohan%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Mohan%20S&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link last"><a href="/search/author1%3ASuyash%2BMohan%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li></ul></div><div class="author-tooltip-5"><div class="author-tooltip-name">Vivek Gupta </div><div class="author-tooltip-affiliation"><span class="author-tooltip-text"><div class='author-affiliation'><span class='nlm-sup'>a</span>From the Department of Radiology (D.S., S.B., A.D., G.B., V.G., N.S., P.V., A.A.), Mayo Clinic, Jacksonville, Florida</div></span></div><ul class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Vivek%2BGupta%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Gupta%20V&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3AVivek%2BGupta%2B" 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class="author-tooltip-find-more"><li class="author-tooltip-gs-link first"><a href="/lookup/google-scholar?link_type=googlescholar&amp;gs_type=author&amp;author%5B0%5D=Prasanna%2BVibhute%2B" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on Google Scholar</a></li><li class="author-tooltip-pubmed-link"><a href="/lookup/external-ref?access_num=Vibhute%20P&amp;link_type=AUTHORSEARCH" target="_blank" class="" data-icon-position="" data-hide-link-title="0">Find this author on PubMed</a></li><li class="author-site-search-link"><a href="/search/author1%3APrasanna%2BVibhute%2B" rel="nofollow" class="" data-icon-position="" data-hide-link-title="0">Search for this author on this site</a></li><li class="author-orcid-link last"><a href="https://orcid.org/0000-0003-4767-1867" target="_blank" class="" data-icon-position="" data-hide-link-title="0">ORCID record for Prasanna Vibhute</a></li></ul></div><div class="author-tooltip-8"><div 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        	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-volume highwire-cite-metadata">Vol. 45</span>, <span  class="highwire-cite-metadata-issue highwire-cite-metadata">Issue 10 </span><div  class="highwire-cite-metadata-pubdate highwire-cite-metadata">1 Oct 2024 </div></div>
      
      
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    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Oct 2024, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">45 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1394-1402; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8236 </span></div>
  
  
  
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    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Oct 2024, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">45 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(10) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1394-1402; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8236 </span></div>
  
  
  
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