{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T17:44:02Z","timestamp":1792172642931,"version":"4.4.0"},"reference-count":37,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,2,23]],"date-time":"2017-02-23T00:00:00Z","timestamp":1487808000000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100014535","name":"Mayo Clinic Center for Individualized Medicine","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100014535","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Department of Laboratory Medicine and Pathology, Mayo Clinic"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2017,2]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>A novel autoimmune central nervous system (CNS) disorder with glial fibrillary acidic protein (GFAP)\u2010IgG as biomarker was recently characterized. Here, 102 patients with GFAP\u2010IgG positivity are described.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The 102 included patients had: (1) serum, cerebrospinal fluid (CSF), or both that yielded a characteristic astrocytic pattern of mouse tissue immunostaining; (2) confirmation of IgG reactive with specific GFAP isoforms (\u03b1, \u025b, or \u03ba) by cell\u2010based assays; and (3) clinical data available. Control specimens (n = 865) were evaluated by tissue (n = 542) and cell\u2010based (n = 323) assays.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Median symptom onset age was 44 years (range = 8\u2013103), and 54% were women. The predominant phenotype (83 patients; 81%) was inflammation of meninges, brain, spinal cord, or all 3 (meningoencephalomyelitis). Among patients, highest specificity for those phenotypes was observed for CSF testing (94%), and highest sensitivity was for the GFAP\u03b1 isoform (100%). Rare GFAP\u2010IgG positivity was encountered in serum controls by tissue\u2010based assay (0.5%) or cell\u2010based assay (1.5%), and in CSF controls by cell\u2010based assay (0.9%). Among patients, striking perivascular radial enhancement was found on brain magnetic resonance imaging in 53%. Although cases frequently mimicked vasculitis, angiography was uniformly negative, and spinal imaging frequently demonstrated longitudinally extensive myelitic lesions. Diverse neoplasms encountered were found prospectively in 22%. Ovarian teratoma was most common and was predicted best when both N\u2010methyl\u2010D\u2010aspartate receptor\u2013IgG and aquaporin\u20104\u2013IgG coexisted (71%). Six patients with prolonged follow\u2010up had brisk corticosteroid response, but required additional immunosuppression to overcome steroid dependency.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>GFAP\u03b1\u2010IgG, when detected in CSF, is highly specific for an immunotherapy\u2010responsive autoimmune CNS disorder, sometimes with paraneoplastic cause. Ann Neurol 2017;81:298\u2013309<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ana.24881","type":"journal-article","created":{"date-parts":[[2017,1,24]],"date-time":"2017-01-24T22:33:39Z","timestamp":1485297219000},"page":"298-309","source":"Crossref","is-referenced-by-count":538,"title":["Glial fibrillary acidic protein immunoglobulin\n                    <scp>G<\/scp>\n                    as biomarker of autoimmune astrocytopathy: Analysis of 102 patients"],"prefix":"10.1002","volume":"81","author":[{"given":"Eoin P.","family":"Flanagan","sequence":"first","affiliation":[{"name":"Department of Neurology, College of Medicine Mayo Clinic Rochester MN"},{"name":"Department of Laboratory Medicine and Pathology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon R.","family":"Hinson","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanda A.","family":"Lennon","sequence":"additional","affiliation":[{"name":"Department of Neurology, College of Medicine Mayo Clinic Rochester MN"},{"name":"Department of Laboratory Medicine and Pathology, College of Medicine Mayo Clinic Rochester MN"},{"name":"Department of Immunology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boyan","family":"Fang","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allen J.","family":"Aksamit","sequence":"additional","affiliation":[{"name":"Department of Neurology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. Pearse","family":"Morris","sequence":"additional","affiliation":[{"name":"Department of Radiology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eati","family":"Basal","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josephe A.","family":"Honorat","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nora B.","family":"Alfugham","sequence":"additional","affiliation":[{"name":"Department of Neurology, College of Medicine Mayo Clinic Rochester MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny J.","family":"Linnoila","sequence":"additional","affiliation":[{"name":"Department of Neurology, College 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