{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T14:44:22Z","timestamp":1777387462611,"version":"3.51.4"},"reference-count":35,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,1,3]],"date-time":"2022-01-03T00:00:00Z","timestamp":1641168000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003385","name":"Georg-August-Universit\u00e4t G\u00f6ttingen","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003385","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Frontotemporal lobar degeneration is a heterogeneous disorder entailing a semantic variant of primary progressive aphasia (svPPA). A subtype of frontotemporal dementia associated with glutamate receptor subunit 3 (GluA3) antibody of the \u03b1-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) was recently identified. Here, we describe the novelty of a svPPA associated with anti-glial fibrillary acid protein (GFAP) antibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>To diagnose this 68-year-old woman we conducted a clinical examination, neuropsychological testing, CSF analysis, MRI and 18F-fluorodeoxyglucose (18F-FDG) Positron Emission Tomography (PET)\/computed tomography (CT) imaging.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The clinical phenotype corresponds to a svPPA based on impaired confrontation naming and single-word comprehension. In addition, we observed spared speech production, impaired object knowledge, and surface dyslexia - further supporting the diagnosis of svPPA. Additional characteristic imaging features such as anterior temporal hypometabolism in 18F-FDG PET\/CT confirmed patient\u2019s svPPA diagnosis. CSF analysis revealed signs of axonal degeneration, as both tau and phosphorylated tau proteins exceeded normal levels. Her serum showed anti-GFAP autoantibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We diagnosed a svPPA in this patient and report an association between serum anti-GFAP antibodies and svPPA never reported in the literature so far, thereby expanding the clinical spectrum of svPPA and anti-GFAP-antibody related disease. Further research is needed to elucidate the underlying immunopathology of this disease entity to ultimately improve treatment.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2021.760021","type":"journal-article","created":{"date-parts":[[2022,1,3]],"date-time":"2022-01-03T05:17:19Z","timestamp":1641187039000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies"],"prefix":"10.3389","volume":"12","author":[{"given":"Niels","family":"Hansen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Winfried","family":"St\u00f6cker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jens","family":"Wiltfang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Bartels","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kristin","family":"Rentzsch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Bouter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2022,1,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"6723","DOI":"10.1038\/s41598-017-06117-y","article-title":"Anti-AMPA GluA3 Antibodies in Frontotemporal Dementia: A New Molecular Target","volume":"7","author":"Borroni","year":"2017","journal-title":"Sci Rep"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2019.10.015","article-title":"Anti-GluA3 Antibodies in Frontotemporal Dementia: Effects on Glutamatergic Neurotransmission and Synaptic Failure","volume":"86","author":"Palese","year":"2020","journal-title":"Neurobiol Aging"},{"key":"B3","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/0165-5728(94)00180-v","article-title":"Serum Anti-GFAP and Anti-S100 Autoantibodies in Brain Aging, Alzheimer\u2019s Disease and Vascular Dementia","volume":"57","author":"Mecocci","year":"1995","journal-title":"J Neuroimmunol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1989.tb03926.x","article-title":"Enzyme-Linked Immunosorbent Assay for Human Autoantibody to Glial Fibrillary Acidic Protein: Higher Titer of the Antibody Is Detected in Serum of Patients With Alzheimer's Disease","volume":"80","author":"Tanaka","year":"1989","journal-title":"Acta Neurol Scand"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31821103e6","article-title":"Classification of Primary Progressive Aphasia and Its Variants","volume":"76","author":"Gorno-Tempini","year":"2011","journal-title":"Neurology"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1590\/1980-57642021dn15-030014","article-title":"Heterogeneity of Repetition Abilities in Logopenic Variant Primary Progressive Aphasia","volume":"15","author":"Macoir","year":"2021","journal-title":"Dement Neuropsychol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.cortex.2021.09.015","article-title":"Task Performance to Discriminate Among Variants of Primary Progressive Aphasia","volume":"145","author":"Stockbridge","year":"2021","journal-title":"Cortex"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1093\/braincomms\/fcab210","article-title":"A Category-Selective Semantic Memory Deficit for Animate Objects in Semantic Variant Primary Progressive Aphasia","volume":"3","author":"Henderson","year":"2021","journal-title":"Brain Commun"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.02802","article-title":"Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature","volume":"9","author":"Shan","year":"2018","journal-title":"Front Immunol"},{"key":"B10","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/s12883-021-02070-6","article-title":"Uncommon Manifestations of a Rare Disease: A Case of Autoimmune GFAP Astrocytopathy","volume":"21","author":"Di","year":"2021","journal-title":"BMC Neurol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000013087","article-title":"Glial Fibrillary Acidic Protein Autoimmunity: A French Cohort Study","author":"Dumonceau","year":"2021","journal-title":"Neurology"},{"key":"B12","doi-asserted-by":"publisher","first-page":"e000142","DOI":"10.1136\/bmjno-2021-000142","article-title":"Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presented as Ataxia, Myoclonus and Bulbar Syndrome: A Case Report and Review of the Literature","volume":"3","author":"Novo","year":"2021","journal-title":"BMJ Neurol Open"},{"key":"B13","doi-asserted-by":"publisher","first-page":"e17588","DOI":"10.7759\/cureus.17588","article-title":"Subacute Blindness Revealing an Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy","volume":"13","author":"Mabrouki","year":"2021","journal-title":"Cureus"},{"key":"B14","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1159\/000505730","article-title":"Overlapping Clinical Syndromes in Patients With Glial Fibrillary Acidic Protein IgG","volume":"27","author":"Lin","year":"2020","journal-title":"Neuroimmunomodulation"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1186\/s12974-021-02247-3","article-title":"Differential Chemokine Alteration in the Variants of Primary Progressive Aphasia-A Role for Neuroinflammation","volume":"18","author":"Sogorb-Esteve","year":"2021","journal-title":"J Neuroinflamm"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1111\/ene.14778","article-title":"CD8 + T-Cell Predominance in Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy","volume":"28","author":"Yuan","year":"2021","journal-title":"Eur J Neurol"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1002\/pmic.200900066","article-title":"Autoantibody to Glial Fibrillary Acidic Protein in the Sera of Cattle With Bovine Spongiform Encephalopathy","volume":"9","author":"Nomura","year":"2009","journal-title":"Proteomics"},{"key":"B18","doi-asserted-by":"publisher","first-page":"e92698","DOI":"10.1371\/journal.pone.0092698","article-title":"Human Traumatic Brain Injury Induces Autoantibody Response Against Glial Fibrillary Acidic Protein and Its Breakdown Products","volume":"9","author":"Zhang","year":"2014","journal-title":"PloS One"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2017-316583","article-title":"Clinical and Immunological Characteristics of the Spectrum of GFAP Autoimmunity: A Case Series of 22 Patients","volume":"89","author":"Iorio","year":"2017","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1111\/ene.14244","article-title":"Black Holes and High Levels of Neurofilaments in Glial Fibrillary Acidic Protein - Astrocytopathy: A Case Report","volume":"27","author":"Papa","year":"2020","journal-title":"Eur J Neurol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1111\/ene.14919","article-title":"Disease-Specific Metabolic Imaging Marker for Diagnosis and Progression Evaluation of Semantic Variant Primary Progressive Aphasia","volume":"28","author":"Lu","year":"2021","journal-title":"Eur J Neurol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1002\/alz.12323","article-title":"Quantifying Progression in Primary Progressive Aphasia With Structural Neuroimaging","volume":"17","author":"Lombardi","year":"2021","journal-title":"Alzheimers Dement"},{"key":"B23","doi-asserted-by":"publisher","first-page":"e63905","DOI":"10.7554\/eLife.63905","article-title":"Neural Dynamics of Semantic Categorization in Semantic Variant of Primary Progressive Aphasia","volume":"10","author":"Borghesani","year":"2021","journal-title":"Elife"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1159\/000358233","article-title":"Evaluation of the New Consensus Criteria for the Diagnosis of Primary Progressive Aphasia Using Fluorodeoxyglucose Positron Emission Tomography","volume":"38","author":"Matias-Guiu","year":"2014","journal-title":"Dement Geriatr Cognit Disord"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1159\/000504938","article-title":"Qualitative and Quantitative Analyses of Brain 18Fluoro-Deoxy-Glucose Positron Emission Tomography in Primary Progressive Aphasia","volume":"48","author":"Nuvoli","year":"2019","journal-title":"Dement Geriatr Cogn Disord"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1007\/s00259-018-4034-z","article-title":"EANM-EAN Task Force for the Prescription of FDG-PET for Dementing Neurodegenerative Disorders. Diagnostic Utility of FDG-PET in the Differential Diagnosis Between Different Forms of Primary Progressive Aphasia","volume":"45","author":"Bouwman","year":"2018","journal-title":"Eur J Nucl Med Mol Imaging"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A5126","article-title":"Can MRI Visual Assessment Differentiate the Variants of Primary-Progressive Aphasia","volume":"38","author":"Sajjadi","year":"2017","journal-title":"AJNR Am J Neuroradiol"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-021-10484-y","article-title":"Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: Case Report of a Treatable Cause of Rapidly Progressive Dementia","volume":"268","author":"Toledano-Ill\u00e1n","year":"2021","journal-title":"J Neurol"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.4065\/mcp.2010.0326","article-title":"Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response","volume":"85","author":"Flanagan","year":"2010","journal-title":"Mayo Clin Proc"},{"key":"B30","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1017\/S0033291720002895","article-title":"Autoantibody-Associated Psychiatric Syndromes: A Systematic Literature Review Resulting in 145 Cases","volume":"7","author":"Endres","year":"2020","journal-title":"Psychol Med"},{"key":"B31","doi-asserted-by":"publisher","first-page":"100154","DOI":"10.1016\/j.bbih.2020.100154","article-title":"Autoantibody-Associated Psychiatric Symptoms and Syndromes in Adults: A Narrative Review and Proposed Diagnostic Approach","volume":"9","author":"Hansen","year":"2020","journal-title":"Brain Behav Immun \u2013 Health"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.3389\/fpsyt.2020.00576","article-title":"Figural Memory Impairment in Conjunction With Neuropsychiatric Symptoms in IgLON5 Antibody-Associated Autoimmune Encephalitis","volume":"11","author":"Hansen","year":"2020","journal-title":"Front Psych"},{"key":"B33","doi-asserted-by":"publisher","first-page":"e301","DOI":"10.1212\/NXI.0000000000000301","article-title":"Increased Prevalence of Autoimmune Disease Within C9 and FTD\/MND Cohorts: Completing the Picture","volume":"3","author":"Miller","year":"2016","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"key":"B34","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1038\/s41586-020-2625-x","article-title":"C9orf72 in Myeloid Cells Suppresses STING-Induced Inflammation","volume":"585","author":"McCauley","year":"2020","journal-title":"Nature"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1038\/s41582-019-0231-z","article-title":"Neuroinflammation in Frontotemporal Dementia","volume":"15","author":"Bright","year":"2019","journal-title":"Nat Rev Neurol"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2021.760021\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,1,3]],"date-time":"2022-01-03T05:17:22Z","timestamp":1641187042000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2021.760021\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,3]]},"references-count":35,"alternative-id":["10.3389\/fimmu.2021.760021"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2021.760021","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,3]]},"article-number":"760021"}}