%0 Journal Article
%A Hansen, Niels
%A Stöcker, Winfried
%A Wiltfang, Jens
%A Bartels, Claudia
%A Rentzsch, Kristin
%A Bouter, Caroline
%T Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies.
%J Frontiers in immunology
%V 12
%@ 1664-3224
%C Lausanne
%I Frontiers Media
%M DZNE-2022-00429
%P 760021
%D 2022
%X 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 α-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.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.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's 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.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.
%K Aged
%K Aphasia, Primary Progressive: immunology
%K Autoantibodies: immunology
%K Autoantigens: immunology
%K Female
%K Glial Fibrillary Acidic Protein: immunology
%K Humans
%K anti-GFAP antibody (Other)
%K autoimmunity (Other)
%K frontotemporal lobar degeneration (FTLD) (Other)
%K immunotherapy (Other)
%K semantic variant of primary progressive aphasia (svPPA) (Other)
%K Autoantibodies (NLM Chemicals)
%K Autoantigens (NLM Chemicals)
%K GFAP protein, human (NLM Chemicals)
%K Glial Fibrillary Acidic Protein (NLM Chemicals)
%K anti-GFAP autoantibodies (NLM Chemicals)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:35046935
%2 pmc:PMC8761624
%R 10.3389/fimmu.2021.760021
%U https://pub.dzne.de/record/163683