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@ARTICLE{Hansen:163683,
      author       = {Hansen, Niels and Stöcker, Winfried and Wiltfang, Jens and
                      Bartels, Claudia and Rentzsch, Kristin and Bouter, Caroline},
      title        = {{C}ase {R}eport: {S}emantic {V}ariant of {P}rimary
                      {P}rogressive {A}phasia {A}ssociated {W}ith {A}nti-{G}lial
                      {F}ibrillary {A}cid {P}rotein {A}utoantibodies.},
      journal      = {Frontiers in immunology},
      volume       = {12},
      issn         = {1664-3224},
      address      = {Lausanne},
      publisher    = {Frontiers Media},
      reportid     = {DZNE-2022-00429},
      pages        = {760021},
      year         = {2022},
      abstract     = {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.},
      keywords     = {Aged / Aphasia, Primary Progressive: immunology /
                      Autoantibodies: immunology / Autoantigens: immunology /
                      Female / Glial Fibrillary Acidic Protein: immunology /
                      Humans / anti-GFAP antibody (Other) / autoimmunity (Other) /
                      frontotemporal lobar degeneration (FTLD) (Other) /
                      immunotherapy (Other) / semantic variant of primary
                      progressive aphasia (svPPA) (Other) / Autoantibodies (NLM
                      Chemicals) / Autoantigens (NLM Chemicals) / GFAP protein,
                      human (NLM Chemicals) / Glial Fibrillary Acidic Protein (NLM
                      Chemicals) / anti-GFAP autoantibodies (NLM Chemicals)},
      cin          = {AG Wiltfang},
      ddc          = {610},
      cid          = {I:(DE-2719)1410006},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:35046935},
      pmc          = {pmc:PMC8761624},
      doi          = {10.3389/fimmu.2021.760021},
      url          = {https://pub.dzne.de/record/163683},
}