TY  - JOUR
AU  - Hansen, Niels
AU  - Stöcker, Winfried
AU  - Wiltfang, Jens
AU  - Bartels, Claudia
AU  - Rentzsch, Kristin
AU  - Bouter, Caroline
TI  - Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies.
JO  - Frontiers in immunology
VL  - 12
SN  - 1664-3224
CY  - Lausanne
PB  - Frontiers Media
M1  - DZNE-2022-00429
SP  - 760021
PY  - 2022
AB  - 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.
KW  - Aged
KW  - Aphasia, Primary Progressive: immunology
KW  - Autoantibodies: immunology
KW  - Autoantigens: immunology
KW  - Female
KW  - Glial Fibrillary Acidic Protein: immunology
KW  - Humans
KW  - anti-GFAP antibody (Other)
KW  - autoimmunity (Other)
KW  - frontotemporal lobar degeneration (FTLD) (Other)
KW  - immunotherapy (Other)
KW  - semantic variant of primary progressive aphasia (svPPA) (Other)
KW  - Autoantibodies (NLM Chemicals)
KW  - Autoantigens (NLM Chemicals)
KW  - GFAP protein, human (NLM Chemicals)
KW  - Glial Fibrillary Acidic Protein (NLM Chemicals)
KW  - anti-GFAP autoantibodies (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:35046935
C2  - pmc:PMC8761624
DO  - DOI:10.3389/fimmu.2021.760021
UR  - https://pub.dzne.de/record/163683
ER  -