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000163683 037__ $$aDZNE-2022-00429
000163683 041__ $$aEnglish
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000163683 1001_ $$aHansen, Niels$$b0
000163683 245__ $$aCase Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies.
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000163683 520__ $$aFrontotemporal 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.
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000163683 650_7 $$2Other$$aanti-GFAP antibody
000163683 650_7 $$2Other$$aautoimmunity
000163683 650_7 $$2Other$$afrontotemporal lobar degeneration (FTLD)
000163683 650_7 $$2Other$$aimmunotherapy
000163683 650_7 $$2Other$$asemantic variant of primary progressive aphasia (svPPA)
000163683 650_7 $$2NLM Chemicals$$aAutoantibodies
000163683 650_7 $$2NLM Chemicals$$aAutoantigens
000163683 650_7 $$2NLM Chemicals$$aGFAP protein, human
000163683 650_7 $$2NLM Chemicals$$aGlial Fibrillary Acidic Protein
000163683 650_7 $$2NLM Chemicals$$aanti-GFAP autoantibodies
000163683 650_2 $$2MeSH$$aAged
000163683 650_2 $$2MeSH$$aAphasia, Primary Progressive: immunology
000163683 650_2 $$2MeSH$$aAutoantibodies: immunology
000163683 650_2 $$2MeSH$$aAutoantigens: immunology
000163683 650_2 $$2MeSH$$aFemale
000163683 650_2 $$2MeSH$$aGlial Fibrillary Acidic Protein: immunology
000163683 650_2 $$2MeSH$$aHumans
000163683 7001_ $$aStöcker, Winfried$$b1
000163683 7001_ $$0P:(DE-2719)2811317$$aWiltfang, Jens$$b2$$udzne
000163683 7001_ $$0P:(DE-2719)9000444$$aBartels, Claudia$$b3$$udzne
000163683 7001_ $$0P:(DE-2719)2501892$$aRentzsch, Kristin$$b4$$udzne
000163683 7001_ $$aBouter, Caroline$$b5
000163683 773__ $$0PERI:(DE-600)2606827-8$$a10.3389/fimmu.2021.760021$$gVol. 12, p. 760021$$p760021$$tFrontiers in immunology$$v12$$x1664-3224$$y2022
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