Home > Publications Database > Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies. > print

001     163683
005     20230915090531.0
024 7 _ |a 10.3389/fimmu.2021.760021
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037 _ _ |a DZNE-2022-00429
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Hansen, Niels
|b 0
245 _ _ |a Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies.
260 _ _ |a Lausanne
|c 2022
|b Frontiers Media
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520 _ _ |a 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.
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650 _ 7 |a anti-GFAP antibody
|2 Other
650 _ 7 |a autoimmunity
|2 Other
650 _ 7 |a frontotemporal lobar degeneration (FTLD)
|2 Other
650 _ 7 |a immunotherapy
|2 Other
650 _ 7 |a semantic variant of primary progressive aphasia (svPPA)
|2 Other
650 _ 7 |a Autoantibodies
|2 NLM Chemicals
650 _ 7 |a Autoantigens
|2 NLM Chemicals
650 _ 7 |a GFAP protein, human
|2 NLM Chemicals
650 _ 7 |a Glial Fibrillary Acidic Protein
|2 NLM Chemicals
650 _ 7 |a anti-GFAP autoantibodies
|2 NLM Chemicals
650 _ 2 |a Aged
|2 MeSH
650 _ 2 |a Aphasia, Primary Progressive: immunology
|2 MeSH
650 _ 2 |a Autoantibodies: immunology
|2 MeSH
650 _ 2 |a Autoantigens: immunology
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Glial Fibrillary Acidic Protein: immunology
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
700 1 _ |a Stöcker, Winfried
|b 1
700 1 _ |a Wiltfang, Jens
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700 1 _ |a Bartels, Claudia
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700 1 _ |a Rentzsch, Kristin
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700 1 _ |a Bouter, Caroline
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773 _ _ |a 10.3389/fimmu.2021.760021
|g Vol. 12, p. 760021
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|t Frontiers in immunology
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|y 2022
|x 1664-3224
856 4 _ |y OpenAccess
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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