Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies
BackgroundFrontotemporal 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...
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Frontiers Media S.A.
2022-01-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2021.760021/full |
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| author | Niels Hansen Winfried Stöcker Jens Wiltfang Jens Wiltfang Jens Wiltfang Claudia Bartels Kristin Rentzsch Caroline Bouter |
| author_facet | Niels Hansen Winfried Stöcker Jens Wiltfang Jens Wiltfang Jens Wiltfang Claudia Bartels Kristin Rentzsch Caroline Bouter |
| author_sort | Niels Hansen |
| collection | DOAJ |
| description | BackgroundFrontotemporal 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.MethodsTo 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.ResultsThe 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.ConclusionWe 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. |
| first_indexed | 2024-12-17T23:36:47Z |
| format | Article |
| id | doaj.art-41eac78fb65d4262a993512616ea4887 |
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| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-17T23:36:47Z |
| publishDate | 2022-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-41eac78fb65d4262a993512616ea48872022-12-21T21:28:32ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-01-011210.3389/fimmu.2021.760021760021Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein AutoantibodiesNiels Hansen0Winfried Stöcker1Jens Wiltfang2Jens Wiltfang3Jens Wiltfang4Claudia Bartels5Kristin Rentzsch6Caroline Bouter7Department of Psychiatry and Psychotherapy, University Medical Center Goettingen, Goettingen, GermanyEuroimmun Reference Laboratory, Luebeck, GermanyDepartment of Psychiatry and Psychotherapy, University Medical Center Goettingen, Goettingen, GermanyGerman Center for Neurodegenerative Diseases (DZNE), Goettingen, GermanyNeurosciences and Signaling Group, Institute of Biomedicine (iBiMED), Department of Medical Sciences, University of Aveiro, Aveiro, PortugalDepartment of Psychiatry and Psychotherapy, University Medical Center Goettingen, Goettingen, GermanyEuroimmun Reference Laboratory, Luebeck, GermanyDepartment of Nuclear Medicine, University Medical Center Göttingen, Goettingen, GermanyBackgroundFrontotemporal 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.MethodsTo 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.ResultsThe 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.ConclusionWe 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.https://www.frontiersin.org/articles/10.3389/fimmu.2021.760021/fullfrontotemporal lobar degeneration (FTLD)semantic variant of primary progressive aphasia (svPPA)autoimmunityanti-GFAP antibodyimmunotherapy |
| spellingShingle | Niels Hansen Winfried Stöcker Jens Wiltfang Jens Wiltfang Jens Wiltfang Claudia Bartels Kristin Rentzsch Caroline Bouter Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies Frontiers in Immunology frontotemporal lobar degeneration (FTLD) semantic variant of primary progressive aphasia (svPPA) autoimmunity anti-GFAP antibody immunotherapy |
| title | Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies |
| title_full | Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies |
| title_fullStr | Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies |
| title_full_unstemmed | Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies |
| title_short | Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies |
| title_sort | case report semantic variant of primary progressive aphasia associated with anti glial fibrillary acid protein autoantibodies |
| topic | frontotemporal lobar degeneration (FTLD) semantic variant of primary progressive aphasia (svPPA) autoimmunity anti-GFAP antibody immunotherapy |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2021.760021/full |
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