Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies
| Autor: | Hansen, Niels, Stöcker, Winfried, Wiltfang, Jens, Bartels, Claudia, Rentzsch, Kristin, Bouter, Caroline |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
immunology [Aphasia
Primary Progressive] anti-GFAP antibody frontotemporal lobar degeneration (FTLD) GFAP protein human immunology [Autoantigens] Immunology autoimmunity Case Report anti-GFAP autoantibodies Autoantigens semantic variant of primary progressive aphasia (svPPA) immunology [Glial Fibrillary Acidic Protein] Glial Fibrillary Acidic Protein immunology [Autoantibodies] Humans Female ddc:610 immunotherapy Aged Autoantibodies |
| Zdroj: | Frontiers in immunology 12, 760021 (2022). doi:10.3389/fimmu.2021.760021 Frontiers in Immunology |
| DOI: | 10.3389/fimmu.2021.760021 |
| Popis: | Background 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. Methods 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. Results 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. Conclusion 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. |
| Databáze: | OpenAIRE |
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