Journal Article

DZNE-2022-00429

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Case Report: Semantic Variant of Primary Progressive Aphasia Associated With Anti-Glial Fibrillary Acid Protein Autoantibodies.

Hansen, N. ; Stöcker, W. ; Wiltfang, J.DZNE* ; Bartels, C.Extern* ; Rentzsch, K.Extern* ; Bouter, C.

2022
Frontiers Media Lausanne

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Please use a persistent id in citations: doi:10.3389/fimmu.2021.760021

Abstract: 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.

Keyword(s): Aged (MeSH) ; Aphasia, Primary Progressive: immunology (MeSH) ; Autoantibodies: immunology (MeSH) ; Autoantigens: immunology (MeSH) ; Female (MeSH) ; Glial Fibrillary Acidic Protein: immunology (MeSH) ; Humans (MeSH) ; anti-GFAP antibody ; autoimmunity ; frontotemporal lobar degeneration (FTLD) ; immunotherapy ; semantic variant of primary progressive aphasia (svPPA) ; Autoantibodies ; Autoantigens ; GFAP protein, human ; Glial Fibrillary Acidic Protein ; anti-GFAP autoantibodies

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Contributing Institute(s):

1. Alzheimer Biomarker and Clinical Study Group (AG Wiltfang)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Appears in the scientific report 2022

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Database coverage:
; ; ; ; Article Processing Charges ; Clarivate Analytics Master Journal List ; DOAJ Seal ; Essential Science Indicators ; Fees ; IF >= 5 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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