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Introduction: Frontotemporal dementia (FTD) with right anterior temporal lobe (rATL) predominance lacks universally agreed-upon diagnostic criteria. This study validated the Amsterdam diagnostic tree (ADT) for right temporal variant FTD (rtvFTD) and the diagnostic criteria for semantic behavioral variant FTD (sbvFTD), examining clinical, behavioral, and imaging differences.
Methods: The study included 138 patients with behavioral variant FTD and 87 with semantic variant primary progressive aphasia who had 3D T1-weighted magnetic resonance imaging scans. The participants were grouped into rtvFTD and sbvFTD by respective imaging criteria.
Results: We identified 42 rtvFTD and 20 sbvFTD patients. ADT showed 81% sensitivity and 29% specificity, whereas sbvFTD criteria had 45% sensitivity and 55% specificity.
Discussion: The unfavorable validation of both diagnostic criteria in Korean FTD patients may result from socio-cultural differences, the lack of standardized tools for assessing abnormal behaviors, and the retrospective nature of the study. The perspectives on rATL also differed between the two studies.
Highlights: Frontotemporal dementia (FTD) with right anterior temporal lobe (rATL) predominance is a controversial FTD syndrome, also referred to as right temporal variant FTD (rtvFTD), or right predominant semantic variant primary progressive aphasia. Two clinical criteria for rATL have been proposed: One is the Amsterdam diagnostic tree for rtvFTD and the other is for semantic behavioral variant FTD (sbvFTD). Our validation study suggested the need for standardized tools and highlighted theoretical distinctions between rtvFTD and sbvFTD.
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http://dx.doi.org/10.1002/alz.70443 | DOI Listing |
FEBS J
September 2025
Translational Research Institute, Academic Health System, Hamad Medical Corporation, Doha, Qatar.
TDP-linked proteinopathies, including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and limbic-predominant age-related TDP-43 encephalopathy (LATE), are characterised by pathogenic deposits containing transactive response DNA-binding protein 43 (TDP-43) in the brain and spinal cord of patients. These hallmark pathological features are associated with widespread neuronal dysfunction and progressive neurodegeneration. TDP-43's role as an essential RNA/DNA-binding protein in RNA metabolism and gene expression regulation is clear, but deciphering the intricate pathophysiological mechanisms underpinning TDP-43-mediated neurodegeneration is paramount for developing effective therapies and novel diagnostic tools for early detection before frank neuronal loss occurs.
View Article and Find Full Text PDFBMC Neurol
August 2025
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, 560029, India.
Background/objectives: Frontotemporal Dementia (FTD) is one of the common causes of early-onset degenerative dementia and is a clinically and pathologically heterogeneous group of neurodegenerative disorders. Globally, Microtubule Associated Protein Tau (MAPT), progranulin (GRN), and Chromosome 9 open reading frame 72(C9orf72) are the common FTD genetic mutations. However, they have not been reported from India, and only one progranulin (PGRN) mutation has been reported so far.
View Article and Find Full Text PDFAmyotroph Lateral Scler Frontotemporal Degener
August 2025
Department of Neurology, Fundación Jiménez Díaz, Madrid, Spain.
To characterize the phenotypic spectrum of patients with frontotemporal dementia (FTD) carrying the P392L mutation. We describe the clinical phenotype of three well-characterized probands carrying the P392L mutation, and review ten previously published FTD cases with the mutation. All three cases were male with a presenile age of onset (52 or 64 years).
View Article and Find Full Text PDFJ Neurol
August 2025
Department of Pathophysiology and Transplantation, "Dino Ferrari" Center, Università Degli Studi Di Milano, Milan, Italy.
Background: Primary Lateral Sclerosis (PLS) is a rare, adult-onset neurodegenerative disease that predominantly affects upper motor neurons. Despite being considered mostly sporadic, familial cases and rare genetic variants in genes associated with amyotrophic lateral sclerosis, hereditary spastic paraplegia and other neurological disorders have been reported in some PLS cases. Due to its rare prevalence among general population, large genetic studies of PLS are lacking.
View Article and Find Full Text PDFEpilepsy Res
August 2025
Department of Neurology, University of Virginia School of Medicine, Charlottesville, VA, United States. Electronic address:
Objectives: Comorbid seizures occur in 2-11 % of frontotemporal dementia(FTD). Despite the high risk for seizures, the risk factors, clinical characteristics, and seizure outcomes in FTD patients with comorbid seizures remain understudied.
Methods: All patients who presented to our hospital from 5/1/2011-4/30/2024 with a clinical diagnosis of FTD were included and subclassified into behavioral-variant FTD(bvFTD), sematic-variant-primary-progressive-aphasia(svPPA), or non-fluent-primary-progressive-aphasia(nfPPA).