Semantic dementia is a kind of neurodegenerative disorder, characterized by prominent semantic impairments and anterior temporal lobe atrophy. Since 2010, more studies have devoted to this rare disorder, revealing that it is more complex than we think. Clinical advances include more specific findings of semantic impairments and other higher order cognitive deficits. Neuroimaging techniques can help revealing the different brain networks affected (both structurally and functionally) in this condition. Pathological and genetic studies have also found more complex situations of semantic dementia, which might explain the huge variance existing in semantic dementia. Moreover, the current diagnosis criteria mainly focus on semantic dementia's classical prototype. We further delineated the features of three subtypes of semantic dementia based on atrophy lateralization with three severity stages. In a broader background, as a part of the continuum of neurodegenerative disorders, semantic dementia is commonly compared with other resembling conditions. Therefore, we summarized the differential diagnosis between semantic dementia and them. Finally, we introduced the challenges and achievements of its diagnosis, treatment, care and cross cultural comparison. By providing a comprehensive picture of semantic dementia on different aspects of advances, we hope to deepen the understanding of semantic dementia and promote more inspirations on both clinical and theoretical studies about it.
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http://dx.doi.org/10.1016/j.arr.2024.102375 | DOI Listing |
Handb Clin Neurol
March 2025
University School for Advanced Studies (IUSS-Pavia), Pavia, Italy; Dementia Research Center, IRCCS Mondino Foundation, Pavia, Italy. Electronic address:
Hemispheric asymmetry in pathologic involvement is frequently observed in neurodegenerative disorders (NDD) and is responsible for differences in cognitive and motor clinical manifestations in individual patients. While asymmetry is modest in typical Alzheimer disease (AD), atypical AD presentations with prominent language impairment [logopenic/phonologic variant of primary progressive aphasia (L/Phv-PPA)] are associated with prevalent involvement of the language-dominant hemisphere. Similarly, in the frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum, the semantic (Sv) and nonfluent/agrammatic (Nf/Av) variants of PPA are due to asymmetric pathology involving the language-dominant hemisphere.
View Article and Find Full Text PDFNeuron
March 2025
Department of Physiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; Brain Science Institute, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA; The Solomon H. Snyder Department of Neuroscience and Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. Electronic address:
The most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) is the repeat expansion in C9ORF72. Dipeptide repeat (DPR) proteins translated from both sense and antisense repeats, especially arginine-rich DPRs (R-DPRs), contribute to neurodegeneration. Through CRISPR interference (CRISPRi) screening in human-derived neurons, we identified receptor-type tyrosine-protein phosphatase S (PTPσ) as a strong modifier of poly-GR-mediated toxicity.
View Article and Find Full Text PDFAlzheimers Res Ther
March 2025
Department of Biomedical Engineering, University of California, Irvine, CA, USA.
Background: Nicotinamide, a form of B3 vitamin, is an NAD precursor that reduces pTau levels via histone deacetylase inhibition in murine models of Alzheimer's disease (AD). A recent phase 2a randomized placebo-controlled trial tested high-dose oral nicotinamide for the treatment of early AD. While nicotinamide demonstrated good safety and tolerability, it did not significantly lower CSF pTau, the primary biomarker endpoint of the study.
View Article and Find Full Text PDFJ Neurol Sci
March 2025
The University of Sydney, School of Psychology, Sydney, NSW 2006, Australia; The University of Sydney, Brain and Mind Centre, Sydney, NSW 2050, Australia. Electronic address:
Background: The effect of cultural differences in neurodegenerative diseases is not well understood. We aimed to investigate the cognitive profiles of English- and Spanish-speaking individuals with primary progressive aphasia (PPA).
Methodology: A comparative cross-sectional study was conducted with 461 participants: 215 participants from Australia and 246 from Spain.
Clin Gerontol
March 2025
Department of Psychological Sciences, Kent State University, Ohio, USA.
Objectives: Dementia caregiving often results in caregiver burden, but self-mastery may buffer against burden's negative impact. This work explores the link between these variables, examining potential moderators, through systematic review and meta-analysis.
Methods: A PubMed, PsychInfo, Scopus, and Medline search in April 2024 resulted in 25 eligible studies.
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