Today, frontotemporal dementia (FTD) remains one of the most common forms of early-onset dementia, that is, before the age of 65, thus posing several diagnostic challenges to clinicians since symptoms are often mistaken for psychiatric or neurological diseases causing a delay in correct diagnosis, and the majority of patients with FTD present with symptoms at ages between 50 and 60. Genetic components are established risk factors for FTD, but the influence of lifestyle, comorbidity, and environmental factors on the risk of FTD is still unclear. Approximately 40% of individuals with FTD have a family history of dementia but less than 10% have a clear autosomal dominant pattern of inheritance. Lack of insight is often an early clue to FTD. A tailored treatment option at an early phase can mitigate suffering and improve patients' and caregivers' quality of life.
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http://dx.doi.org/10.1177/0891988716654986 | DOI Listing |
World J Clin Cases
January 2025
Department of Neurology, Guizhou Medical University, Guiyang 550004, Guizhou Province, China.
Dementia is a group of diseases, including Alzheimer's disease (AD), vascular dementia, Lewy body dementia, frontotemporal dementia, Parkinson's disease dementia, metabolic dementia and toxic dementia. The treatment of dementia mainly includes symptomatic treatment by controlling the primary disease and accompanying symptoms, nutritional support therapy for repairing nerve cells, psychological auxiliary treatment, and treatment that improves cognitive function through drugs. Among them, drug therapy to improve cognitive function is important.
View Article and Find Full Text PDFUnlabelled: The neurodegenerative disorder Frontotemporal Dementia (FTD) can be caused by a repeat expansion (GGGGCC; G4C2) in C9orf72. The function of wild-type C9orf72 and the mechanism by which the C9orf72-G4C2 mutation causes FTD, however, remain unresolved. Diverse disease models including human brain samples and differentiated neurons from patient-derived induced pluripotent stem cells (iPSCs) identified some hallmarks associated with FTD, but these models have limitations, including biopsies capturing only a static snapshot of dynamic processes and differentiated neurons being labor-intensive, costly, and post-mitotic.
View Article and Find Full Text PDFNat Commun
January 2025
Biogen Inc, Cambridge, MA, USA.
Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder characterized by physical, cognitive, and behavioral impairments. The PSP Rating Scale (PSPRS) is a widely used and validated, clinical scale to monitor disease progression. Here we show the modification of PSPRS to improve clinical meaningfulness and sensitivity.
View Article and Find Full Text PDFFEBS J
January 2025
Department of Biochemistry, Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Biomolecular condensates are dynamic membraneless compartments that regulate a myriad of cellular functions. A particular type of physiological condensate called stress granules (SGs) has gained increasing interest due to its role in the cellular stress response and various diseases. SGs, composed of several hundred RNA-binding proteins, form transiently in response to stress to protect mRNAs from translation and disassemble when the stress subsides.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Protein Science, SciLifeLab, KTH Royal Institute of Technology, Stockholm, Sweden.
Accurate diagnosis and monitoring of neurodegenerative diseases require reliable biomarkers. Cerebrospinal fluid (CSF) proteins are promising candidates for reflecting brain pathology; however, their diagnostic utility may be compromised by natural variability between individuals, weakening their association with disease. Here, we measured the levels of 69 pre-selected proteins in cerebrospinal fluid using antibody-based suspension bead array technology in a multi-disease cohort of 499 individuals with neurodegenerative disorders including Alzheimer's disease (AD), behavioral variant frontotemporal dementia, primary progressive aphasias, amyotrophic lateral sclerosis (ALS), corticobasal syndrome, primary supranuclear palsy, along with healthy controls.
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