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Decision Making Cognition in Primary Progressive Aphasia
Published 2012-01-01“…We sought to investigate the decision making profile of Primary Progressive Aphasia (PPA) by assessing patients diagnosed with this disease (n = 10), patients diagnosed with behavioral variant frontotemporal dementia (bvFTD, n = 35), and matched controls (n = 14) using the Iowa Gambling Task, a widely used test that mimics real-life decision making. …”
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22
Antibody-mediated degradation of 4R-tau restores mitochondrial membrane polarization in human induced pluripotent stem cell-derived neurons with the MAPT 10+16 mutation
Published 2024-12-01“…An imbalance causing excess 4 R tau is associated with diseases such as progressive supranuclear palsy and frontotemporal dementia. The mechanisms by which increased 4 R results in neuronal dysfunction and neurodegeneration are not fully understood, and progress has been limited partly by a lack of suitable tools to investigate tau isoform imbalance. …”
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23
Analyzing the potential of neuronal pentraxin 2 as a biomarker in neurological disorders: A literature review
Published 2024-12-01“…Furthermore, NP2's involvement in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) emphasizes its broad impact on neuronal health. …”
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24
Peripheral proteinopathy in neurodegenerative diseases
Published 2025-01-01“…Abstract Proteinopathies in neurology typically refer to pathological changes in proteins associated with neurological diseases, such as the aggregation of amyloid β and Tau in Alzheimer’s disease, α-synuclein in Parkinson’s disease and multiple system atrophy, and TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal dementia. Interestingly, these proteins are also commonly found in peripheral tissues, raising important questions about their roles in neurological disorders. …”
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25
Advanced Magnetic Resonance Imaging for Early Diagnosis and Monitoring of Movement Disorders
Published 2025-01-01“…These techniques enable earlier and more accurate differentiation of movement disorders, including Parkinson’s disease, progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, Lewy body and frontotemporal dementia, Huntington’s disease, and dystonia. …”
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26
Mutations in human prion-like domains: pathogenic but not always amyloidogenic
Published 2024-12-01“…To date, over 60 mutations targeting the LCDs of hnRNPs have been identified and associated with a spectrum of neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and Alzheimer’s disease (AD). …”
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27
Oligonucleotide-Based Therapy for FTD/ALS Caused by the C9orf72 Repeat Expansion: A Perspective
Published 2013-01-01“…Amyotrophic lateral sclerosis (ALS) is a progressive and lethal disease of motor neuron degeneration, leading to paralysis of voluntary muscles and death by respiratory failure within five years of onset. Frontotemporal dementia (FTD) is characterised by degeneration of frontal and temporal lobes, leading to changes in personality, behaviour, and language, culminating in death within 5–10 years. …”
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28
Understanding retinal tau pathology through functional 2D and 3D iPSC-derived in vitro retinal models
Published 2025-01-01“…This study investigates the impact of the Frontotemporal Dementia-linked IVS 10 + 16 MAPT mutation on retinal development and function using 2D and 3D retinal models derived from human induced pluripotent stem cells. …”
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29
The Tree-Drawing Test (Koch’s Baum Test): A Useful Aid to Diagnose Cognitive Impairment
Published 2015-01-01“…Consecutive outpatients were classified as affected by dementia (Alzheimer’s disease (AD), frontotemporal dementia (FTD), and vascular dementia (VD)) or by MCI. …”
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Kaempferol enhances ER-mitochondria coupling and protects motor neurons from mitochondrial dysfunction and ER stress in C9ORF72-ALS
Published 2025-02-01“…Abstract Repeat expansions in the C9ORF72 gene are a frequent cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Considerable progress has been made in identifying C9ORF72-mediated disease and resolving its underlying etiopathogenesis. …”
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31
Nonfluent/Agrammatic PPA with In-Vivo Cortical Amyloidosis and Pick’s Disease Pathology
Published 2013-01-01“…The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. …”
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32
Delaying Onset of Dementia: Are Two Languages Enough?
Published 2014-01-01“…Moreover, the Hyderabad study extended the findings to frontotemporal dementia and vascular dementia.…”
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Molecular Mechanisms of Neurodegeneration Related to C9orf72 Hexanucleotide Repeat Expansion
Published 2019-01-01“…Two clinically distinct diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), have recently been classified as two extremes of the FTD/ALS spectrum. …”
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RIPK1 expression and inhibition in tauopathies: implications for neuroinflammation and neuroprotection
Published 2025-01-01“…We examined mouse models of frontotemporal dementia (FTD), as well as brain tissue samples from patients with progressive supranuclear palsy (PSP), a primary form of 4R tauopathy, and Alzheimer’s disease (AD), which is considered a secondary tauopathy. …”
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A variant in GRN of Spanish origin presenting with heterogeneous phenotypes
Published 2025-01-01“…Results: Phenotypes were strikingly different, including cases presenting with behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia, rapidly progressive motor neuron disease (pathologically documented), and tremor-dominant parkinsonism. …”
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Investigating neuropathological correlates of hyperactive and psychotic symptoms in dementia: a systematic review
Published 2025-01-01“…Of the 18,823 cases analyzed, the most common diagnoses were Alzheimer's Disease (83.44%), Dementia with Lewy Bodies (5.37%), and Frontotemporal Dementia (13.40%). HIDA-P symptoms were distributed across all clinical diagnoses, with agitation (14.00%), delusions (11.60%), disinhibition (7.61%), and hallucinations (6.83%) being the most frequently reported behaviors. …”
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