Results 21 to 30 of about 53,213 (267)
NeuroImage: Clinical, 2017 Objective: This present study aims to address the gap in the literature regarding the severity and underlying neural correlates of psychotic symptoms in frontotemporal dementia with and without the C9orf72 gene expansion. Methods: Fifty-six patients with
Emma M Devenney +7 more
doaj +1 more source
Altered Time Awareness in Dementia
Frontiers in Neurology, 2020 Our awareness of time, specifically of longer intervals spanning hours, days, months, and years, is critical for ensuring our sense of self-continuity.
Maï-Carmen Requena-Komuro +12 more
doaj +1 more source
Atypical Frontotemporal Dementia Associated With SQSTM1 Gene Mutation: A Clinicopathological Case. [PDF]
NeuropathologyABSTRACT A 78‐year‐old man presented with a six‐year history of progressive memory decline, initially manifesting as recent memory impairment and mild anomia, which gradually evolved into motor clumsiness, gait disturbances, language difficulties, behavioral changes, and late‐onset parkinsonism. He had been diagnosed with Paget disease of bone (PDB) at
Espinoza-Vinces C +8 more
europepmc +2 more sources
Nerve Excitability in Asymptomatic Carriers and Amyotrophic Lateral Sclerosis Patients With C9orf72
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Objective We investigated the effects of C9orf72 mutation carriership on peripheral nerve excitability in asymptomatic individuals from families with a history of C9orf72 amyotrophic lateral sclerosis (ALS) and patients. Methods We included 47 asymptomatic individuals from families with a history of C9orf72 ALS, of whom 23 were carriers (C9 ...
Diederik J. L. Stikvoort García +3 more
wiley +1 more source
Genetic Creutzfeldt‒Jakob disease with 5-octapeptide repeats presented as frontotemporal dementia
Human Genome Variation, 2023 The N-terminus of the PRNP gene normally contains a 5-octapeptide repeat (R1-R2-R2-R3-R4), and insertions at this locus can cause hereditary prion diseases.
Shinsuke Hamada +6 more
doaj +1 more source
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Introduction Neuronal pentraxin 2 (NPTX2) is a synaptic protein involved in synaptic plasticity and regulation of neuronal excitability. Lower baseline cerebrospinal fluid (CSF) NPTX2 levels have been shown to be associated with an earlier onset of mild cognitive impairment (MCI), a pre‐dementia syndrome, even after CSF Alzheimer's Disease (AD)
Juan P. Vazquez +12 more
wiley +1 more source
Frontotemporal dementia – a catastrophic form of dementia praecox
European Psychiatry, 2023 Introduction Frontotemporal dementia (FTD) is a devastating neurodegenerative condition with several clinical presentations for which there is currently no effective treatment.
A. R. Costa, S. Jesus, C. Vicente
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Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Background Mitofusin 2 (MFN2) is a major causative gene for axonal Charcot – Marie – Tooth disease type 2A (CMT2A), with a wide phenotypic spectrum. Comprehensive large ‐ scale genotype – phenotype association studies are essential for understanding disease pathogenesis and improved clinical management.
Masahiro Ando +13 more
wiley +1 more source
Olfactory impairment in frontotemporal dementia: A systematic review and meta-analysis
Dementia & Neuropsychologia, 2019 . Frontotemporal dementia (FTD) presents clinically in three variants: one behavioral and two with progressive primary aphasia - non-fluent/agrammatic and semantic. Defined by the degenerative process and cerebral atrophy, olfactory dysfunction occurs in
Maren de Moraes e Silva +7 more
doaj +1 more source
Altered plasma protein profiles in genetic FTD – a GENFI study
Molecular Neurodegeneration, 2023 Background Plasma biomarkers reflecting the pathology of frontotemporal dementia would add significant value to clinical practice, to the design and implementation of treatment trials as well as our understanding of disease mechanisms.
Abbe Ullgren +35 more
doaj +1 more source