Results 51 to 60 of about 14,742 (206)
Neurobiology of Disease, 2008 Progranulin gene (PGRN) mutations cause ubiquitin-positive frontotemporal lobar degeneration linked to chromosome 17 (FTLDU-17). The spectrum of known mutations strongly suggests that neurodegeneration results from a partial loss of PGRN function and ...
Anne Rovelet-Lecrux +10 more
doaj +1 more source
Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach
Brain Sciences, 2023 Frontotemporal lobar degeneration (FTLD) belongs to a heterogeneous group of highly complex neurodegenerative diseases and represents the second cause of presenile dementia in individuals under 65.
Genaro Gabriel Ortiz +4 more
doaj +1 more source
Brainstem : neglected locus in neurodegenerative diseases
, 2011 The most frequent neurodegenerative diseases (NDs) are Alzheimer’s disease (AD), Parkinson’s disease (PD), and frontotemporal lobar degeneration associated with protein TDP-43 (FTLD–TDP).
Lea Tenenholz Grinberg +8 more
core +1 more source
Value of MRI Outcomes for Preventive and Early‐Stage Trials in Spinocerebellar Ataxias 1 and 3
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Objective To examine the value of MRI outcomes as endpoints for preventive and early‐stage trials of two polyglutamine spinocerebellar ataxias (SCAs). Methods A cohort of 100 participants (23 SCA1, 63 SCA3, median Scale for the Assessment and Rating of Ataxia (SARA) score = 5, 42% preataxic, and 14 gene‐negative controls) was scanned at 3T up ...
Thiago J. R. Rezende +26 more
wiley +1 more source
Predictive Ability of Plasma p‐tau217 for β‐Amyloid Status: A Prospective Multicenter Study
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Objective Plasma tau phosphorylated at threonine 217 (p‐tau217) measured with fully automated platforms has shown high accuracy for Alzheimer's disease (AD) diagnosis, but real‐world multicenter data remain limited. We aimed to validate the diagnostic performance of p‐tau217 for identifying AD pathology in a real‐world multicenter cohort ...
Miquel Massons +33 more
wiley +1 more source
Problematic Internet Use in Frontotemporal Dementia: A Case Series
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT The present study investigated problematic internet use (PIU) among 61 patients with frontotemporal dementia (FTD) compared to a cohort of 354 patients with mild cognitive impairment (MCI) and Alzheimer's dementia. PIU was identified in 22.9% of FTD patients compared to only 0.8% of AD patients (p < 0.001). Behaviors included compulsive social
Daniele Urso +9 more
wiley +1 more source
Metabolomic changes associated with frontotemporal lobar degeneration syndromes.
, 2020 OBJECTIVE: Widespread metabolic changes are seen in neurodegenerative disease and could be used as biomarkers for diagnosis and disease monitoring. They may also reveal disease mechanisms that could be a target for therapy.
core +1 more source
Archives of Epilepsy, 2019 Seizures may occur in frontotemporal lobar degeneration syndromes as an element of a heterogeneous group of disorders, according to both clinical phenotype and neuropathology.
Yasemin DİNÇ, Gönül ÇELİK AKDAĞ
doaj +1 more source
Cognitive and olfactory function profiles of frontotemporal lobar degeneration
Chinese Journal of Contemporary Neurology and Neurosurgery, 2021 Objective To describe the cognitive and olfactory function profiles of frontotemporal lobar degeneration (FTLD). Methods A total of 52 FTLD patients admitted to Tianjin Huanhu Hospital from July 2014 to April 2017 were enrolled.
DOU Yu⁃chao +4 more
doaj +1 more source
Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis
Annals of Clinical and Translational Neurology, EarlyView.ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs +7 more
wiley +1 more source