ArticleAlzheimer's & dementia : the journal of the Alzheimer's Association2020
Clinical and volumetric changes with increasing functional impairment in familial frontotemporal lobar degeneration.
Article in Alzheimer's & dementia : the journal of the Alzheimer's Association, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02372773 (Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects), which is not on this map. Cited by 32 papers, 4 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects
Who cites it
32 citing papers in PubMed, 4 syntheses or guidelines pooled it, 44 citations in OpenAlex.
- The Differential Effects of Genetic Mutations in ALS and FTD Genes on Behavioural and Cognitive Changes: A Systematic Review and Meta-Analysis.International journal of molecular sciences · 2025Pooled it
- Neuropsychological Profiles in Genetic Frontotemporal Dementia: A Meta-Analysis and Systematic Review.Aging and disease · 2024Pooled it
- Pre-symptomatic radiological changes in frontotemporal dementia: propagation characteristics, predictive value and implications for clinical trials.Brain imaging and behavior · 2022Pooled it
- The imaging signature of C9orf72 hexanucleotide repeat expansions: implications for clinical trials and therapy development.Brain imaging and behavior · 2021Pooled it
- Cortical free-water imaging in familial frontotemporal dementia associated with MAPT, GRN, and C9orf72 pathogenic variants.Research square · 2026Article
- Intrathalamic delivery of adeno-associated viral vector expressing progranulin as gene therapy for GRN-related frontotemporal dementia.Molecular therapy. Methods & clinical development · 2025Article
- Cortical microstructure in familial frontotemporal dementia associated with MAPT, GRN, and C9orf72 pathogenic variants: Looking beyond atrophy.The journal of prevention of Alzheimer's disease · 2025Article
- Functional Connectivity Associations With Markers of Disease Progression in GRN Pathogenic Variant Carriers.Annals of clinical and translational neurology · 2025Article
- Sex differences in clinical phenotypes of behavioral variant frontotemporal dementia.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- Sex differences in the executive and behavioral reserve of autosomal dominant frontotemporal dementia.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- Montreal Cognitive Assessment vs the Mini-Mental State Examination as a Screening Tool for Patients With Genetic Frontotemporal Dementia.Neurology · 2025Article
- Analysis of convolutional neural networks for fronto-temporal dementia biomarker discovery.International journal of computer assisted radiology and surgery · 2024Article
- Examining Associations Between Smartphone Use and Clinical Severity in Frontotemporal Dementia: Proof-of-Concept Study.JMIR aging · 2024Article
- The Miami Framework for ALS and related neurodegenerative disorders: an integrated view of phenotype and biology.Nature reviews. Neurology · 2024Review
- Reliability and Validity of Smartphone Cognitive Testing for Frontotemporal Lobar Degeneration.JAMA network open · 2024Article
- The ScreeLing: Detecting Semantic, Phonological, and Syntactic Deficits in the Clinical Subtypes of Frontotemporal and Alzheimer's Dementia.Assessment · 2023Article
- Longitudinal changes in qualitative aspects of semantic fluency in presymptomatic and prodromal genetic frontotemporal dementia.Journal of neurology · 2023Article
- Presymptomatic grey matter alterations in ALS kindreds: a computational neuroimaging study of asymptomatic C9orf72 and SOD1 mutation carriers.Journal of neurology · 2023Article
- Clinical features and biomarkers of semantic variant primary progressive aphasia with MAPT mutation.Alzheimer's research & therapy · 2023Article
- Dissecting the Many Faces of Frontotemporal Dementia: An Imaging Perspective.International journal of molecular sciences · 2022Review
Corrections and comments
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Authors and funding
88 authors at 20 institutions in 3 countries.
Funding
Abstract
introductionThe Advancing Research and Treatment in Frontotemporal Lobar Degeneration and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects longitudinal studies were designed to describe the natural history of familial-frontotemporal lobar degeneration due to autosomal dominant mutations.
methodsWe examined cognitive performance, behavioral ratings, and brain volumes from the first time point in 320 MAPT, GRN, and C9orf72 family members, including 102 non-mutation carriers, 103 asymptomatic carriers, 43 mildly/questionably symptomatic carriers, and 72 carriers with dementia.
resultsAsymptomatic carriers showed similar scores on all clinical measures compared with noncarriers but reduced frontal and temporal volumes. Those with mild/questionable impairment showed decreased verbal recall, fluency, and Trail Making Test performance and impaired mood and self-monitoring. Dementia was associated with impairment in all measures. All MAPT carriers with dementia showed temporal atrophy, but otherwise, there was no single cognitive test or brain region that was abnormal in all subjects. DISCUSSION: Imaging changes appear to precede clinical changes in familial-frontotemporal lobar degeneration, but specific early clinical and imaging changes vary across individuals.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.