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Record W7117293752 · doi:10.1002/alz70857_105621

Cognitive and Behavioral Abnormalities in FTD Mutation Carriers: Insights into Shared Mechanisms with ADD/ADHD Disorder

2025· article· en· W7117293752 on OpenAlexaff
Yara Alkhodair, Ian R. Mackenzie, Emily Dwosh, Howard J. Rosen, Brad F Boeve, Adam L. Boxer, Hsiung Gd

Bibliographic record

VenueAlzheimer s & Dementia · 2025
Typearticle
Languageen
FieldMedicine
TopicAttention Deficit Hyperactivity Disorder
Canadian institutionsUniversity of British Columbia HospitalVancouver Coastal HealthPositive Living Society of British ColumbiaUniversity of British Columbia
Fundersnot available
KeywordsCognitionNeurodevelopmental disorderMutationMechanism (biology)

Abstract

fetched live from OpenAlex

BACKGROUND: Frontotemporal dementia (FTD) is a neurodegenerative disorder marked by early impairments in executive function and altered prefrontal neural circuitry. These traits are also observed in Attention Deficit Disorder (ADD) and Attention Deficit Hyperactivity Disorder (ADHD), suggesting the possibility of shared mechanisms and a role for developmental neurocognitive factors in the onset of FTD. High-penetrance mutations in GRN, C9orf72, and MAPT increase FTD risk and are suspected to influence neurodevelopment. This study hypothesizes that pre-symptomatic carriers of these mutations exhibit cognitive and behavioral traits that overlap with those observed in ADD/ADHD. METHOD: This cross-sectional study analyzed participants from the ARTFL-LEFFTDS Longitudinal FTLD (ALLFTD) Study. Inclusion criteria restricted the sample to mutation carriers with pathogenic variants in GRN, C9orf72, or MAPT while excluding individuals with prior psychiatric diagnoses (e.g., bipolar disorder, major depressive disorder) and those taking antipsychotic or anxiolytic medications to avoid potential confounding effects on test validity and interpretation. The final sample included N = 338 participants, categorized as pre-symptomatic mutation carriers (N = 227) or asymptomatic noncarriers (N = 111). To assess potential overlaps with ADD/ADHD traits, a targeted subset of measures reflecting executive function, working memory, attention, social behavior, and hyperactivity were extracted from validated cognitive and neuropsychiatric assessments. Mutation carriers were further classified as either converters (N = 20) who progressed to symptomatic FTD (CDR ≥ 1) or non-converters (N = 95) who remained asymptomatic. Only pre-conversion scores were analyzed for converters. Group comparisons were conducted using general linear models adjusted for age and sex. RESULT: Pre-symptomatic mutation carriers showed significantly lower verbal fluency (p = 0.004) and a trend toward reduced working memory (p = 0.05) compared to noncarriers. Converters exhibited poorer overall cognitive performance (p <0.0001), verbal fluency (p = 0.001), and memory and attention (p = 0.04) compared to non-converters. Converters demonstrated higher Neuropsychiatric Inventory scores (p = 0.0001), particularly in hyperactivity (p = 0.003), reflecting greater behavioral disturbances. CONCLUSION: Baseline impairments in cognitive function, memory, and neuropsychiatric profiles distinguish converters from non-converters, highlighting overlaps between ADD/ADHD traits and prodromal FTD. These findings suggest shared neurobiological mechanisms and underscore the need for mutation-specific studies to further explore the neurodevelopmental factors driving FTD progression.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.010
Threshold uncertainty score0.019

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0020.001
Science and technology studies0.0010.000
Scholarly communication0.0010.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0020.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.034
GPT teacher head0.326
Teacher spread0.293 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations0
Published2025
Admission routes1
Has abstractyes

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