Differences in Sex Distribution Between Genetic and Sporadic Frontotemporal Dementia

Boer, Sterre C.M. de; Riedl, Lina; Lee, Sven J. van der; Otto, Markus; Anderl‐Straub, Sarah; Landín-Romero, Ramón; Sorrentino, Federica; Fieldhouse, Jay L.P.; Reus, Lianne M.; Vacaflor, Blanca; Halliday, Glenda M.; Galimberti, Daniela; Diehl‐Schmid, Janine; Ducharme, Simon; Piguet, Olivier; Pijnenburg, Yolande A.L.

doi:10.3233/jad-210688

Cited by 15 publications

(11 citation statements)

References 48 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recently, late onset psychiatric symptoms have been proposed as one of the three prototypic prodromal DLB syndromes [49]. In addition, decreased motivation and psychotic symptoms have been found to be strong predictors of disease progression in frontotemporal degeneration (FTD), which appears to be more common in males [50][51][52].…”

Section: Discussionmentioning

confidence: 99%

Gender/Sex Differences in the Association of Mild Behavioral Impairment with Cognitive Aging

Wolfová

Creese

Aarsland

et al. 2022

JAD

View full text Add to dashboard Cite

Background: While the gender/sex differences in neuropsychiatric symptoms in dementia population are well described, gender/sex differences in mild behavioral impairment (MBI) in dementia-free populations and the relationship to cognitive performance and to subsequent cognitive decline have not been studied. Objective: We aimed to explore gender/sex differences in the association of MBI with the level of cognitive performance and its rate of decline in a dementia-free cohort. Methods: We studied 8,181 older adults enrolled in the online PROTECT UK Study. MBI was assessed using the MBI Checklist and cognition was measured by digit span, paired associate learning, spatial working memory, and verbal reasoning. Statistical analysis was conducted using linear regression models and linear mixed-effects models. Results: Out of 8,181 individuals (median age 63 years, 73% females), 11% of females and 14% of males had MBI syndrome. Females exhibited less often symptoms of decreased motivation (45% versus 36% in males), impulse dyscontrol (40% versus 44% in males; p = 0.001) and social inappropriateness (12% versus 15% ; p < 0.001), while they showed more often symptoms of emotional dysregulation (45% versus 36% ; p < 0.001). The associations of MBI domains with some measures of cognitive performance and decline were stronger in males than females, with the exception of the association of emotional dysregulation with the rate of cognitive decline in verbal reasoning, which was present exclusively in females. Conclusion: MBI may influence cognition to a greater extent in males than in females. We propose that predictors and biomarkers of dementia should consider gender/sex as an effect modifier.

show abstract

Section: Discussionmentioning

confidence: 99%

Gender/Sex Differences in the Association of Mild Behavioral Impairment with Cognitive Aging

Wolfová

Creese

Aarsland

et al. 2022

JAD

View full text Add to dashboard Cite

show abstract

“…Second, the groups differed substantially in proportions of males versus females due to inherent overrepresentation of males in both bvAD 2 and bvFTD. 49 As females are known to show better performance on social cognition tests, 50 this may have influenced results in favour of the tAD and SCD groups. However, as all results were corrected for age and sex, the effects in the current work are deemed minimal.…”

Section: Discussionmentioning

confidence: 99%

Social cognition deficits and biometric signatures in the behavioural variant of Alzheimer’s disease

Singleton

Fieldhouse

Hooft

et al. 2022

Brain

View full text Add to dashboard Cite

The behavioral variant of Alzheimer’s disease is characterized by early predominant behavioral changes, mimicking the behavioral variant of frontotemporal dementia, which is characterized by social cognition deficits and altered biometric responses to socioemotional cues. These functions remain understudied in the behavioral variant of Alzheimer’s disease. We investigated multiple social cognition components(i.e., emotion recognition, empathy, social norms and moral reasoning), using the Ekman-60-faces-test, Interpersonal-Reactivity-Index, empathy eliciting videos, Social-Norms-Questionnaire and moral dilemmas, while measuring eye-movements and galvanic skin response. We compared 12 patients with the behavioral variant of Alzheimer’s disease with patients with the behavioral variant of frontotemporal dementia(n = 14), typical Alzheimer’s disease(n = 13) and individuals with subjective cognitive decline(n = 13), using ANCOVAs and age and sex adjusted post hoc testing. Patients with the behavioral variant of Alzheimer’s disease(40.1 ± 8.6) showed lower scores on the Ekman-60-faces-test compared to individuals with subjective cognitive decline(49.7 ± 5.0, p < 0.001), and patients with typical Alzheimer’s disease(46.2 ± 5.3, p = 0.05) and higher scores compared to patients with behavioral variant of frontotemporal dementia(32.4 ± 7.3, p = 0.002). Eye-tracking during the Ekman-60-faces-test revealed no differences in dwell time on the eyes(all p > 0.05), but patients with the behavioral variant of Alzheimer’s disease(18.7 ± 9.5%) and frontotemporal dementia(19.4 ± 14.3%) spent significantly less dwell time on the mouth than individuals with subjective cognitive decline(30.7 ± 11.6%, p < 0.01) and patients with typical Alzheimer’s disease(32.7 ± 12.1%, p < 0.01). Patients with the behavioral variant of Alzheimer’s disease(11.3 ± 4.6) exhibited lower scores on the Interpersonal-Reactivity-Index compared with individuals with subjective cognitive decline(15.6 ± 3.1, p = 0.05) and similar scores to patients with the behavioral variant of frontotemporal dementia(8.7 ± 5.6, p = 0.19) and typical Alzheimer’s disease(13.0 ± 3.2, p = 0.43). The galvanic skin response to empathy eliciting videos did not differ between groups(all p > 0.05). Patients with the behavioral variant of Alzheimer’s disease(16.0 ± 1.6) and frontotemporal dementia(15.2 ± 2.2) showed lower scores on the Social-Norms-Questionnaire than patients with typical Alzheimer’s disease(17.8 ± 2.1, p < 0.05) and individuals with subjective cognitive decline(18.3 ± 1.4, p < 0.05). No group differences were observed in scores on moral dilemmas(all p > 0.05), while only patients with the behavioral variant of frontotemporal dementia(0.9 ± 1.1) showed a lower galvanic skin response during personal dilemmas compared with subjective cognitive decline(3.4 ± 3.3 peaks per minute, p = 0.01). Concluding, patients with the behavioral variant of Alzheimer’s disease showed a similar though milder social cognition profile and a similar eye-tracking signature to patients with the behavioral variant of frontotemporal dementia and greater social cognition impairments and divergent eye-movement patterns compared with patients with typical Alzheimer’s disease. Our results suggest reduced attention to salient facial features in these phenotypes, potentially contributing to their emotion recognition deficits.

show abstract

“…Finally, VENs which are selectively targeted in bvFTD are more numerous in the right than left hemisphere. 71,72 A trend towards a later AAO in females with sporadic bvFTD 73 and all cause bvFTD 74 has been reported. Others have demonstrated better than expected executive function in females despite similar levels of atrophy to males.…”

Section: Discussionmentioning

confidence: 96%

Anterior cingulate sulcation is associated with onset and survival in frontotemporal dementia

Harper

Boer

Lindberg

et al. 2023

Preprint

View full text Add to dashboard Cite

Background: Frontotemporal dementia is the second most common form of early onset dementia (< 65 years). Despite this there are few known disease modifying factors. The anterior cingulate is a focal point of pathology in behavioural variant frontotemporal dementia. Sulcation of the anterior cingulate is denoted by the presence of a paracingulate sulcus, a tertiary sulcus developing, where present during the third gestational trimester and remaining stable throughout life. This study aims to examine the impact of right paracingulate sulcal presence on the expression and prognosis of behavioural variant Frontotemporal Dementia. Methods: This retrospective analysis drew its population from two clinical samples recruited from memory clinics at University Hospitals in The United States of America and The Netherlands. Individuals with sporadic behavioural variant Frontotemporal Dementia were enrolled between 2004 and 2022 and followed up for an average of 7.71 years. T1-MRI data were evaluated for hemispheric paracingulate sulcal presence in accordance with an established protocol by two blinded raters. Outcome measures included age at onset, survival, cortical thickness, and Frontotemporal Lobar Degeneration-modified Clinical Dementia Rating determined clinical disease progression. Results: The study population consisted of 186 individuals with sporadic behavioural variant Frontotemporal Dementia, (113 males and 73 females) mean age 63.28 years (SD 8.32). The mean age at onset was 2.44 years later in individuals possessing a right paracingulate sulcus (60.2 years (SD 8.54)) versus individuals who did not (57.76 (8.05)), 95% CI >0.41, P = 0.02. Education was not associated with age at onset (Beta = -0.05, P=0.75). Presence of a right paracingulate sulcus was associated with a 119% increased risk of death per year after age at onset (HR 2.19, CI [1.21 - 3.96], P<0.01), whilst the mean age at death was similar for individuals with a present and absent right paracingulate sulcus (P = 0.7). Right paracingulate sulcal presence was not associated with baseline cortical thickness. Conclusion. Right paracingulate sulcal presence is associated with disease expression and survival in sporadic behavioural variant Frontotemporal Dementia. Findings provide evidence of neurodevelopmental brain reserve in behavioural variant Frontotemporal Dementia which may be important in the design of trials for future therapeutic approaches.

show abstract

Differences in Sex Distribution Between Genetic and Sporadic Frontotemporal Dementia

Cited by 15 publications

References 48 publications

Gender/Sex Differences in the Association of Mild Behavioral Impairment with Cognitive Aging

Gender/Sex Differences in the Association of Mild Behavioral Impairment with Cognitive Aging

Social cognition deficits and biometric signatures in the behavioural variant of Alzheimer’s disease

Anterior cingulate sulcation is associated with onset and survival in frontotemporal dementia

Contact Info

Product

Resources

About