scholarly journals Clinical Phenotypes of Behavioral Variant Frontotemporal Dementia by Age at Onset

2021 ◽  
pp. 1-10
Author(s):  
Jay L.P. Fieldhouse ◽  
Flora T. Gossink ◽  
Thomas C. Feenstra ◽  
Sterre C.M. de Boer ◽  
Afina W. Lemstra ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Mortality Risk ◽  
Late Onset ◽  
Neuropsychiatric Symptoms ◽  
Age At Onset ◽  
Depression Scale ◽  
Age At Diagnosis ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Clinical Criteria ◽  
Symptom Profile

Background: Behavioral variant frontotemporal dementia (bvFTD) is generally considered a young-onset dementia, although age at onset is highly variable. While several studies indicate clinical differences regarding age at onset, no biomarker validated cohort studies with updated clinical criteria have been performed. Objective: We aimed to examine behavior, cognition, and mortality over the full age spectrum in a cohort of bvFTD patients with neuroimaging, genetic, or histopathological confirmation and exclusion of positive Alzheimer’s disease biomarkers or severe cerebrovascular damage. Methods: In total, 315 patients with a clinical diagnosis of probable or definite bvFTD were included from the Amsterdam Dementia Cohort and grouped into quartiles by age-at-diagnosis. Neuropsychiatric symptoms and cognitive functioning were assessed with the neuropsychiatric inventory, the geriatric depression scale and a neuropsychological test battery. Data on mortality was obtained from the Dutch municipal register. Associations between age-at-diagnosis and clinical features and mortality risk were examined. Results: Age-at-diagnosis ranged from 26 to 85 years and established quartiles with mean ages of 52±6, 61±2, 66±2, and 74±3 years. In the total sample, 44.4%exceeded an age of 65 years at time of diagnosis. Earlier age-at-diagnosis was associated with more severe behavioral symptoms, while later age-at-diagnosis was associated with more severe memory impairment. Unexpectedly, mortality risk was not associated with age-at-diagnosis. Conclusion: In bvFTD, symptom profile is associated with age-at-diagnosis. This should be taken into account with regard to diagnostics, patient management, and trial design. Additionally, based on our sample, the prevalence of late-onset bvFTD is higher than generally thought.

scholarly journals Distinct network topology in Alzheimer’s disease and behavioral variant frontotemporal dementia

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Adeline Su Lyn Ng ◽  
Juan Wang ◽  
Kwun Kei Ng ◽  
Joanna Su Xian Chong ◽  
Xing Qian ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Network Topology ◽  
Neuropsychiatric Symptoms ◽  
Brain Network ◽  
Salience Network ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Control Networks ◽  
And Control

Abstract Background Alzheimer’s disease (AD) and behavioral variant frontotemporal dementia (bvFTD) cause distinct atrophy and functional disruptions within two major intrinsic brain networks, namely the default network and the salience network, respectively. It remains unclear if inter-network relationships and whole-brain network topology are also altered and underpin cognitive and social–emotional functional deficits. Methods In total, 111 participants (50 AD, 14 bvFTD, and 47 age- and gender-matched healthy controls) underwent resting-state functional magnetic resonance imaging (fMRI) and neuropsychological assessments. Functional connectivity was derived among 144 brain regions of interest. Graph theoretical analysis was applied to characterize network integration, segregation, and module distinctiveness (degree centrality, nodal efficiency, within-module degree, and participation coefficient) in AD, bvFTD, and healthy participants. Group differences in graph theoretical measures and empirically derived network community structures, as well as the associations between these indices and cognitive performance and neuropsychiatric symptoms, were subject to general linear models, with age, gender, education, motion, and scanner type controlled. Results Our results suggested that AD had lower integration in the default and control networks, while bvFTD exhibited disrupted integration in the salience network. Interestingly, AD and bvFTD had the highest and lowest degree of integration in the thalamus, respectively. Such divergence in topological aberration was recapitulated in network segregation and module distinctiveness loss, with AD showing poorer modular structure between the default and control networks, and bvFTD having more fragmented modules in the salience network and subcortical regions. Importantly, aberrations in network topology were related to worse attention deficits and greater severity in neuropsychiatric symptoms across syndromes. Conclusions Our findings underscore the reciprocal relationships between the default, control, and salience networks that may account for the cognitive decline and neuropsychiatric symptoms in dementia.

scholarly journals Very late-onset behavioral variant frontotemporal dementia

2013 ◽  
Vol 7 (1) ◽  
pp. 136-139
Author(s):  
Henrique Cerqueira Guimarães ◽  
Tatiana de Carvalho Espindola
Keyword(s):  
Case Report ◽  
Frontotemporal Dementia ◽  
Frontotemporal Lobar Degeneration ◽  
Clinical Presentation ◽  
Late Onset ◽  
Ground Level ◽  
Dementia Diagnosis ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Genetically Determined ◽  
Late Onset Dementia

ABSTRACT Current concepts regarding frontotemporal lobar degeneration (FTLD) have evolved rapidly in recent years. Genetically determined FTLD cohorts have broadened our knowledge pertaining to its clinical presentation, neuroimaging findings and demographics. In this study we present a case report of a patient diagnosed with behavioral variant frontotemporal dementia diagnosis in her nineties during hospital admission for a ground-level fall. We believe this case reinforces the pervasive nature of this clinical entity, and may contribute to an increased awareness of this diagnostic possibility in late-onset dementia.

Impact of Imaging and Cerebrospinal Fluid Biomarkers on Behavioral Variant Frontotemporal Dementia Diagnosis within a Late-Onset Frontal Lobe Syndrome Cohort

2015 ◽  
Vol 41 (1-2) ◽  
pp. 16-26 ◽  
Author(s):  
Welmoed A. Krudop ◽  
Annemiek Dols ◽  
Cora J. Kerssens ◽  
Niels D. Prins ◽  
Christiane Möller ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Frontotemporal Dementia ◽  
Frontal Lobe ◽  
Fdg Pet ◽  
Late Onset ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Diagnostic Certainty ◽  
18F Fdg ◽  
Frontal Lobe Syndrome ◽  
The Impact

Background: The criteria for behavioral variant frontotemporal dementia (bvFTD) incorporate MRI and [18F]-FDG-PET. Cerebrospinal fluid (CSF) analysis is merely advised for excluding Alzheimer's disease. Aims: We aimed to assess the impact of biomarkers on diagnostic certainty and contingent changes of bvFTD diagnosis within the clinically relevant neuropsychiatric differential diagnosis of subjects with a late-onset frontal lobe syndrome (LOF). Methods: We included 137 patients with LOF, aged 45-75 years, 72% males. Biomarker disclosure was considered contributing after any substantial difference in diagnostic certainty or a diagnostic change. Percentages of contributing biomarkers were compared between three major diagnostic groups (bvFTD, psychiatry, other neurological disorders). Certainty levels in stable diagnostic groups were compared to those with a diagnostic change. Results: Biomarkers contributed in 53, 60 and 41% of the LOF patients for MRI, [18F]-FDG-PET and CSF, respectively. Biomarkers changed the diagnosis in 14% of cases towards bvFTD and in 13% from bvFTD into an alternative. Those that changed had a lower level of a priori diagnostic certainty compared to stable diagnoses. Conclusion: Our study not only supports the widely accepted use of MRI and [18F]-FDG-PET in diagnosing or excluding bvFTD, but also shows that CSF biomarkers aid clinicians in the diagnostic process.

scholarly journals Kraepelin’s description of chronic mania: a clinical picture that meets the behavioral variant frontotemporal dementia phenotype

2016 ◽  
Vol 74 (9) ◽  
pp. 775-777 ◽  
Author(s):  
Leandro Boson Gambogi ◽  
Henrique Cerqueira Guimarães ◽  
Maurício Viotti Daker ◽  
Leonardo Cruz de Souza ◽  
Paulo Caramelli
Keyword(s):  
Frontotemporal Dementia ◽  
Clinical Picture ◽  
Historical Data ◽  
Clinical Phenotype ◽  
Case Reports ◽  
Neuropsychiatric Symptoms ◽  
Ideal Case ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Behavioral Neurology ◽  
A Current

ABSTRACT Chronic mania is an under-investigated condition and few reports have associated this disorder with an organic background. The present work examines Kraepelin’s reliable description of chronic mania from a current behavioral neurology viewpoint. Kraepelin had described a cluster of symptoms that are now recognized as core manifestations of the behavioral variant frontotemporal dementia (bvFTD) clinical phenotype. We also carried out additional reviews of original manuscripts from Kraepelin’s peers, in order to find any case reports that might fulfill the current diagnostic proposal for bvFTD. Even though we failed to find an ideal case, we found some scholars who seemed to agree that chronic mania should be considered a special form of dementia. The present work highlights, through historical data, the possible overlapping features between primary psychiatric disorders and neuropsychiatric symptoms secondary to neurodegenerative conditions.

scholarly journals Anosognosia in Early- and Late-Onset Dementia and Its Association With Neuropsychiatric Symptoms

2021 ◽  
Vol 12 ◽  
Author(s):  
Manuela Tondelli ◽  
Chiara Galli ◽  
Giulia Vinceti ◽  
Luigi Fiondella ◽  
Simone Salemme ◽  
...  
Keyword(s):  
Regression Analysis ◽  
Disease Progression ◽  
Frontotemporal Dementia ◽  
Late Onset ◽  
Neuropsychiatric Symptoms ◽  
Diagnostic Delay ◽  
Early Onset Dementia ◽  
Dementia Patients ◽  
Clinical Variants ◽  
Late Onset Dementia

Background: The symptom anosognosia or unawareness of disease in dementia has mainly been studied in patients with late-onset dementia (LOD, ≥65 years), whereas little is known on whether it is also present in patients with early-onset dementia (EOD, <65 years). We aimed at investigating differences in anosognosia between LOD and EOD, by also studying its association with different clinical variants of EOD and the presence of neuropsychiatric symptoms.Methods: A total of 148 patients, 91 EOD and 57 LOD, were recruited and underwent extended clinical assessment and caregiver interview that included questionnaires aimed at measuring anosognosia and neuropsychiatric symptoms. Differences in anosognosia between EOD and LOD and between subgroups with different clinical variants were investigated, as well as correlation between anosognosia and neuropsychiatric symptoms. A regression analysis was applied to explore the association between anosognosia and development of neuropsychiatric symptoms during disease progression.Results: Median levels of anosognosia were not significantly different between EOD and LOD. Anosognosia increased overtime with disease progression and was higher in frontotemporal dementia patients or, more precisely, in frontotemporal dementia and Alzheimer's disease variants associated with involvement of the frontal lobes. Higher levels of early anosognosia were associated with higher frequency and severity of subsequent neuropsychiatric symptoms, in particular apathy, later in the course of the disease.Conclusion: Anosognosia is a frequent symptom of EOD, occurring in 94.5% of all-cause EOD, and it is associated with higher risk of developing neuropsychiatric symptoms during disease progression. Recognising anosognosia may be helpful for clinicians and families to reduce diagnostic delay and improve disease managment.

scholarly journals Neuropsychiatric Symptoms in Behavioral Variant Frontotemporal Dementia and Alzheimer's Disease: A 12-Month Follow-Up Study

2021 ◽  
Vol 12 ◽  
Author(s):  
Thais Bento Lima Da Silva ◽  
Tiago Nascimento Ordonez ◽  
Allan Gustavo Bregola ◽  
Valéria Santoro Bahia ◽  
Mário Amore Cecchini ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Neuropsychiatric Symptoms ◽  
Linear Regression Analysis ◽  
Predictor Variable ◽  
Wilcoxon Test ◽  
Chi Square ◽  
Behavioral Variant Frontotemporal Dementia

Introduction: Neuropsychiatric symptoms in patients with frontotemporal dementia (FTD) are highly prevalent and may complicate clinical managements.Objective: To test whether the Neuropsychiatry Inventory (NPI) could detect change in neuropsychiatric symptoms and caregiver's distress in patients diagnosed with behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD) from baseline to a 12-month follow-up and to investigate possible predictors of change in NPI scores.Methods: The sample consisted of 31 patients diagnosed with bvFTD and 28 patients with AD and their caregivers. The Mini-Mental State Examination (MMSE), Addenbrooke's Cognitive Examination Revised (ACE-R), the INECO Frontal Screening (IFS), the Frontal Assessment Battery (FAB), the Executive Interview (EXIT-25) and the NPI were applied. Descriptive statistics, Mann-Whitney U test, Wilcoxon test, Chi square (χ2) test and Linear Regression Analysis were used.Results: NPI total and caregiver distress scores were statistically higher among bvFTD patients at both assessment points. MMSE, ACE-R scores significantly declined and NPI Total and Distress scores significantly increased in both groups. In the bvFTD group, age was the only independent predictor variable for the NPI total score at follow up. In the AD group, ACE-R and EXIT-25, conjunctively, were associated with the NPI total score at follow up.Conclusions: In 12 months, cognition declined and neuropsychiatric symptoms increased in bvFTD and AD groups. In the AD group only, cognitive impairment was a significant predictor of change in neuropsychiatric symptoms.

scholarly journals Neuropsychiatric Effects on Decision-Making in Early Alzheimer Disease

2020 ◽  
Vol 33 (2) ◽  
pp. 68-72
Author(s):  
Oleg Yerstein ◽  
Andrew R. Carr ◽  
Elvira Jimenez ◽  
Mario F. Mendez
Keyword(s):  
Decision Making ◽  
Alzheimer Disease ◽  
Frontotemporal Dementia ◽  
Ultimatum Game ◽  
Neuropsychiatric Symptoms ◽  
The Other ◽  
Healthy Controls ◽  
Neuropsychiatric Inventory ◽  
Behavioral Variant Frontotemporal Dementia

Background: Neuropsychiatric symptoms can impact decision-making in patients with Alzheimer disease (AD). Methods: Using a simple decision-making task, a variant of the ultimatum game (UG) modified to control feelings of unfairness, this study investigated rejection responses among responders to unfair offers. The UG was administered to 11 patients with AD, 10 comparably demented patients with behavioral variant frontotemporal dementia (bvFTD), and 9 healthy controls (HC). The results were further compared with differences on the caregiver Neuropsychiatric Inventory (NPI). Results: Overall, patients with AD significantly rejected more total offers than did the patients with bvFTD and the HC ( P < .01). On the NPI, the only domain that was significantly worse among the patients with AD compared to the other groups was dysphoria/depression. Conclusions: These results suggest that early AD can be distinguished based on increased rejections of offers in decision-making, possibly consequent to a heightened sense of unfairness from dysphoria/depression.

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