End Stage Clinical Features and Cause of Death of Behavioral Variant Frontotemporal Dementia and Young-Onset Alzheimer’s Disease

Background: Limited literature exists regarding the clinical features of end stage behavioral variant frontotemporal dementia (bvFTD). This data is indispensable to inform and prepare family members as well as professional caregivers for the expected disease course and to anticipate with drug-based and non-pharmacological treatment strategies. Objective: The aim of the present study was to describe end stage bvFTD in a broad explorative manner and to subsequently evaluate similarities and dissimilarities with the end stage of the most prevalent form of young-onset dementia, Alzheimer’s disease (yoAD). Methods: We analyzed medical files on patients, using a mixed model of qualitative and quantitative approaches. Included were previously deceased patients with probable bvFTD and probable yoAD. End stage was defined as the last 6 months prior to death. Primary outcome measures comprised somatic, neurological, and psychiatric symptoms and the secondary outcome measure was cause of death. Results: Out of 89 patients, a total of 30 patients were included (bvFTD; n = 12, yoAD; n = 18). Overall, the end stages of bvFTD and yoAD were characterized by a broad spectrum of clinical symptoms including severe autonomic dysfunction and an increased muscle tone. Patients with bvFTD displayed more mutism compared with yoAD while compulsiveness was only present in bvFTD. Conclusion: Our study describes the full clinical spectrum of end stage bvFTD and yoAD. In this study, symptoms extend far beyond the initial behavioral and cognitive features. By taking both somatic, psychiatric, and neurological features into account, family members and professional caregivers may anticipate (non) pharmacological treatment.

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P2-511: NOT ALL DEMENTIA CAREGIVING IS THE SAME: BEHAVIORAL VARIANT FRONTOTEMPORAL DEMENTIA COMPARED TO YOUNG-ONSET ALZHEIMER'S DISEASE CAREGIVER BURDEN

Alzheimer s & Dementia ◽

10.1016/j.jalz.2019.06.2919 ◽

2019 ◽

Vol 15 ◽

pp. P813-P813

Author(s):

Elvira E. Jimenez ◽

Li-Jung F. Liang ◽

Mario F. Mendez

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Frontotemporal Dementia ◽

Caregiver Burden ◽

Behavioral Variant Frontotemporal Dementia ◽

Young Onset ◽

Dementia Caregiving

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

Alzheimer s Research & Therapy ◽

10.1186/s13195-020-00752-w ◽

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.

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Different apathy clinical profile and neural correlates in behavioral variant frontotemporal dementia and Alzheimer's disease

International Journal of Geriatric Psychiatry ◽

10.1002/gps.4695 ◽

2017 ◽

Vol 33 (1) ◽

pp. 141-150 ◽

Cited By ~ 18

Author(s):

Marta Fernández-Matarrubia ◽

Jordi A. Matías-Guiu ◽

María Nieves Cabrera-Martín ◽

Teresa Moreno-Ramos ◽

María Valles-Salgado ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Frontotemporal Dementia ◽

Neural Correlates ◽

Clinical Profile ◽

Behavioral Variant Frontotemporal Dementia

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Brazilian Version of Addenbrooke’s Cognitive Examination—Revised in the Differential Diagnosis of Alzheimer’S Disease and Behavioral Variant Frontotemporal Dementia

Archives of Clinical Neuropsychology ◽

10.1093/arclin/acab071 ◽

2021 ◽

Author(s):

Viviane Amaral-Carvalho ◽

Thais Bento Lima-Silva ◽

Luciano Inácio Mariano ◽

Leonardo Cruz de Souza ◽

Henrique Cerqueira Guimarães ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Differential Diagnosis ◽

Diagnostic Accuracy ◽

Frontotemporal Dementia ◽

Predictive Value ◽

Univariate Analysis ◽

Behavioral Variant Frontotemporal Dementia ◽

Present Sample ◽

Addenbrooke’S Cognitive Examination

Abstract Introduction Alzheimer’s disease (AD) and behavioral variant frontotemporal dementia (bvFTD) are frequent causes of dementia and, therefore, instruments for differential diagnosis between these two conditions are of great relevance. Objective To investigate the diagnostic accuracy of Addenbrooke’s Cognitive Examination-Revised (ACE-R) for differentiating AD from bvFTD in a Brazilian sample. Methods The ACE-R was administered to 102 patients who had been diagnosed with mild dementia due to probable AD, 37 with mild bvFTD and 161 cognitively healthy controls, matched according to age and education. Additionally, all subjects were assessed using the Mattis Dementia Rating Scale and the Neuropsychiatric Inventory. The performance of patients and controls was compared by using univariate analysis, and ROC curves were calculated to investigate the accuracy of ACE-R for differentiating AD from bvFTD and for differentiating AD and bvFTD from controls. The verbal fluency plus language to orientation plus name and address delayed recall memory (VLOM) ratio was also calculated. Results The optimum cutoff scores for ACE-R were <80 for AD, <79 for bvFTD, and <80 for dementia (AD + bvFTD), with area under the receiver operating characteristic curves (ROC) (AUC) >0.85. For the differential diagnosis between AD and bvFTD, a VLOM ratio of 3.05 showed an AUC of 0.816 (Cohen’s d = 1.151; p < .001), with 86.5% sensitivity, 71.4% specificity, 72.7% positive predictive value, and 85.7% negative predictive value. Conclusions The Brazilian ACE-R achieved a good diagnostic accuracy for differentiating AD from bvFTD patients and for differentiating AD and bvFTD from the controls in the present sample.

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Fronto-Striatal Atrophy in Behavioral Variant Frontotemporal Dementia and Alzheimer’s Disease

Frontiers in Neurology ◽

10.3389/fneur.2015.00147 ◽

2015 ◽

Vol 6 ◽

Cited By ~ 28

Author(s):

Maxime Bertoux ◽

Claire O’Callaghan ◽

Emma Flanagan ◽

John R. Hodges ◽

Michael Hornberger

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Frontotemporal Dementia ◽

Behavioral Variant Frontotemporal Dementia

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Interactions between decision-making and emotion in behavioral-variant frontotemporal dementia and Alzheimer’s disease

Social Cognitive and Affective Neuroscience ◽

10.1093/scan/nsaa085 ◽

2020 ◽

Vol 15 (6) ◽

pp. 681-694

Author(s):

Aurélie L Manuel ◽

Daniel Roquet ◽

Ramon Landin-Romero ◽

Fiona Kumfor ◽

Rebekah M Ahmed ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Decision Making ◽

Frontotemporal Dementia ◽

Delay Discounting ◽

Animal Studies ◽

Negative Emotion ◽

Emotion Processing ◽

Brain Regions ◽

Behavioral Variant Frontotemporal Dementia

Abstract Negative and positive emotions are known to shape decision-making toward more or less impulsive responses, respectively. Decision-making and emotion processing are underpinned by shared brain regions including the ventromedial prefrontal cortex (vmPFC) and the amygdala. How these processes interact at the behavioral and brain levels is still unclear. We used a lesion model to address this question. Study participants included individuals diagnosed with behavioral-variant frontotemporal dementia (bvFTD, n = 18), who typically present deficits in decision-making/emotion processing and atrophy of the vmPFC, individuals with Alzheimer’s disease (AD, n = 12) who present with atrophy in limbic structures and age-matched healthy controls (CTRL, n = 15). Prior to each choice on the delay discounting task participants were cued with a positive, negative or neutral picture and asked to vividly imagine witnessing the event. As hypothesized, our findings showed that bvFTD patients were more impulsive than AD patients and CTRL and did not show any emotion-related modulation of delay discounting rate. In contrast, AD patients showed increased impulsivity when primed by negative emotion. This increased impulsivity was associated with reduced integrity of bilateral amygdala in AD but not in bvFTD. Altogether, our results indicate that decision-making and emotion interact at the level of the amygdala supporting findings from animal studies.

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