Early stage of behavioral variant frontotemporal dementia: clinical and neuroimaging correlates

2015 ◽  
Vol 36 (11) ◽  
pp. 3108-3115 ◽  
Author(s):  
Barbara Borroni ◽  
Maura Cosseddu ◽  
Andrea Pilotto ◽  
Enrico Premi ◽  
Silvana Archetti ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Early Stage ◽  
Behavioral Variant Frontotemporal Dementia

scholarly journals 433 - A possible link between Bipolar Disease and Frontotemporal Dementia

2021 ◽  
Vol 33 (S1) ◽  
pp. 52-53
Author(s):  
B. Jorge ◽  
C. Pedro ◽  
J. Carvalho ◽  
S. Carneiro ◽  
M. Mangas
Keyword(s):  
Frontotemporal Dementia ◽  
Neurodegenerative Disorder ◽  
Psychiatric Symptoms ◽  
Early Stage ◽  
Synaptic Proteins ◽  
Behavioral Disturbances ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Bipolar Disease ◽  
Personality Changes ◽  
Relationship Of

Background:Both neurological and psychiatric symptoms are observed among mental disorders and represent a challenge for the differential diagnosis, specially in old adults presenting behavioral changes. Investigations have documented manic/hypomanic symptoms from behavioral variant frontotemporal dementia(bvFTD), suggesting a relationship of bipolar disease (BD) with bvFTD.Research objective:This work aims to determine the relationship between patients with bipolar disease and behavioral variant frontotemporal dementia.Method:A non-systematic review of the literature is presented. Bibliographic selection was carried out through keyword research in MEDLINE and Google Scholar.Results:An early stage of bvFTD often displays a mix of behavioral disturbances and personality changes. Also, BP is associated with a higher risk of dementia in older adults and with cognitive impairment, where a subset of patients presents a neuroprogressive pattern during the disease course. It was shown a specific type of post-BD dementia with clinical features of bvFTD and cases of patients with marked manic symptoms for the first time in their life had subsequent diagnosis of FTD. Mutations in the progranulin gene (GRN) were the most frequent causes of autosomal dominant FTD and have also been reported in sporadic FTD. Genetic polymorphisms in this gene are also associated with schizophrenia and BD. An hypothetical model of shared mechanisms between bvFTD and BD was proposed, including specific mendelian mutations associated with genetic predisposition (e.g. brain-derived neurotrophic factor-BDNF gene) and environmental factors with an effect on cellular homeostasis (e.g. increased cell deaths, decreased synthesis of synaptic proteins) and an influence over behavioural and cognitive symptoms. Nevertheless, comparison of the executive functions, social cognition profiles and structural neuroimaging of bvFTD and elderly patients with BD showed difference in patterns.Discussion:Although BD is principally considered a neurodevelopment disorder, while FTD is a neurodegenerative disorder, follow-up studies of cognitive deficits, imaging, and genetics in BD patients could elucidate the possible correlation between these major diseases and may have implications for pathogenesis, as well as for treatment.

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 The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Hannah D. Franklin ◽  
Lucy L. Russell ◽  
Georgia Peakman ◽  
Caroline V. Greaves ◽  
Martina Bocchetta ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Medial Temporal Lobe ◽  
Social Cognitive ◽  
Early Stage ◽  
Self Monitoring ◽  
Self Presentation ◽  
Genetic Groups ◽  
Mutation Carriers ◽  
Large Patient ◽  
Weighted Magnetic Resonance Imaging

Abstract Background Although social cognitive dysfunction is a major feature of frontotemporal dementia (FTD), it has been poorly studied in familial forms. A key goal of studies is to detect early cognitive impairment using validated measures in large patient cohorts. Methods We used the Revised Self-Monitoring Scale (RSMS) as a measure of socioemotional sensitivity in 730 participants from the genetic FTD initiative (GENFI) observational study: 269 mutation-negative healthy controls, 193 C9orf72 expansion carriers, 193 GRN mutation carriers and 75 MAPT mutation carriers. All participants underwent the standardised GENFI clinical assessment including the ‘CDR® plus NACC FTLD’ scale and RSMS. The RSMS total score and its two subscores, socioemotional expressiveness (EX score) and modification of self-presentation (SP score) were measured. Volumetric T1-weighted magnetic resonance imaging was available from 377 mutation carriers for voxel-based morphometry (VBM) analysis. Results The RSMS was decreased in symptomatic mutation carriers in all genetic groups but at a prodromal stage only in the C9orf72 (for the total score and both subscores) and GRN (for the modification of self-presentation subscore) groups. RSMS score correlated with disease severity in all groups. The VBM analysis implicated an overlapping network of regions including the orbitofrontal cortex, insula, temporal pole, medial temporal lobe and striatum. Conclusions The RSMS indexes socioemotional impairment at an early stage of genetic FTD and may be a suitable outcome measure in forthcoming trials.

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