scholarly journals Behavioral Variant Frontotemporal Dementia: Diagnosis and Treatment Interventions

2021 ◽  
Author(s):  
Silpa Balachandran ◽  
Elizabeth L. Matlock ◽  
Michelle L. Conroy ◽  
Chadrick E. Lane
Keyword(s):  
Frontotemporal Dementia ◽  
Neurodegenerative Disorder ◽  
Diagnosis And Treatment ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Caregiver Support ◽  
Psychiatric Illnesses ◽  
Heritable Disorder ◽  
Pharmacologic Management ◽  
Gradual Accumulation ◽  
Disease Modifying Agents

Abstract Purpose of Review The diagnosis and treatment of behavioral variant frontotemporal dementia is challenging and often delayed because of overlapping symptoms with more common dementia syndromes or primary psychiatric illnesses. The purpose of this paper is to explore the relevant presentation, diagnostic workup, pathophysiology, and both pharmacologic and non-pharmacologic management. Recent Findings Behavioral variant frontotemporal dementia is a highly heritable disorder. The gradual accumulation of diseased protein culminates in the destruction of those brain circuits responsible for much of one’s emotional and social functioning. Summary Behavioral variant frontotemporal dementia is a progressive neurodegenerative disorder with a far-reaching impact on patients and caregivers. Patients often present with emotional blunting, lack of empathy, apathy, and behavioral disinhibition. Non-pharmacologic interventions and caregiver support are the cornerstone of treatment. The use of cholinesterase inhibitors and memantine is not supported by the evidence. While current pharmacologic therapies target only certain symptoms, there are disease modifying agents currently in or nearing the clinical research stage.

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.

Autoimmunity and Frontotemporal Dementia

2018 ◽  
Vol 15 (7) ◽  
pp. 602-609 ◽  
Author(s):  
Antonella Alberici ◽  
Viviana Cristillo ◽  
Stefano Gazzina ◽  
Alberto Benussi ◽  
Alessandro Padovani ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Language Impairment ◽  
Neurodegenerative Disorder ◽  
Chromosome 9 ◽  
Bibliographic Databases ◽  
Extensive Literature ◽  
Genetic Studies ◽  
Reading Frame ◽  
Current State ◽  
Extensive Evaluation

Background: Frontotemporal Dementia (FTD) is a neurodegenerative disorder which asymmetrically affects the frontotemporal lobe, characterized by behavioural abnormalities, language impairment, and deficits of executive functions. Genetic studies identified mutations causing the disease, namely Microtubule Associated Protein Tau (MAPT), Granulin (GRN) and chromosome 9 open reading frame 72 (C9orf72) mutations, which contributed to elucidate the molecular pathways involved in brain depositions of either Tau or TAR DNA-binding protein 43 (TDP43) inclusions. However, in the majority of sporadic FTD patients, the mechanisms triggering Tau or TDP43 protein deposition are still to be uncovered. Objective: We aimed to present an extensive evaluation of literature data on immune homeostasis in FTD, in order to provide potentially evidence-based approaches for a disease still orphan of any treatment. Methods: A structured search of bibliographic databases from peer-reviewed literature was pursued focusing on autoimmunity in the brain and FTD. Results: One-hundred-fourteen papers were included in this review. The majority of studies (32) were represented by extensive literature revision on immunity, central nervous system (CNS) and autoimmunity; neuroimaging papers (11) in autoimmune diseases were evaluated, and immunomodulatory approaches (25) were revised. Six papers were found specifically related to FTD and autoimmune hypothesis, the other papers referring to current state of art on FTD. Conclusion: Overall this review contribute to expand the knowledge of a possible immune hypothesis in FTD, suggesting therapeutic perspectives in autoimmune related neurodegeneration, to reduce or revert the disease.

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.

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