scholarly journals 037 Frontotemporal dementia or frontal variant alzheimer’s disease? A case series

2019 ◽  
Vol 90 (e7) ◽  
pp. A13.1-A13
Author(s):  
Alice Powell ◽  
David Foxe ◽  
Glenda M Halliday ◽  
Olivier Piguet ◽  
John R Hodges ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Neuropsychiatric Symptoms ◽  
Neuropsychological Testing ◽  
Significant Proportion ◽  
Case Series ◽  
Imaging Features ◽  
Topographical Distribution ◽  
Visuospatial Skills

IntroductionAccurate prediction of the underlying neuropathology in behavioural variant frontotemporal dementia (bvFTD) is essential for future targeted therapy trials and prognostication. Alzheimer’s disease (AD) pathology has been reported in a significant proportion of patients with clinical bvFTD. We sought to determine whether detailed clinical and neuroradiological assessment was sufficient to distinguish bvFTD with AD pathology from bvFTD with frontotemporal lobar degeneration (FTLD).MethodsTwo patients with clinically diagnosed probable bvFTD but AD pathology at autopsy, were identified. The clinical, neuropsychological and imaging features of these patients were compared with those of ten patients with clinically probable bvFTD and proven FTLD pathology (tau, TDP-43, FUS).ResultsBoth patients with AD pathology presented with behavioural symptoms typical of bvFTD as well as memory impairment. Executive function, memory and visuospatial skills were impaired in both pathologic groups. Language skills were relatively spared in those with AD pathology. Neuropsychiatric symptoms were frequent in both groups but significant depression and anxiety were seen only in those with FTLD pathology. Dementia severity and caregiver burden were similar. The degree or topographical distribution of atrophy on MRI did not differ.ConclusionsAlzheimer’s pathology may cause bvFTD symptoms which are otherwise indistinguishable to those caused by FTLD pathology. While there may be subtle differences in patterns of cognitive deficits, standard neuropsychological testing is insufficient to discern the underlying pathology. Similarly, structural imaging cannot be used to reliably identify AD pathology. Better access to amyloid biomarkers may be needed to more accurately define bvFTD caused by AD pathology.

Mild Cognitive Impairment: A Neuropsychological Perspective

CNS Spectrums ◽  
2008 ◽  
Vol 13 (1) ◽  
pp. 56-64 ◽  
Author(s):  
Aaron P. Nelson ◽  
Margaret G. O'Connor
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cognitive Impairment ◽  
Executive Function ◽  
Mild Cognitive Impairment ◽  
Neuropsychiatric Symptoms ◽  
Neuropsychological Testing ◽  
Intellectual Ability ◽  
Transitional State ◽  
Visuospatial Skills

ABSTRACTMild cognitive impairment (MCI) is a clinical diagnosis in which deficits in cognitive function are evident but not of sufficient severity to warrant a diagnosis of dementia. For the majority of patients, MCI represents a transitional state between normal aging and mild dementia, usually Alzheimer's disease. Multiple subtypes of MCI are now recognized. In addition to presentations featuring memory impairment, symptoms in other cognitive domains (eg, executive function, language, visuospatial) have been identified. Neuropsychological testing can be extremely useful in making the MCI diagnosis and tracking the evolution of cognitive symptoms over time. A comprehensive test battery includes measures of baseline intellectual ability, attention, executive function, memory, language, visuospatial skills, and mood. Informant-bassed measures of neuropsychiatric symptoms, behaviors, and competency in instrumental activity are also included. Careful assessment can identify subtle deficits that may otherwise elude detection, particularly in individuals of superior baseline intellectual ability. As we move closer to disease-modifying therapy for Alzheimer's disease, early identification becomes critical for identifying patients who have an opportunity to benefit from treatment.

scholarly journals Death Rate Due to COVID-19 in Alzheimer’s Disease and Frontotemporal Dementia

2020 ◽  
Vol 78 (2) ◽  
pp. 537-541
Author(s):  
Jordi A. Matias-Guiu ◽  
Vanesa Pytel ◽  
Jorge Matías-Guiu
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Death Rate ◽  
Case Series ◽  
Care Homes ◽  
Relevant Factor ◽  
Increased Risk ◽  
Care Facilities ◽  
Probability Of Death

We aimed to evaluate the frequency and mortality of COVID-19 in patients with Alzheimer’s disease (AD) and frontotemporal dementia (FTD). We conducted an observational case series. We enrolled 204 patients, 15.2% of whom were diagnosed with COVID-19, and 41.9% of patients with the infection died. Patients with AD were older than patients with FTD (80.36±8.77 versus 72.00±8.35 years old) and had a higher prevalence of arterial hypertension (55.8% versus 26.3%). COVID-19 occurred in 7.3% of patients living at home, but 72.0% of those living at care homes. Living in care facilities and diagnosis of AD were independently associated with a higher probability of death. We found that living in care homes is the most relevant factor for an increased risk of COVID-19 infection and death, with AD patients exhibiting a higher risk than those with FTD.

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.

Caregiver burden in atypical dementias: comparing frontotemporal dementia, Creutzfeldt–Jakob disease, and Alzheimer's disease

2015 ◽  
Vol 28 (2) ◽  
pp. 269-273 ◽  
Author(s):  
Alice Uflacker ◽  
Mary C. Edmondson ◽  
Chiadi U. Onyike ◽  
Brian S. Appleby
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Caregiver Burden ◽  
Neuropsychiatric Symptoms ◽  
Published Data ◽  
Caregiver Distress ◽  
Severity Scores ◽  
Symptom Domains ◽  
Jakob Disease

ABSTRACTBackground:Caregiver burden is a significant issue in the treatment of dementia and a known contributor to institutionalization of patients with dementia. Published data have documented increased caregiver burden in behavioral variant frontotemporal dementia (bvFTD) compared to Alzheimer's disease (AD). Another atypical dementia with high-perceived caregiver burden is sporadic Creutzfeldt–Jakob disease (sCJD), but no formal studies have assessed this perception. The aim of this study was to compare caregiver burden across atypical dementia etiologies.Methods:76 adults with atypical dementia (young-onset AD [YOAD], bvFTD, language variant FTD [lvFTD], and sCJD) were administered an abbreviated version of the Zarit Burden Interview (ZBI), Neuropsychiatric Inventory (NPI-Q), and other assessment instruments during a five-year time period at Johns Hopkins Hospital (JHH). A Cox regression model examined differences between disease categories that impact mean ZBI scores.Results:Mean ZBI scores were significantly different between dementia etiologies, with bvFTD and sCJD having the highest caregiver burden (p = 0.026). Mean NPI-Q caregiver distress scores were highest in bvFTD and sCJD (p = 0.002), with sCJD and bvFTD also having the highest number of endorsed symptom domains (p = 0.012). On regression analyses, an interactive variable combining final diagnosis category and NPI-Q total severity score demonstrated statistically significant differences in mean ZBI scores for sCJD and bvFTD.Conclusions:This study demonstrates that bvFTD and sCJD have increased levels of caregiver burden, NPI-Q caregiver distress, total severity scores, and number of endorsed symptom domains. These results suggest that higher caregiver burden in bvFTD and sCJD are disease specific and possibly related to neuropsychiatric symptoms.

scholarly journals Simultaneous PET-MRI Studies of the Concordance of Atrophy and Hypometabolism in Syndromic Variants of Alzheimer’s Disease and Frontotemporal Dementia: An Extended Case Series

2015 ◽  
Vol 46 (3) ◽  
pp. 639-653 ◽  
Author(s):  
Kuven K. Moodley ◽  
Daniela Perani ◽  
Ludovico Minati ◽  
Pasquale Anthony Della Rosa ◽  
Frank Pennycook ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Case Series ◽  
Mri Studies

scholarly journals Demência fronto-temporal em paciente feminina de 56 anos: relato de caso

2019 ◽  
Vol 8 (3) ◽  
Author(s):  
Gabriel Pina Paiva ◽  
Fábio Henrique Ribeiro Maldonado ◽  
Amanda Oliva Spaziani
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Work Group ◽  
Neuropsychiatric Symptoms ◽  
The Elderly ◽  
Dementia Patient ◽  
Evaluation Instrument ◽  
Mutual Help ◽  
Diagnosis Of Dementia

A demência é uma das mais importantes causas de morbimortalidade entre os idosos e se caracteriza pelo declínio progressivo em múltiplos domínios cognitivos. Paciente do sexo feminino, 56 anos, iniciou quadro há 3 anos, caracterizado por apatia, anedonia e isolamento social. Procurou atendimento com médico que atribuiu sintomas a depressão. Contudo, não houve melhora. Há dois anos evoluiu com delírios persecutórios, confabulações, alucinação visual. Acompanhante notou que a paciente tinha dificuldades em se expressar e na compreensão. Devido à refratariedade ao tratamento foi solicitada avaliação de neurologista.  À consulta inicial, paciente apresentava-se orientada no tempo, espaço. Mini exame do estado mental 26/30 pontos. Fluência verbal semântica. Após 6 meses, evoluiu com empobrecimento do vocabulário. À época estava dependente de familiares para realização de atividades de vida diária. Na ressonância magnética encefálica apresentou atrofia cortical difusa, com predomínio em regiões frontais e temporais à esquerda. Atualmente está em uso de risperidona e memantina. A atrofia cerebral dos lobos frontais e temporais ou demência fronto temporal (DFT) afeta predominantemente o lobo frontal do cérebro, podendo se estender para o temporal. A patologia caracteriza-se por significativa alteração da personalidade e do comportamento, com relativa preservação das funções mnésticas e visuoespaciais. A linguagem é progressivamente afetada. A memória encontra-se preservada no início da doença e as alterações comportamentais e da personalidade são bastante significativas. A variante comportamental é a mais comum. Ela apresenta uma deterioração gradual da função executiva e da personalidade, enquanto a capacidade visuoespacial é afetada apenas em estádios avançados.Descritores: Transtornos Neurocognitivos; Demência Frontotemporal; Testes de Estado Mental e Demência.ReferênciasCarrabba LHG, Menta C, Fasolin EM, Loureiro F, Gomes I. Características psicométricas das versões completa e reduzida do IQCODE-BR em idosos de baixa renda e escolaridade. Rev bras geriatr gerontol. 2015;18(4):715-23.Lopes MCBT, Lage JSS, Vancini-Campanharo CR, Okuno MFP, Batista REA. Factors associated with functional impairment of elderly patients in the emergency departments. Einstein. 2015;13(2):209-14.Trindade APNT, Barboza MA, Oliveira FB, Borges APO. Repercussão do declínio cognitivo na capacidade funcional em idosos institucionalizados e não institucionalizados. Fisioter mov. 2013;26(2):281-89.Santos JI, Rodrigues Junior C, Zogheib JB, Malachias MVB, Rezende BA.  Assessment of hemodynamic and vascular parameters in Alzheimer's disease, vascular dementia and mild cognitive abnormalities: a pilot study. Rev bras geriatr gerontol. 2017;20(5):670-78.Burlá C, Camarano AA, Kanso S, Fernandes D, Nunes R. Panorama prospectivo das demências no Brasil: um enfoque demográfico. Ciênc saúde coletiva. 2013;18(10):2949-56.Costa GD, Souza RA, Yamashita CH, Pinheiro JCF, Alvarenga MRM, Oliveira MAC. Evaluation of professional knowledge and attitudes on dementia patient care: a trans-cultural adaptation of an evaluation instrument. Rev esc enferm USP. 2015;49(2):298-308.Bosch B, Isidro R, Zayas Ll, Hernández T, Ulloa E. Algunos determinantes sociales y su impacto en las demencias. Rev Cubana Salud Pública. 2017;43(3):449-60.Josviak ND, Batistela MS, Simão-Silva DP, Bono GF, Furtado-Alle L, Souza RLR. Revisão dos principais genes e proteínas associadas à demência frontotemporal tau-positiva. Rev bras geriatr gerontol. 2015;18(1):201-11.McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack CR Jr, Kawas CH, et al. The diagnosis of dementia due to Alzheimer’s disease: Recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement. 2011;7(3):263-69.Pires FRO, Santos SMA, Mello ALSF, Silva KM. Mutual Help Group for Family Members of Older Adults with Dementia: Unveiling perspectives. Texto contexto - enferm.. 2017;26(2):e00310016.Storti LB, Quintino DT, Silva NM, Kusumota L, Marques S. Neuropsychiatric symptoms of the elderly with Alzheimer's disease and the family caregivers' distress. Rev Latino-Am Enfermagem. 2016;24:e2751.Teixeira-Jr AL, Salgado JV. Demência fronto-temporal: aspectos clínicos e terapêuticos. Rev psiquiatr Rio Gd Sul. 2006;28(1):69-76.Mendes RAB. Demência Frontotemporal. Evolução do conceito e desafios diagnósticos [dissertação]. Covilhã: Faculdade de Medicina,Universidade da Beira Interior (UBI); 2015.Moreira S, Duarte S, Moreira I, Santos E. et al. Variante comportamental da demência frontotemporal: relato de caso. Rev Port Med Geral Fam. 2017;33(2):155-61.McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ et al. Clinical and pathological diagnosis of frontotemporal dementia: Report of the work group on frontotemporal dementia and pick's disease. Arch Neurol. 2001;58(11):1803-9.Rivas Nieto JC. Frontotemporal dementia: clinical, neuropsychological, and neuroimaging description. Colomb. Med (Cali). 2014;45(3):122-26.Fernádez-Matarrubia M, Matías-Guiu JA, Moreno-Ramos T,  Matías-Guiu J. Demencia frontotemporal variante conductual: aproximación clínica y terapéutica. Neurología. 2014;29(8):464-72.Lanata SC, Miller BL. The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry. J Neurol Neurosurg Psychiatry. 2016;87(5):501-11.

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 A systematic review of the prevalence of depression, anxiety, and apathy in frontotemporal dementia, atypical and young-onset Alzheimer’s disease, and inherited dementia

2020 ◽  
pp. 1-20
Author(s):  
Jessica D. Collins ◽  
Susie M. D. Henley ◽  
Aida Suárez-González
Keyword(s):  
Systematic Review ◽  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Neuropsychiatric Symptoms ◽  
Primary Progressive Aphasia ◽  
Small Sample ◽  
Cortical Atrophy ◽  
Sample Sizes ◽  
Young Onset

ABSTRACT Objectives: Depression, anxiety, and apathy are the most commonly reported neuropsychiatric symptoms (NPS) in Alzheimer’s disease (AD). Understanding their prevalence in rarer dementias such as frontotemporal dementia (FTD), primary progressive aphasia (PPA), posterior cortical atrophy (PCA), young-onset AD (YOAD), and inherited dementias has implications for both clinical practice and research. In this study, we aimed to examine the current state of knowledge of the prevalence of these three NPS in less prevalent dementias. Design: We conducted a systematic review based on searches of EMBASE, PsycINFO, and PubMed up to September 2019. Results: 47 articles meeting inclusion criteria were identified. Depression, anxiety, and apathy were commonly reported across the phenotypes studied but their prevalence showed large variation between studies. Apathy showed the highest reported frequency in FTD (50–100% across studies), behavioral variant frontotemporal dementia (bvFTD) (73–100%), and YOAD (44–100%). Anxiety was frequently reported in FTD (0–100%) and bvFTD (19–63%). Depression showed the highest prevalence in FTD (7–69%) and YOAD (11–55%). Among the three variants of PPA, sv-PPA is the one most investigated (seven articles). Three or fewer articles were identified examining NPS in the remaining PPA variants, PCA, familial AD, and familial FTD. Inconsistency in the tools used to measure symptoms and small sample sizes were common methodological limitations. Conclusions: Future studies should consider the inclusion of larger sample sizes (e.g. through multicenter collaborations) and the use of harmonized protocols that include the combination of caregiver and patient-derived measures and symptom-specific questionnaires. More research is needed on the phenotype-specific barriers and facilitators for people living with dementia to successfully engage in self-reports of NPS.

Neuropsychiatric Symptoms, Caregiver Burden and Distress in Behavioral-Variant Frontotemporal Dementia and Alzheimer's Disease

2015 ◽  
Vol 40 (5-6) ◽  
pp. 268-275 ◽  
Author(s):  
Thais Bento Lima-Silva ◽  
Valéria Santoro Bahia ◽  
Viviane Amaral Carvalho ◽  
Henrique Cerqueira Guimarães ◽  
Paulo Caramelli ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Frontotemporal Dementia ◽  
Sex Education ◽  
Caregiver Burden ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Clinical Dementia Rating ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Significant Difference

Background/Aims: We aimed to compare caregiver burden and distress in behavioral-variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD) and to investigate which factors contribute to caregivers' burden and distress. Methods: Fifty patients and their caregivers were invited to participate. Among the patients, 20 had a diagnosis of bvFTD and 30 had AD. Caregivers and patients were statistically equivalent for age, sex, education and dementia severity according to Clinical Dementia Rating. The protocol included the Short Zarit Burden Inventory, the Neuropsychiatric Inventory (NPI), Disability Assessment for Dementia (DAD), the Cornell Scale for Depression in Dementia (CSDD), Addenbrooke's Cognitive Examination-Revised, the Executive Interview with 25 Items, Direct Assessment of Functional Status and the Geriatric Anxiety Inventory (GAI). Results: In the NPI, caregivers of bvFTD patients reported a higher presence and severity of neuropsychiatric symptoms and caregiver distress compared to caregivers of AD patients. There was no significant difference in the perceived burden. In bvFTD, DAD and GAI scores were significantly correlated with burden, whereas in AD, burden was correlated with CSDD and NPI scores. Psychiatric symptoms were associated with distress in both groups. Conclusions: Caregivers of bvFTD patients experienced higher levels of distress than caregivers of AD patients. Patients' functional limitations were associated with burden of caregivers of bvFTD patients, whereas neuropsychiatric symptoms were associated with caregiver strain in both groups.

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