Current clinical and research practices on frontotemporal dementia in Brazil: a national survey.

BACKGROUND: Frontotemporal dementia (FTD) is a frequent cause of young-onset dementia and represents a major challenge for the diagnosis and clinical management. It is essential to evaluate the difficulties faced by physicians on the diagnostic workup and on patient care. OBJECTIVE: The aim of this study was to investigate the current practices and the local limits on the diagnosis and management of FTD in Brazil. METHODS: We elaborated an online survey, composed of 29 questions and divided in four parts, comprising questions about existing health facilities, clinical practices related to FTD, and suggestions to increment the national research on FTD. The invitation to participate was sent by email to all neurologists affiliated to the Brazilian Academy of Neurology (n = 3658), and to all physicians who attended the XII Meeting of Researchers on Alzheimer's disease, in 2019 (n = 187). The invitation was also diffused through social media. RESULTS: 256 Brazilian physicians answered the questionnaire. The three most relevant disorders for the differential diagnosis of FTD were Alzheimer's disease (AD) (n = 211), bipolar disorder (n = 117) and dementia with Lewy bodies (n = 92). Most respondents (125/256) reported the difficulty in performing genetic testing as the main limit in the diagnostic of FTD. 93% and 63% of participants considered that the assessment of social cognition and AD CSF biomarkers are useful for the diagnosis of FTD, respectively. CONCLUSIONS: The present study may provide valuable insights for the medical education and clinical training of physicians, and to foster future research on FTD in Brazil.

ANTECEDENTES: A demência frontotemporal (DFT) é causa frequente de demência pré-senil e representa um desafio em termos de diagnóstico e de manejo clínico. É essencial avaliar as dificuldades existentes na propedêutica e nos cuidados médicos. OBJETIVO: Investigar as práticas médicas e as dificuldades para diagnóstico e manejo da DFT no Brasil. MéTODOS: Elaborou-se um questionário online, composto de 29 questões, divididas em quatro partes, com perguntas sobre infraestrutura existente, práticas clínicas relacionadas à DFT e sugestões para desenvolver a pesquisa nacional na área. O convite para participação foi enviado por e-mail a todos neurologistas afiliados à Academia Brasileira de Neurologia (n = 3658), e aos médicos que participaram da XII Reunião de Pesquisadores de Doença de Alzheimer, em 2019 (n = 187). O convite também foi divulgado através de mídias sociais. RESULTADOS: 256 médicos brasileiros responderam o questionário. Os três principais diagnósticos diferenciais de DFT foram doença de Alzheimer (n = 211), transtorno bipolar (n = 117) e demência com corpos de Lewy (n = 92). A maior parte dos respondedores (125/256) apontou a dificuldade em realizar testagem genética como o maior limite no diagnóstico de DFT. 93% e 63% dos respondedores indicaram que a avaliação de cognição social e o uso de biomarcadores liquóricos de doença de Alzheimer são úteis no diagnóstico de DFT, respectivamente. CONCLUSõES: Estes resultados devem ser considerados na educação e treinamento médicos, e no desenvolvimento da pesquisa brasileira em DFT.

Current clinical and research practices on frontotemporal dementia in Brazil: a national survey / Práticas clínicas e cenário de pesquisa em demência frontotemporal no Brasil: uma enquete nacional

Abstract Background Frontotemporal dementia (FTD) is a frequent cause of young-onset dementia and represents a major challenge for the diagnosis and clinical management. It is essential to evaluate the difficulties faced by physicians on the diagnostic workup and on patient care. Objective The aim of this study was to investigate the current practices and the local limits on the diagnosis and management of FTD in Brazil. Methods We elaborated an online survey, composed of 29 questions and divided in four parts, comprising questions about existing health facilities, clinical practices related to FTD, and suggestions to increment the national research on FTD. The invitation to participate was sent by email to all neurologists affiliated to the Brazilian Academy of Neurology (n = 3658), and to all physicians who attended the XII Meeting of Researchers on Alzheimer's disease, in 2019 (n = 187). The invitation was also diffused through social media. Results 256 Brazilian physicians answered the questionnaire. The three most relevant disorders for the differential diagnosis of FTD were Alzheimer's disease (AD) (n = 211), bipolar disorder (n = 117) and dementia with Lewy bodies (n = 92). Most respondents (125/256) reported the difficulty in performing genetic testing as the main limit in the diagnostic of FTD. 93% and 63% of participants considered that the assessment of social cognition and AD CSF biomarkers are useful for the diagnosis of FTD, respectively. Conclusions The present study may provide valuable insights for the medical education and clinical training of physicians, and to foster future research on FTD in Brazil.

Resumo Antecedentes A demência frontotemporal (DFT) é causa frequente de demência pré-senil e representa um desafio em termos de diagnóstico e de manejo clínico. É essencial avaliar as dificuldades existentes na propedêutica e nos cuidados médicos. Objetivo Investigar as práticas médicas e as dificuldades para diagnóstico e manejo da DFT no Brasil. Métodos Elaborou-se um questionário online, composto de 29 questões, divididas em quatro partes, com perguntas sobre infraestrutura existente, práticas clínicas relacionadas à DFT e sugestões para desenvolver a pesquisa nacional na área. O convite para participação foi enviado por e-mail a todos neurologistas afiliados à Academia Brasileira de Neurologia (n = 3658), e aos médicos que participaram da XII Reunião de Pesquisadores de Doença de Alzheimer, em 2019 (n = 187). O convite também foi divulgado através de mídias sociais. Resultados 256 médicos brasileiros responderam o questionário. Os três principais diagnósticos diferenciais de DFT foram doença de Alzheimer (n = 211), transtorno bipolar (n = 117) e demência com corpos de Lewy (n = 92). A maior parte dos respondedores (125/256) apontou a dificuldade em realizar testagem genética como o maior limite no diagnóstico de DFT. 93% e 63% dos respondedores indicaram que a avaliação de cognição social e o uso de biomarcadores liquóricos de doença de Alzheimer são úteis no diagnóstico de DFT, respectivamente. Conclusões Estes resultados devem ser considerados na educação e treinamento médicos, e no desenvolvimento da pesquisa brasileira em DFT.

Diagnosis of frontotemporal dementia: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology.

"Frontotemporal dementia" (FTD) is a clinical syndrome characterized by the focal involvement of the frontal and/or temporal lobes. FTD has three clinical phenotypes: the behavioral variant and two linguistic subtypes, namely, non-fluent/agrammatic primary progressive aphasia (PPA-NF/A) and semantic PPA (PPA-S). FTD is the second most common cause of dementia in individuals under the age of 65 years. This article presents recommendations for the diagnosis of FTD in the Brazilian scenario, considering the three levels of complexity of the health system: primary health care, secondary and tertiary levels. Diagnostic guidelines are proposed, including cognitive testing, behavioral and language assessments, laboratory tests, and neuroimaging.

A "demência frontotemporal" (DFT) é uma síndrome clínica, cujo denominador comum é o acometimento focal dos lobos frontais e/ou temporais. A DFT tem três fenótipos clínicos distintos: a variante comportamental e dois subtipos linguísticos, a saber, a afasia progressiva primária não-fluente/agramática (APP-NF/A) e a afasia progressiva primária semântica (APP-S). A DFT é a segunda causa mais comum de demência em indivíduos com idade inferior a 65 anos, após a doença de Alzheimer. O presente artigo apresenta recomendações para diagnóstico da DFT no cenário brasileiro, considerando os três níveis de complexidade do sistema de saúde: atenção primária à saúde e níveis secundários. São propostos protocolos de investigação diagnóstica abrangendo testagem cognitiva, avaliação comportamental, avaliação fonoaudiológica, exames laboratoriais e de neuroimagem.

Diagnóstico da demência frontotemporal: recomendações do Departamento Científico de Neurologia Cognitiva e do Envelhecimento da Academia Brasileira de Neurologia / Diagnosis of frontotemporal dementia: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology

RESUMO A "demência frontotemporal" (DFT) é uma síndrome clínica, cujo denominador comum é o acometimento focal dos lobos frontais e/ou temporais. A DFT tem três fenótipos clínicos distintos: a variante comportamental e dois subtipos linguísticos, a saber, a afasia progressiva primária não-fluente/agramática (APP-NF/A) e a afasia progressiva primária semântica (APP-S). A DFT é a segunda causa mais comum de demência em indivíduos com idade inferior a 65 anos, após a doença de Alzheimer. O presente artigo apresenta recomendações para diagnóstico da DFT no cenário brasileiro, considerando os três níveis de complexidade do sistema de saúde: atenção primária à saúde e níveis secundários. São propostos protocolos de investigação diagnóstica abrangendo testagem cognitiva, avaliação comportamental, avaliação fonoaudiológica, exames laboratoriais e de neuroimagem.

ABSTRACT "Frontotemporal dementia" (FTD) is a clinical syndrome characterized by the focal involvement of the frontal and/or temporal lobes. FTD has three clinical phenotypes: the behavioral variant and two linguistic subtypes, namely, non-fluent/agrammatic primary progressive aphasia (PPA-NF/A) and semantic PPA (PPA-S). FTD is the second most common cause of dementia in individuals under the age of 65 years. This article presents recommendations for the diagnosis of FTD in the Brazilian scenario, considering the three levels of complexity of the health system: primary health care, secondary and tertiary levels. Diagnostic guidelines are proposed, including cognitive testing, behavioral and language assessments, laboratory tests, and neuroimaging.

Changes in personality traits in patients with Alzheimer's Disease.

Changes in personality traits in patients with Alzheimer's disease (AD) are extremely common throughout the course of the pathology, and these behavioral changes present themselves as challenges in clinical management and as a significant cause of caregivers' burden. Objective: Using a personality inventory based on the five-factor model of personality, this study aimed to assesses the change in these factors by comparing the premorbid and current personality of individuals recently diagnosed with AD. Methods: A total of 30 AD patients were recruited, and their respective family members responded to the personality inventory at home through a hosted site. The patients were also divided into two groups according to the Clinical Dementia Rating (CDR): mild dementia (CDR 1) and moderate dementia (CDR 2). Results: Among all patients, there was a significant increase in neuroticism factor levels and a significant decrease in the extraversion, conscientiousness, openness, and socialization factors. When comparing the groups, only the extraversion factor showed a difference, with CDR 1 group accusing a higher change in scores. Higher scores in the factor neuroticism in the premorbid personality correlated with the current severity of the disease. Conclusions: This research draws the attention of family members and health professionals to changes in personality traits or behavior of relatives or patients, because it can reflect an underlying neurodegenerative process.

Mudanças em traços de personalidade em pacientes com doença de Alzheimer (DA) são extremamente comuns ao longo do curso da referida patologia, e essas alterações comportamentais apresentam-se como desafios no manejo clínico e como causa significativa de esgotamento dos cuidadores. Objetivo: Por meio de um inventário de personalidade baseado nos cinco fatores de personalidade, este estudo avalia a mudança nos escores desses fatores comparando a personalidade pré-mórbida e a atual dos indivíduos com DA. Métodos: O total de 30 pacientes com DA foi recrutado, e seus familiares responderam ao inventário de personalidade. Os pacientes também foram divididos em dois grupos conforme a avaliação clínica da demência: demência leve (CDR1) e demência moderada (CDR2). Resultados: Em todos os pacientes, houve aumento significativo nos escores do fator neuroticismo e decréscimos significativos nos fatores extroversão, realização, abertura e socialização. Quando feita a comparação entre grupos, apenas o fator extroversão apresentou diferença, com o grupo CDR 1 mostrando maiores mudanças nos escores. Os níveis do fator neuroticismo da personalidade pré-mórbida correlacionaram-se com a gravidade da doença no momento do diagnóstico. Conclusões: Este estudo procura esclarecer aos familiares e profissionais de saúde que mudanças em traços de personalidade de seus parentes ou pacientes podem refletir processos neurodegenerativos subjacentes.

Changes in personality traits in patients with Alzheimer's Disease / Mudanças nos traços de personalidade em pacientes com doença de alzheimer

ABSTRACT. Changes in personality traits in patients with Alzheimer's disease (AD) are extremely common throughout the course of the pathology, and these behavioral changes present themselves as challenges in clinical management and as a significant cause of caregivers' burden. Objective: Using a personality inventory based on the five-factor model of personality, this study aimed to assesses the change in these factors by comparing the premorbid and current personality of individuals recently diagnosed with AD. Methods: A total of 30 AD patients were recruited, and their respective family members responded to the personality inventory at home through a hosted site. The patients were also divided into two groups according to the Clinical Dementia Rating (CDR): mild dementia (CDR 1) and moderate dementia (CDR 2). Results: Among all patients, there was a significant increase in neuroticism factor levels and a significant decrease in the extraversion, conscientiousness, openness, and socialization factors. When comparing the groups, only the extraversion factor showed a difference, with CDR 1 group accusing a higher change in scores. Higher scores in the factor neuroticism in the premorbid personality correlated with the current severity of the disease. Conclusions: This research draws the attention of family members and health professionals to changes in personality traits or behavior of relatives or patients, because it can reflect an underlying neurodegenerative process.

RESUMO. Mudanças em traços de personalidade em pacientes com doença de Alzheimer (DA) são extremamente comuns ao longo do curso da referida patologia, e essas alterações comportamentais apresentam-se como desafios no manejo clínico e como causa significativa de esgotamento dos cuidadores. Objetivo: Por meio de um inventário de personalidade baseado nos cinco fatores de personalidade, este estudo avalia a mudança nos escores desses fatores comparando a personalidade pré-mórbida e a atual dos indivíduos com DA. Métodos: O total de 30 pacientes com DA foi recrutado, e seus familiares responderam ao inventário de personalidade. Os pacientes também foram divididos em dois grupos conforme a avaliação clínica da demência: demência leve (CDR1) e demência moderada (CDR2). Resultados: Em todos os pacientes, houve aumento significativo nos escores do fator neuroticismo e decréscimos significativos nos fatores extroversão, realização, abertura e socialização. Quando feita a comparação entre grupos, apenas o fator extroversão apresentou diferença, com o grupo CDR 1 mostrando maiores mudanças nos escores. Os níveis do fator neuroticismo da personalidade pré-mórbida correlacionaram-se com a gravidade da doença no momento do diagnóstico. Conclusões: Este estudo procura esclarecer aos familiares e profissionais de saúde que mudanças em traços de personalidade de seus parentes ou pacientes podem refletir processos neurodegenerativos subjacentes.

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

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.

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

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.

Swallowing in behavioral variant frontotemporal dementia.

BACKGROUND: Swallowing and feeding problems may occur with the progression of behavioral variant frontotemporal dementia (bvFTD) and can impair the anticipatory and oral preparatory phases of swallowing. OBJECTIVE: To characterize swallowing problems and the feeding situation of patients with bvFTD and to correlate the swallowing problems with functionality, executive functions, cognitive and behavioral features. METHODS: Consecutive outpatients with bvFTD in mild, moderate and severe dementia stages were recruited along with their caregivers. Patients and caregivers were screened with the following scales: "Mini-Mental State Examination", "Severe Mini-Mental State Examination", "FTLD-modified Clinical Dementia Rating", "Neuropsychiatric Inventory", "Frontal Assessment Battery", "Index of Independence in Activities of Daily Living", "Swallowing Rating Scale" and "Assessment of Feeding and Swallowing Difficulties in Dementia". RESULTS: Overall, thirty patients with bvFTD were included along with their caregivers. Patients with bvFTD showed feeding and swallowing difficulties such as: messy to eat, passivity, coughing and choking, difficulty with some food consistencies and with specific food. Swallowing problems in bvFTD correlated with impaired functionality (p<0.05) and cognition (p<0.05), executive dysfunction (p<0.01) and behavioral features (p<0.01). Caregivers had great difficulty in managing the feeding situation during mealtime, with different characteristics in each dementia stage. CONCLUSION: Patients with bvFTD had inappropriate speed eating, passivity, coughing and choking starting in the mild dementia stage, and these problems worsen in the severe stage. Such difficulties affected caregiver performance during mealtime. The correlations indicated that swallowing difficulties tend to follow cognitive and behavioral decline in patients with bvFTD.

Swallowing in behavioral variant frontotemporal dementia / Deglutição na variante comportamental da demência frontotemporal

ABSTRACT Background: Swallowing and feeding problems may occur with the progression of behavioral variant frontotemporal dementia (bvFTD) and can impair the anticipatory and oral preparatory phases of swallowing. Objective: To characterize swallowing problems and the feeding situation of patients with bvFTD and to correlate the swallowing problems with functionality, executive functions, cognitive and behavioral features. Methods: Consecutive outpatients with bvFTD in mild, moderate and severe dementia stages were recruited along with their caregivers. Patients and caregivers were screened with the following scales: "Mini-Mental State Examination", "Severe Mini-Mental State Examination", "FTLD-modified Clinical Dementia Rating", "Neuropsychiatric Inventory", "Frontal Assessment Battery", "Index of Independence in Activities of Daily Living", "Swallowing Rating Scale" and "Assessment of Feeding and Swallowing Difficulties in Dementia". Results: Overall, thirty patients with bvFTD were included along with their caregivers. Patients with bvFTD showed feeding and swallowing difficulties such as: messy to eat, passivity, coughing and choking, difficulty with some food consistencies and with specific food. Swallowing problems in bvFTD correlated with impaired functionality (p<0.05) and cognition (p<0.05), executive dysfunction (p<0.01) and behavioral features (p<0.01). Caregivers had great difficulty in managing the feeding situation during mealtime, with different characteristics in each dementia stage. Conclusion: Patients with bvFTD had inappropriate speed eating, passivity, coughing and choking starting in the mild dementia stage, and these problems worsen in the severe stage. Such difficulties affected caregiver performance during mealtime. The correlations indicated that swallowing difficulties tend to follow cognitive and behavioral decline in patients with bvFTD.

RESUMO Introdução: Os problemas na situação de alimentação e deglutição podem ocorrer com a progressão da variante comportamental da demência frontotemporal (DFT-vc) e alterar as fases antecipatória e preparatória oral da deglutição. Objetivo: Caracterizar os problemas de deglutição e a situação de alimentação de pacientes com DFT-vc e correlacionar os problemas de deglutição com a funcionalidade, funções executivas, aspectos cognitivos e comportamentais. Métodos: Foram recrutados pacientes ambulatoriais com DFT-vc nas fases leve, moderada e grave da demência, e seus respectivos cuidadores. Os pacientes e cuidadores foram avaliados com as escalas: "Mini-Exame do Estado Mental", "Mini-Exame do Estado Mental Grave", "Escala de Avaliação Clínica da Demência Modificada - DFT", "Inventário Neuropsiquiátrico", "Bateria de Avaliação Frontal", "Índice de Independência nas Atividades da Vida Diária", "Escala Funcional de Avaliação da Deglutição" e "Avaliação das Dificuldades de Alimentação e Deglutição na Demência". Resultados: Foram incluídos 30 pacientes com DFT-vc, e seus cuidadores. Pacientes com DFT-vc apresentaram dificuldades de alimentação e deglutição como: confusão na alimentação, passividade, tosse e asfixia, dificuldades com algumas consistências alimentares e alimentos específicos. Problemas de deglutição na DFT-vc correlacionaram-se com funcionalidade prejudicada (p<0,05) e cognição (p<0,05), disfunção executiva (p<0,01) e características comportamentais (p<0,01). Os cuidadores tiveram grande dificuldade em gerenciar a situação de alimentação diante de diferentes problemas em cada fase da demência. Conclusão: Pacientes com DFT-vc apresentaram velocidade de alimentação inapropriada, passividade, tosse e engasgos já na fase leve da doença, com piora na fase grave. As correlações indicaram que as alterações de deglutição tendem a seguir o declínio cognitivo e comportamental na DFT-vc.

Disease Progression in Frontotemporal Dementia and Alzheimer Disease: The Contribution of Staging Scales.

INTRODUCTION: There is a shortage of validated instruments to estimate disease progression in frontotemporal dementia (FTD). OBJECTIVES: To evaluate the ability of the FTD Rating Scale (FTD-FRS) to detect functional and behavioral changes in patients diagnosed with the behavioral variant of FTD (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD) after 12 months of the initial evaluation, compared to the Clinical Dementia Rating scale-frontotemporal lobar degeneration (CDR-FTLD) and the original Clinical Dementia Rating scale (CDR). METHODS: The sample consisted of 70 individuals, aged 40+ years, with at least 2 years of schooling, 31 with the diagnosis of bvFTD, 12 with PPA (8 with semantic variant and 4 with non-fluent variant), and 27 with AD. The FTD-FRS, the CDR, and the 2 additional CDR-FTLD items were completed by a clinician, based on the information provided by the caregiver with frequent contact with the patient. The Addenbrooke Cognitive Examination-Revised was completed by patients. After 12 months, the same protocol was applied. RESULTS: The FTD-FRS, CDR-FTLD, and CDR detected significant decline after 12 months in the 3 clinical groups (exception: FTD-FRS for PPA). The CDR was less sensitive to severe disease stages. CONCLUSIONS: The FTD-FRS and the CDR-FTLD are especially useful tools for dementia staging in AD and in the FTD spectrum.

Profiles of cognitive impairment in the continuum from normal cognition to Alzheimer's clinical syndrome: Contributions of the short-term memory binding tests.

BACKGROUND: Short-term memory binding (STMB) tests assess conjunctive binding, in which participants should remember the integration of features, such as shapes (or objects) and colors, forming a unique representation in memory. In this study, we investigated two STMB paradigms: change detection (CD) and free recall (FR). OBJECTIVE: To investigate the cognitive profile in the CD and FR tasks of three diagnostic groups: cognitively unimpaired (CU), mild cognitive impairment (MCI), and Alzheimer's clinical syndrome (ACS). In addition, we aimed to calculate and compare the accuracy of the CD and FR tasks to identify MCI and ACS. METHODS: Participants were 24 CU, 24 MCI, and 37 ACS. The cognitive scores of the clinical groups were compared using analysis of variance (ANOVA) and receiver-operating characteristic (ROC) analyses were carried out to verify the accuracy of the STMB tasks. RESULTS: In the CD task, CU was different from MCI and ACS (CU > MCI = ACS), while in the FR task all groups were different (CU > MCI > ACS). The ROC analyses showed an area under the curve (AUC) of 0.855 comparing CU with MCI for the CD task and 0.975 for the FR. The AUC comparing CU and ACS was 0.924 for the CD and 0.973 for the FR task. The FR task showed better accuracy to identify MCI patients, and the same accuracy to detect ACS. CONCLUSION: The present findings indicate that impairments in CD and FR of bound representations are features of the cognitive profiles of MCI and ACS patients.

Recalling feature bindings differentiates Alzheimer's disease from frontotemporal dementia.

It has been challenging to identify clinical cognitive markers that can differentiate patients with Alzheimer's disease (AD) from those with behavioral variant frontotemporal dementia (bvFTD). The short-term memory binding (STMB) test assesses the ability to integrate colors and shapes into unified representations and to hold them temporarily during online performance. The objective of this study is to investigate whether free recall deficits during short-term memory binding (STMB) test can differentiate patients with AD from those with bvFTD and controls. Participants were 32 cognitively intact adults, 35 individuals with AD and 18 with bvFTD. All patients were in the mild dementia stage. Receiver-operating characteristic (ROC) analyses were used to examine the diagnostic accuracy of the STMB. The results showed that AD patients performed significantly worse than controls and bvFTD patients in the STMB test, while the latter groups showed equivalent performance. The bound condition of the STMB test showed an AUC of 0.853, with 84.4% of sensitivity and 80% of specificity to discriminate AD from controls and an AUC of 0.794, with 72.2% of sensitivity and 80% of specificity to differentiate AD from bvFTD. Binding deficits seem specific to AD. The free recall version of the STMB test can be used for clinical purposes and may aid in the differential diagnosis of AD. Findings support the view that the STMB may be a suitable cognitive marker for AD.

Swallowing in primary progressive aphasia.

BACKGROUND: Few studies have described characteristics of swallowing in primary progressive aphasia (PPA) and its variants. OBJECTIVE: To describe and characterize swallowing and eating behaviors of patients with PPA, as well as their correlates with neuropsychiatric symptoms and patterns of communication. METHODS: We studied 16 patients with PPA and 16 their caregivers. PPA was subdivided in agrammatic variant (PPA-G), semantic variant (PPA-S) and logopenic variant (PPA-L). All patients and their caregivers were screened with the following scales: "Assessment of Feeding and Swallowing Difficulties in Dementia", "Neuropsychiatric Inventory", and "Functional Outcome Questionnaire for Aphasia". RESULTS: Patients with PPA-S had diverse swallowing problems such as drooling of saliva or food, multiple swallows, delayed swallow and choking, all of which correlated with anxiety, apathy and aberrant motor behavior. Patients with PPA-G and PPA-L had choking and delayed swallow, respectively. Disturbances in eating behaviors were more frequent in the group with PPA-L, and they correlated with difficulties in patterns of communication. CONCLUSIONS: All variants showed swallowing difficulties and they were more frequent in PPA-S. Further studies with larger samples of patients are needed to better characterize swallowing problems and their consequences in the different variants of PPA.

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

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.

Direct and indirect assessments of activities of daily living in behavioral variant frontotemporal dementia and Alzheimer disease.

BACKGROUND: There is limited information about the functional profile of behavioral variant frontotemporal dementia (bvFTD). OBJECTIVE: To compare direct and indirect assessments of activities of daily living (ADLs) in bvFTD and Alzheimer disease (AD) and their relationship with cognitive performance. METHODS: In all, 20 patients with bvFTD, 30 patients with AD, and 34 normal controls (NCs), matched for age, education, and severity of dementia, completed the Direct Assessment of Functional Performance (DAFS-BR) and usual cognitive measures. The Disability Assessment for Dementia (DAD) was completed by caregivers. RESULTS: In DAFS-BR, patients with bvFTD and AD had similar performance but lower than NCs. In DAD, there were no significant differences for effective performance, but patients with bvFTD had lower scores for initiation and planning/organization. Patients with bvFTD were less impaired than AD in cognition. CONCLUSION: Functional changes in bvFTD seem to be better documented by indirect measures.

Performance of patients with frontotemporal lobar degeneration on artistic tasks: A pilot study / Desempenho de pacientes com degeneração lobar frontotemporal em tarefa artística: Estudo piloto

Several studies have addressed visuospatial and executive skills in artistic activities in Frontotemporal Lobar Degeneration (FTLD) and Alzheimer's disease (AD).OBJECTIVE: To investigate the performance of FTLD patients compared to controls on two artistic tasks. METHODS: Four FTLD patients with mean age of 57 (8.7) years and schooling of 12.2 (4.5) years plus 10 controls with mean age of 62.9 (8.6) years and schooling of 12.3 (4.6) years, were assessed using the Lowenstein Occupational Therapy Cognitive Assessment (LOTCA) and by a three-stage artistic protocol including visual observation, copying and collage, based on a Sisley painting. RESULTS: FTLD patients had lower scores than controls on Visuospatial Perception, Copy, Collage, Examiner's Observation, and Total, showing distinct patterns of performance according to FTLD sub-type: semantic PPA, nonfluent PPA and bvFTD. CONCLUSION: FTLD patients presented impairment in the visuospatial and executive skills required to perform artistic tasks. We demonstrated that the application of the instrument as a complimentary method for assessing cognitive skills in this group of patients is possible. Further studies addressing larger and more homogeneous samples of FTLD patients as well as other dementias are warranted.

Vários estudos relatam as habilidades visuoespaciais em atividades artísticas em Degeneração Lobar Frontotemporal (DLFT) e Demência de Alzheimer (DA). OBJETIVO: Investigar as alterações no desempenho de pacientes com DLFT comparados a controles em tarefas artísticas. MÉTODOS: Quatro pacientes com DLFT (idade média de 57 (8,7) e escolaridade de 12,2 (4,5) anos) e 10 controles (idade média de 62,9 (86) e escolaridade de 12,3 (4,6) anos) foram avaliados através da Bateria de Avaliação Cognitiva de Terapia Ocupacional Lowenstein (LOTCA) e um protocolo de arte em três estágios: observação visual, cópia colorida e colagem baseada em uma pintura de Sisley. RESULTADOS: Os pacientes com DLFT tiveram menores pontuações em Percepção Visoespacial, Cópia, Colagem, Observação do examinador e Total, mostrando distintos tipos de desempenho nos subtipos de DFT variante comportamental, APP não fluente e APP semântica. CONCLUSÃO: Os pacientes com DLFT apresentaram prejuízo nas habilidades executivas e visuoespaciais necessárias ao desempenho nas tarefas artísticas propostas. Evidenciamos ser possível a aplicação deste instrumento como método de avaliação complementar de habilidades cognitivas neste grupo de pacientes. Novos estudos em um grupo maior e mais homogêneo de pacientes com DLFT, bem como em outras demências, são necessários.

Performance of patients with frontotemporal lobar degeneration on artistic tasks: A pilot study.

Several studies have addressed visuospatial and executive skills in artistic activities in Frontotemporal Lobar Degeneration (FTLD) and Alzheimer's disease (AD). OBJECTIVE: To investigate the performance of FTLD patients compared to controls on two artistic tasks. METHODS: Four FTLD patients with mean age of 57 (8.7) years and schooling of 12.2 (4.5) years plus 10 controls with mean age of 62.9 (8.6) years and schooling of 12.3 (4.6) years, were assessed using the Lowenstein Occupational Therapy Cognitive Assessment (LOTCA) and by a three-stage artistic protocol including visual observation, copying and collage, based on a Sisley painting. RESULTS: FTLD patients had lower scores than controls on Visuospatial Perception, Copy, Collage, Examiner's Observation, and Total, showing distinct patterns of performance according to FTLD sub-type: semantic PPA, nonfluent PPA and bvFTD. CONCLUSION: FTLD patients presented impairment in the visuospatial and executive skills required to perform artistic tasks. We demonstrated that the application of the instrument as a complimentary method for assessing cognitive skills in this group of patients is possible. Further studies addressing larger and more homogeneous samples of FTLD patients as well as other dementias are warranted.

Vários estudos relatam as habilidades visuoespaciais em atividades artísticas em Degeneração Lobar Frontotemporal (DLFT) e Demência de Alzheimer (DA). OBJETIVO: Investigar as alterações no desempenho de pacientes com DLFT comparados a controles em tarefas artísticas. MÉTODOS: Quatro pacientes com DLFT (idade média de 57 (8,7) e escolaridade de 12,2 (4,5) anos) e 10 controles (idade média de 62,9 (86) e escolaridade de 12,3 (4,6) anos) foram avaliados através da Bateria de Avaliação Cognitiva de Terapia Ocupacional Lowenstein (LOTCA) e um protocolo de arte em três estágios: observação visual, cópia colorida e colagem baseada em uma pintura de Sisley. RESULTADOS: Os pacientes com DLFT tiveram menores pontuações em Percepção Visoespacial, Cópia, Colagem, Observação do examinador e Total, mostrando distintos tipos de desempenho nos subtipos de DFT variante comportamental, APP não fluente e APP semântica. CONCLUSÃO: Os pacientes com DLFT apresentaram prejuízo nas habilidades executivas e visuoespaciais necessárias ao desempenho nas tarefas artísticas propostas. Evidenciamos ser possível a aplicação deste instrumento como método de avaliação complementar de habilidades cognitivas neste grupo de pacientes. Novos estudos em um grupo maior e mais homogêneo de pacientes com DLFT, bem como em outras demências, são necessários.

Functional status in behavioral variant frontotemporal dementia: a systematic review.

The aim was to conduct a systematic review of studies that described the functional profile of patients with behavioral variant frontotemporal dementia (bvFTD), published between 2000 and 2013. The bibliographic search was conducted using the terms "frontotemporal dementia" and "frontotemporal lobar degeneration" in combination with "independence," "functionality," "basic activities of daily living," "disability," and scales that measure functional performance: "Disability Assessment for Dementia-DAD," "Functional Activities Questionnaire (FAQ)," "Direct Assessment of Functional Status (DAFS)." To be included in the review, the study had to mention the characterization of the functional status of patients with bvFTD in the objectives of the study, using a previously validated instrument of functional assessment. Fourteen studies met this criterion. The reviewed studies suggested that individuals with bvFTD have greater functional impairment when compared to those with other subtypes of frontotemporal lobar degeneration or Alzheimer's disease. The studies documented a significant association between cognitive impairment and measures of functionality in these patients. The cognitive profile of patients may predict faster functional decline.

Translation, cross-cultural adaptation and applicability of the Brazilian version of the Frontotemporal Dementia Rating Scale (FTD-FRS) / Tradução, adaptação transcultural e aplicabilidade da Escala de Estadiamento e Progressão da Degeneração Lobar Frontotemporal

ABSTRACT Background: Staging scales for dementia have been devised for grading Alzheimer's disease (AD) but do not include the specific symptoms of frontotemporal lobar degeneration (FTLD). Objective: To translate and adapt the Frontotemporal Dementia Rating Scale (FTD-FRS) to Brazilian Portuguese. Methods: The cross-cultural adaptation process consisted of the following steps: translation, back-translation (prepared by independent translators), discussion with specialists, and development of a final version after minor adjustments. A pilot application was carried out with 12 patients diagnosed with bvFTD and 11 with AD, matched for disease severity (CDR=1.0). The evaluation protocol included: Addenbrooke's Cognitive Examination-Revised (ACE-R), Mini-Mental State Examination (MMSE), Executive Interview (EXIT-25), Neuropsychiatric Inventory (NPI), Frontotemporal Dementia Rating Scale (FTD-FRS) and Clinical Dementia Rating scale (CDR). Results: The Brazilian version of the FTD-FRS seemed appropriate for use in this country. Preliminary results revealed greater levels of disability in bvFTD than in AD patients (bvFTD: 25% mild, 50% moderate and 25% severe; AD: 36.36% mild, 63.64% moderate). It appears that the CDR underrates disease severity in bvFTD since a relevant proportion of patients rated as having mild dementia (CDR=1.0) in fact had moderate or severe levels of disability according to the FTD-FRS. Conclusion: The Brazilian version of the FTD-FRS seems suitable to aid staging and determining disease progression.

RESUMO Introdução: As escalas de estadiamento das demências, como a Clinical Dementia Rating (CDR), foram elaboradas para graduar a doença de Alzheimer (DA) e não incluem os sintomas específicos da degeneração lobar frontotemporal (DLFT). Objetivo: Realizar a tradução e adaptação cultural da Frontotemporal Dementia Rating Scale (FTD-FRS) para o contexto brasileiro e apresentar dados preliminares da sua aplicabilidade. Métodos: O processo de adaptação transcultural consistiu em: tradução, retrotradução (realizadas por tradutores independentes), discussão com especialistas sobre a versão em português e equivalência com a versão original, desenvolvimento da versão final com pequenos ajustes. Foi feita uma aplicação piloto em 12 pacientes com diagnóstico de demência frontotemporal variante comportamental (DFTvc) e 11 com DA, pareados quanto à gravidade da demência (CDR=1). O protocolo de avaliação incluiu a Addenbrooke's Cognitive Examination-Revised (ACE-R), Mini Exame do Estado Mental (MEEM), Executive Interview (EXIT-25), Inventário Neuropsiquiátrico (INP) e a Escala de Avaliação Clínica da Demência (CDR). Resultados: A FTD-FRS na versão brasileira pareceu apropriada. Resultados preliminares revelaram maiores níveis de incapacidade na DFTvc do que em pacientes com DA (DFTvc: 25% leve, 50% moderado, 25% grave; AD: 36.36% leve, 63.64% moderado). A CDR parece subestimar a gravidade da demência na DFTvc, uma vez que uma relevante proporção dos pacientes classificados com leves (CDR=1) de fato apresentaram nível moderado ou grave de comprometimento na FTD-FRS. Conclusão: A versão brasileira da FTD-FRS pode se mostrar adequada para auxiliar no estadiamento e determinar a progressão da DLFT.