Social cognition across bipolar disorder and behavioral-variant frontotemporal dementia: an exploratory study

Objectives: Bipolar disorder type 1 (BD1) and behavioral-variant frontotemporal dementia (bvFTD) share similar behavioral and cognitive symptoms, rendering the differential diagnosis between them a clinical challenge. We investigated the accuracy of social cognition (SC) measures to differentiate bvFTD from BD. Methods: We included three groups of participants: early-onset BD1 (in remission, n=20), bvFTD (n=18), and cognitively healthy controls (HC) (n=40), matched for age, schooling, and sex. All participants underwent cognitive assessment, including the Facial Emotion Recognition (FER) and Modified Faux-Pas (mFP) tests, which assess mentalizing. Results: Compared to HC, BD1 and bvFTD patients underperformed on both SC measures. BD1 and bvFTD did not differ regarding FER or mFP total scores, although patients with bvFTD had significantly higher difficulties than those in the BD1 group to detect social faux-pas (p < 0.001, d = 1.35). Conclusion: BD1 and bvFTD share deficits in the core SC functions. These findings should be considered in the development of tasks aiming to improve clinical differentiation between the two disorders.

Social cognition across bipolar disorder and behavioral variant frontotemporal dementia: an exploratory study.

OBJECTIVES: Bipolar disorder type 1 (BD1) and behavioral-variant frontotemporal dementia (bvFTD) share similar behavioral and cognitive symptoms, rendering the differential diagnosis between them a clinical challenge. We investigated the accuracy of social cognition (SC) measures to differentiate bvFTD from BD. METHODS: We included three groups of participants: early-onset BD1 (in remission, n=20), bvFTD (n=18), and cognitively healthy controls (HC) (n=40), matched for age, schooling, and sex. All participants underwent cognitive assessment, including the Facial Emotion Recognition (FER) and Modified Faux-Pas (mFP) tests, which assess mentalizing. RESULTS: Compared to HC, BD1 and bvFTD patients underperformed on both SC measures. BD1 and bvFTD did not differ regarding FER or mFP total scores, although patients with bvFTD had significantly higher difficulties than those in the BD1 group to detect social faux-pas (p < 0.001, d = 1.35). CONCLUSION: BD1 and bvFTD share deficits in the core SC functions. These findings should be considered in the development of tasks aiming to improve clinical differentiation between the two disorders.

I'm looking through you: Mentalizing in frontotemporal dementia and progressive supranuclear palsy.

Mentalizing and emotion recognition are impaired in behavioral variant frontotemporal dementia (bvFTD). It is not clear whether these abilities are also disturbed in other conditions with prominent frontal lobe involvement, such as progressive supranuclear palsy (PSP). Our aim was to investigate social cognition (facial emotion recognition, recognition of social norms violation and mentalizing) in bvFTD and PSP. The neural basis of these functions in PSP and bvFTD groups, by analysis of structural neuroimaging, were also investigated. Twenty-three bvFTD patients, 21 PSP patients and 23 healthy controls were included. All participants underwent 3T brain MRI and a full cognitive exam including the short version of Social and Emotional Assessment (Mini-SEA), which is composed of a facial emotion recognition test (FERT) and the faux pas test. Two components of the faux pas test were distinguished: a score assessing the recognition of social norms violation and a score assessing mentalizing. Compared to controls, bvFTD and PSP patients had significantly reduced scores in all tests of social cognition but did not differ on these measures. PSP and bvFTD had cerebral atrophy in critical regions for social cognition processes, when compared to controls. The cortical correlates of emotion recognition partially overlapped in bvFTD and PSP, with correlations retrieved within the frontal medial cortex, cingulate, insula and limbic structures. PSP and bvFTD patients also displayed similar patterns of brain correlations for the composite score of social norms, with a significant cluster in anterior temporal lobes. Mentalizing scores were associated with frontal and temporal poles bilaterally, in both bvFTD and PSP. These findings support previous observations that PSP patients exhibit impairment in complex cognitive abilities, such as mentalizing. Moreover, these data extend previous findings showing that PSP and bvFTD share key clinical, cognitive and neuroimaging features.

Do Patients with Progressive Supranuclear Palsy Have Episodic Memory Impairment? A Systematic Review.

Background: Progressive supranuclear palsy (PSP) is the most common atypical parkinsonism and has executive dysfunction as a core feature. The magnitude of episodic memory disturbance in PSP is yet to be clarified. Objectives: To investigate how impaired is episodic memory in PSP compared to healthy controls and other neuropsychiatric disorders. Also, we sought to identify the brain correlates underlying these memory disturbances. Methods: We performed a systematic search on PubMed and Scopus, combining the terms "progressive supranuclear palsy" AND "memory". The search was limited to papers published in English, French, Portuguese or Spanish, with no chronological filters. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. Results: The initial search returned 464 results. After extraction of duplicates, 356 records were screened, leading to inclusion of 38 studies. Most studies found that PSP patients had lower scores on episodic memory compared to healthy controls. In addition, the majority of studies suggest that PSP does not differ from Parkinson's disease and from atypical parkinsonism in terms of episodic memory performance. The same is seen for PSP and frontotemporal dementia. Conversely, episodic memory impairment seems to be greater in typical Alzheimer's disease compared to PSP. Neuroimaging findings indicate that striatofrontal structures may be involved in PSP episodic memory dysfunction, while no associations with mesial structures (including hippocampi) were found. Conclusions: Episodic memory is impaired in PSP. Whether this amnesia refers to executive dysfunction is still controversial. More studies are warranted to clarify the neural basis of memory impairment in PSP.

Episodic memory in progressive supranuclear palsy: a neuropsychological and neuroimaging study.

BACKGROUND: Episodic memory impairment may occur in progressive supranuclear palsy (PSP). However, it remains uncertain whether this is due to executive dysfunction or to the involvement of brain areas responsible for memory. OBJECTIVES: To investigate the specific brain regions underlying episodic memory impairment in PSP. METHODS: Twenty-one patients with PSP and 20 healthy controls underwent the Figure Memory Test (FMT) from the Brief Cognitive Screening Battery and brain MRI. We explored correlations between gray matter volumes and memory scores in PSP patients, adjusting for age and performance on the Frontal Assessment Battery. RESULTS: PSP patients performed worse than controls (p < 0.001) on delayed recall in the FMT. Delayed recall scores correlated to bilateral hippocampal and parahippocampal volumes in PSP patients. CONCLUSIONS: Medial temporal structures may play a role in episodic memory impairment in PSP, suggesting that amnesia in PSP is not solely due to executive dysfunction.

Effects of group Acceptance and Commitment Therapy (ACT) on anxiety and depressive symptoms in adults: A meta-analysis.

BACKGROUND: A comprehensive meta-analysis quantitatively examining the effects of group Acceptance and Commitment Therapy (ACT) on anxiety and depressive symptoms is required to advance our understanding of its efficacy and moderating factors. METHODS: Four electronic databases were searched in August 2018. An update search was conducted in November 2021. Forty-eight randomised controlled trials (RCTs) were included in this review (3292 participants: anxiety = 34 RCTs, depression = 40 RCTs). RESULTS: The overall effect size for anxiety symptoms was medium-to-large (g = 0.52, p < 0.001; 95% CI = 0.30-0.73), while the overall effect size was small-to-medium for depressive symptoms (g = 0.47, p < 0.001; 95% CI = 0.31-0.64). Subgroup analyses demonstrated that group ACT was significantly superior to non-active controls (e.g., waiting list) in reducing anxiety and depressive symptoms. Group ACT was only significantly superior to active controls (e.g., CBT) in reducing depressive symptoms. Subgroup analyses also demonstrated that the effect size can vary depending on the number of sessions provided and the primary condition of participants recruited. LIMITATIONS: The number of studies included in each category of subgroup analyses was small and the risk of bias varied across studies. There was high heterogeneity among the included studies, and this might have affected the results. CONCLUSION: The current evidence suggests that group ACT may be effective in treating anxiety and depressive symptoms, perhaps more so for depressive symptoms when compared to other well-established treatments. The intensity of treatment and the targeted population may need to be considered when delivering group ACT.

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.

Different patterns of gray matter atrophy in behavioral variant frontotemporal dementia with and without episodic memory impairment.

BACKGROUND: Differentiating patients with behavioral variant frontotemporal dementia (bvFTD) from Alzheimer's disease (AD) is important as these two conditions have distinct treatment and prognosis. Using episodic impairment and medial temporal lobe atrophy as a tool to make this distinction has been debatable in the recent literature, as some patients with bvFTD can also have episodic memory impairment and medial temporal lobe atrophy early in the disease. OBJECTIVES: To compare brain atrophy patterns of patients with bvFTD with and without episodic memory impairment to that of patients with AD. METHODS: We analyzed 19 patients with bvFTD, 21 with AD and 21 controls, matched by age, sex, and years of education. They underwent brain MRI and the memory test from the Brief Cognitive Battery (BCB) to assess episodic memory. We then categorized the bvFTD group into amnestic (BCB delayed recall score <7) and non-amnestic. RESULTS: The amnestic bvFTD group (n = 8) had significant gray matter atrophy in the left parahippocampal gyrus, right cingulate and precuneus regions compared with the nonamnestic group. Compared with AD, amnestic bvFTD had more atrophy in the left fusiform cortex, left insula, left inferior temporal gyrus and right temporal pole, whereas patients with AD had more atrophy in the left hippocampus, left frontal pole and left angular gyrus. CONCLUSIONS: There is a group of amnestic bvFTD patients with episodic memory dysfunction and significant atrophy in medial temporal structures, which poses a challenge in considering only these features when differentiating bvFTD from AD clinically.

Social Cognition Tests Can Discriminate Behavioral Variant Frontotemporal Dementia From Alzheimer's Disease Independently of Executive Functioning.

OBJECTIVE: To investigate the accuracy of the Social and Emotional Assessment-short version (Mini-SEA) to differentiate subgroups of behavioral variant of frontotemporal dementia (bvFTD) and Alzheimer's disease (AD) defined according to executive performance. METHODS: bvFTD (n = 21), AD (n = 20), and healthy controls (HC, n = 23) underwent the Mini-SEA, comprising the Facial Emotion Recognition Test (FERT) and the faux-pas test. AD and bvFTD patients were classified according to their performance in the Frontal Assessment Battery into dysexecutive and nondysexecutive subgroups. RESULTS: The area under the curve (AUC) values for the faux-pas test were 0.87 (dysexecutive-bvFTD vs. dysexecutive-AD) and 0.96 (non-dysexecutive-bvFTD vs. nondysexecutive-AD). The AUC values for FERT were 0.99 (dysexecutive-bvFTD vs. dysexecutive-AD) and 0.65 (nondysexecutive-bvFTD vs. nondysexecutive-AD); the AUC values for the Mini-SEA (total-score) were 0.95 (dysexecutive-bvFTD vs. dysexecutive-AD) and 0.88 (nondysexecutive-bvFTD vs. nondysexecutive-AD). DISCUSSION: Social Cognition tests accurately distinguish bvFTD from AD regardless of the executive profile.

Challenges in the diagnosis of dementia: insights from the United Kingdom-Brazil Dementia Workshop.

In July 2019, a group of multidisciplinary dementia researchers from Brazil and the United Kingdom (UK) met in the city of Belo Horizonte, Minas Gerais, Brazil, to discuss and propose solutions to current challenges faced in the diagnosis, public perception and care of dementia. Here we summarize the outcomes from the workshop addressing challenges in diagnosis. Brazil faces a major problem in dementia underdiagnosis, particularly involving the population in an adverse socioeconomic context. There is poor availability of resources and specialists, and the knowledge of general practitioners and other healthcare professionals is far from satisfactory. Low education level is a further obstacle in diagnosing dementia, as the most commonly used screening tests are not designed to evaluate this population. Patients and their families must overcome the stigma of a diagnosis of dementia, which is still prevalent in Brazil and increases the burden of this condition. Whilst the UK has greater resources, dedicated memory services and a National Dementia Strategy plan, the National Health Service (NHS) has limited funding. Therefore, some challenges regarding diagnosis are common across both countries. The authors suggest possible solutions to confront these, with the goal of improving assessment and recognition of dementia and reducing misdiagnosis.

Em julho de 2019, um grupo multidisciplinar de pesquisadores em demência do Brasil e do Reino Unido se reuniu em Belo Horizonte para discutir e propor soluções para os desafios no diagnóstico, percepção pública e tratamento dessa condição. Neste artigo, sintetizamos as conclusões do workshop sobre os desafios no diagnóstico de demência. O Brasil enfrenta um grande problema no subdiagnóstico de demência, principalmente entre a população em condições socioeconômicas adversas. Há pouca disponibilidade de recursos e de especialistas e o conhecimento de médicos generalistas e de outros profissionais de saúde é pouco abrangente. Baixa escolaridade é também um obstáculo no diagnóstico de demência, uma vez que os testes de rastreio mais utilizados na prática clínica não são estruturados para avaliar a população com esse perfil. Os pacientes com demência e suas famílias ainda têm que superar o estigma do diagnóstico, que é ainda muito prevalente no Brasil e colabora para a piora da qualidade de vida. O Reino Unido, por outro lado, dispõe de mais recursos financeiros e de pessoal, possui serviços dedicados à avaliação de problemas de memória e um plano estratégico nacional para demência. Contudo, o National Health Service (NHS) tem verbas limitadas, o que faz com que alguns dos desafios no diagnóstico de demência sejam comuns aos dois países. Os autores sugerem possíveis soluções para enfrentá-los, com o objetivo de melhorar a avaliação e o reconhecimento da demência e reduzir os erros de diagnóstico.

Inside minds, beneath diseases: social cognition in amyotrophic lateral sclerosis-frontotemporal spectrum disorder.

OBJECTIVE: To compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD). METHODS: We included 21 participants with ALS, 20 with bvFTD and 21 healthy controls who underwent a comprehensive cognitive battery, including the short version of the Social Cognition and Emotional Assessment (Mini-SEA), which comprises the faux pas test and Facial Emotion Recognition Test (FERT); Mini-Mental State Examination; Frontal Assessment Battery; lexical fluency (F-A-S), category fluency (animals/minute), digit span (direct and backwards) tests and the Hayling test. A post hoc analysis was conducted with the patients with ALS divided into two subgroups: patients without cognitive impairment (ALScn; n=13) and patients with cognitive impairment (ALSci; n=8). RESULTS: No significant difference was noted between participant groups in terms of the age, sex and education. ALS-total group and patients with bvFTD had similar disease durations. Patients with ALSci performed poorly when compared with controls with regard to the FERT (p<0.001), the faux pas (p<0.004) and the Mini-SEA (p<0.002) total scores. Moreover, patients with bvFTD performed poorly in comparison with controls in executive and social cognition tests. The performance of patients with ALSci was similar to that of patients with bvFTD, while the performance of patients with ALScn was similar to that of controls. DISCUSSION: Our findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

Challenges in the diagnosis of dementia: insights from the United Kingdom-Brazil Dementia Workshop / Desafios no diagnóstico de demência: conclusões do Workshop de Demência Reino Unido-Brasil

ABSTRACT. In July 2019, a group of multidisciplinary dementia researchers from Brazil and the United Kingdom (UK) met in the city of Belo Horizonte, Minas Gerais, Brazil, to discuss and propose solutions to current challenges faced in the diagnosis, public perception and care of dementia. Here we summarize the outcomes from the workshop addressing challenges in diagnosis. Brazil faces a major problem in dementia underdiagnosis, particularly involving the population in an adverse socioeconomic context. There is poor availability of resources and specialists, and the knowledge of general practitioners and other healthcare professionals is far from satisfactory. Low education level is a further obstacle in diagnosing dementia, as the most commonly used screening tests are not designed to evaluate this population. Patients and their families must overcome the stigma of a diagnosis of dementia, which is still prevalent in Brazil and increases the burden of this condition. Whilst the UK has greater resources, dedicated memory services and a National Dementia Strategy plan, the National Health Service (NHS) has limited funding. Therefore, some challenges regarding diagnosis are common across both countries. The authors suggest possible solutions to confront these, with the goal of improving assessment and recognition of dementia and reducing misdiagnosis.

RESUMO. Em julho de 2019, um grupo multidisciplinar de pesquisadores em demência do Brasil e do Reino Unido se reuniu em Belo Horizonte para discutir e propor soluções para os desafios no diagnóstico, percepção pública e tratamento dessa condição. Neste artigo, sintetizamos as conclusões do workshop sobre os desafios no diagnóstico de demência. O Brasil enfrenta um grande problema no subdiagnóstico de demência, principalmente entre a população em condições socioeconômicas adversas. Há pouca disponibilidade de recursos e de especialistas e o conhecimento de médicos generalistas e de outros profissionais de saúde é pouco abrangente. Baixa escolaridade é também um obstáculo no diagnóstico de demência, uma vez que os testes de rastreio mais utilizados na prática clínica não são estruturados para avaliar a população com esse perfil. Os pacientes com demência e suas famílias ainda têm que superar o estigma do diagnóstico, que é ainda muito prevalente no Brasil e colabora para a piora da qualidade de vida. O Reino Unido, por outro lado, dispõe de mais recursos financeiros e de pessoal, possui serviços dedicados à avaliação de problemas de memória e um plano estratégico nacional para demência. Contudo, o National Health Service (NHS) tem verbas limitadas, o que faz com que alguns dos desafios no diagnóstico de demência sejam comuns aos dois países. Os autores sugerem possíveis soluções para enfrentá-los, com o objetivo de melhorar a avaliação e o reconhecimento da demência e reduzir os erros de diagnóstico.

Apathy in frontotemporal dementia is related to medial prefrontal atrophy and is independent of executive dysfunction.

INTRODUCTION: Apathy is the most common neuropsychiatric syndrome in behavioral variant frontotemporal dementia (bvFTD), and encompasses cognitive, behavioral and affective symptoms. The neural basis of apathy in bvFTD is not completely understood. Previous neuroimaging studies have poorly considered executive impairment and dementia severity as possible confounding factors. Herein we investigated the neural basis of apathy in bvFTD through structural neuroimaging taking into account these factors. METHODS: We included patients with probable bvFTD (n = 21) and cognitively healthy controls (HC, n = 22). Participants were matched for age, sex and schooling. All subjects underwent a thorough neuropsychological examination, including tests for executive functions and social cognition. Apathy was assessed with the Starkstein Apathy Scale (SAS). All subjects underwent 3T brain MRI. We investigated correlations between SAS scores and gray matter atrophy within the bvFTD group. Executive function (Frontal Assessment Battery) and disease severity were considered as covariates in neuroimaging analyses. RESULTS: Compared to HC, bvFTD patients had lower scores on global cognitive efficiency, executive functions and social cognition. All bvFTD had clinically relevant apathy (scores greater than 14 in the SAS). Performance in executive function tests did not correlate with apathy scores. The severity of apathy was negatively correlated with gray matter volumes in midline prefrontal regions, namely orbitofrontal cortex and both anterior and dorsal regions of cingulate cortex. CONCLUSIONS: Apathy in bvFTD is related to a specific network of prefrontal cortical areas critically involved in effort-based behavior for rewards and appears to be independent of executive dysfunction.

Can Social Cognition Measurements Differentiate Behavioral Variant Frontotemporal Dementia from Alzheimer's Disease Regardless of Apathy?

BACKGROUND: Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD) share cognitive and behavioral symptoms, such as apathy. Social cognition measurements are useful in distinguishing bvFTD from AD, but their accuracies may be affected by apathy. OBJECTIVE: To investigate whether social cognition measurements can distinguish bvFTD from either apathetic or non-apathetic AD patients. METHODS: Three groups of participants were enrolled in the present study: bvFTD (n = 22), AD (n = 20), and healthy controls (HC, n = 23). The AD group was divided into apathetic (n = 10) and non-apathetic (n = 10). All subjects underwent comprehensive neuropsychological examination, including the short version of the Social and Emotional Assessment (Mini-SEA), which comprises the facial emotion recognition test and the faux-pas recognition test (Faux-Pas Test). Apathy was assessed according to the Starkstein's Apathy (SA) Scale. RESULTS: The bvFTD and AD groups did not differ on global cognitive efficiency and on executive functions. In comparison to the whole AD group, bvFTD displayed lower Faux-Pas Test and Mini-SEA scores. Both AD subgroups, apathetic or non-apathetic, exhibited similar performance on all social cognition measurements. In comparison to either apathetic AD or non-apathetic AD, bvFTD patients underperformed on the Faux-Pas Test and on the Mini-SEA. The area under the curve values for the Mini-SEA total score were 0.87 (bvFTD versus AD), 0.90 (bvFTD versus apathetic AD), and 0.83 (bvFTD versus non-apathetic AD). CONCLUSION: Social cognition tests provide accurate distinction between bvFTD against either apathetic AD or non-apathetic AD. Social cognition measurements did not correlate with apathy severity.

Behavioral variant of frontotemporal dementia or frontal variant of Alzheimer's disease? A case study.

Alzheimer's disease (AD) has heterogeneous clinical presentations. Amnestic progressive disorder leading to dementia is the most typical, but non-amnestic presentations are also recognized. Here we report a case of frontal variant of AD. A right-handed woman, aged 68 years, was referred for progressive behavioral disorders and personality changes. She had a corroborated history of dietary changes, hyperorality, impulsivity, affective indifference and apathy, with functional impairment. Cognitive assessment yielded severe executive deficits. Positron emission tomography with fluorodeoxyglucose showed marked hypometabolism in frontotemporal regions, with relative preservation of parietal regions. CSF AD biomarkers showed low Aß42, high Tau and high P-Tau. The patient fulfilled criteria for probable behavioral variant frontotemporal dementia. However, considering the AD pathophysiological signature on CSF biomarkers, a diagnosis of frontal variant of AD was established. In the perspective of disease-modifying therapies, it is important to identify atypical Alzheimer presentations, as these patients may be candidates for specific treatments.

A doença de Alzheimer (DA) tem apresentações clínicas heterogêneas. Amnésia progressiva associada a demência é a forma mais comum, mas apresentações não-amnésticas são também reconhecidas. Relatamos um caso de variante frontal da DA. Um mulher destra, de 68 anos, consultou-se devido a transtornos comportamentais progressivos, com alterações de personalidade. Modificações de padrão alimentar, hiperoralidade, impulsividade, indiferença afetiva e apatia, com declínio funcional, foram corroboradas pela família. A avaliação cognitiva evidenciou grave disfunção executiva. A tomografia de emissão de pósitrons com flúordeoxiglicose revelou proeminente hipometabolismo em regiões frontotemporais, com relativa preservação de regiões parietais. A análise de biomarcadores de DA no líquido cefalorraquidiano mostrou redução de níveis de Aß42, com aumento de níveis de Tau e P-Tau. A paciente preencheu critérios diagnósticos para demência frontotemporal (variante comportamental) provável. Contudo, considerando o perfil de biomarcadores em favor de fisiopatologia da DA, o diagnóstico de variante frontal de DA foi estabelecido. Na perspectiva de tratamentos modificadores da doença, é crucial identificar apresentações atípicas da DA, visto que esses pacientes podem ser candidatos a terapias específicas.

Behavioral variant of frontotemporal dementia or frontal variant of Alzheimer's disease? A case study / Variante comportamental da demência frontotemporal ou variante frontal da doença de alzheimer? um estudo de caso

ABSTRACT. Alzheimer's disease (AD) has heterogeneous clinical presentations. Amnestic progressive disorder leading to dementia is the most typical, but non-amnestic presentations are also recognized. Here we report a case of frontal variant of AD. A right-handed woman, aged 68 years, was referred for progressive behavioral disorders and personality changes. She had a corroborated history of dietary changes, hyperorality, impulsivity, affective indifference and apathy, with functional impairment. Cognitive assessment yielded severe executive deficits. Positron emission tomography with fluorodeoxyglucose showed marked hypometabolism in frontotemporal regions, with relative preservation of parietal regions. CSF AD biomarkers showed low Aß42, high Tau and high P-Tau. The patient fulfilled criteria for probable behavioral variant frontotemporal dementia. However, considering the AD pathophysiological signature on CSF biomarkers, a diagnosis of frontal variant of AD was established. In the perspective of disease-modifying therapies, it is important to identify atypical Alzheimer presentations, as these patients may be candidates for specific treatments.

RESUMO. A doença de Alzheimer (DA) tem apresentações clínicas heterogêneas. Amnésia progressiva associada a demência é a forma mais comum, mas apresentações não-amnésticas são também reconhecidas. Relatamos um caso de variante frontal da DA. Um mulher destra, de 68 anos, consultou-se devido a transtornos comportamentais progressivos, com alterações de personalidade. Modificações de padrão alimentar, hiperoralidade, impulsividade, indiferença afetiva e apatia, com declínio funcional, foram corroboradas pela família. A avaliação cognitiva evidenciou grave disfunção executiva. A tomografia de emissão de pósitrons com flúordeoxiglicose revelou proeminente hipometabolismo em regiões frontotemporais, com relativa preservação de regiões parietais. A análise de biomarcadores de DA no líquido cefalorraquidiano mostrou redução de níveis de Aß42, com aumento de níveis de Tau e P-Tau. A paciente preencheu critérios diagnósticos para demência frontotemporal (variante comportamental) provável. Contudo, considerando o perfil de biomarcadores em favor de fisiopatologia da DA, o diagnóstico de variante frontal de DA foi estabelecido. Na perspectiva de tratamentos modificadores da doença, é crucial identificar apresentações atípicas da DA, visto que esses pacientes podem ser candidatos a terapias específicas.

Phenocopy syndrome of behavioral variant frontotemporal dementia: a systematic review.

BACKGROUND: The phenocopy syndrome of behavioral variant of frontotemporal dementia (phFTD) refers to patients presenting with neuropsychiatric symptoms mimicking the behavioral variant frontotemporal dementia (bvFTD), but lacking frontotemporal atrophy/hypometabolism on neuroimaging and not evolving to dementia during the follow-up. It is important to recognize phFTD for clinical and research purposes. OBJECTIVE: The aim of this study was to perform a systematic review of the available literature on phFTD taking into account its clinical, cognitive, imaging, genetic, and pathological features. METHODS AND RESULTS: We searched for the following terms in two electronic databases (PubMed and Scopus): "frontotemporal dementia and slowly progressive," "frontotemporal dementia and phenocopy," "frontotemporal dementia and non-progressive," "frontotemporal dementia and benign progression," and "frontotemporal dementia and benign." We did not include review articles. Papers had to be written in English, French, Portuguese, or Spanish. Overall, 235 studies were retrieved in the initial search. A total of 31 studies composed the final selection, comprising 292 patients. Patients with phFTD are predominantly male and have no major cognitive deficits, with globally preserved executive functions and episodic memory. Some cases (n = 7) of slowly progressive FTD have been associated with C9orf72 genetic expansion. There are only four reports of phFTD neuropathological data, with two patients with no neurodegenerative findings and two with frontotemporal lobar degeneration with ubiquitin-positive inclusions. CONCLUSION: The neurobiological underpinnings of phFTD remain unknown. It is controversial whether phFTD belongs to the FTD spectrum. Studies with biomarkers and pathological data are needed to solve the phFTD conundrum.

Divulgando a Psicologia no rádio: relato de uma experiência / Broadcasting Psychology on the radio: an experience report / Divulgando la Psicología en la radio / relato de una experiencia

Em virtude das inúmeras dificuldades envolvidas no seu processo de planejamento e de execução, a divulgação científica tem tradicionalmente representado um desafio para pesquisadores de diversas áreas. Apesar da reconhecida importância dessa tarefa, é preciso admitir que a própria Academia tem dedicado a ela insuficiente atenção. O relato aqui apresentado descreve o processo de produção semanal de um programa de rádio que tem como objetivo, utilizando o saber psicológico como uma de suas referências, abordar temas cotidianos. Foram produzidos, até o momento, 20 programas com duração média de 15 minutos. É destacada no relato a importância da atividade de divulgação científica baseada na interlocução de saberes. São descritas as principais dificuldades enfrentadas pela equipe quanto à linguagem e aos aspectos técnicos da produção dos programas e as soluções encontradas na busca por um formato que conjugue a responsabilidade ética no tratamento dos temas, a fidelidade aos pressupostos teóricos dos entrevistados e a acessibilidade da linguagem para um público não especialista em Psicologia....(AU)

The diffusion of science knowledge has traditionally represented a challenge for researchers from several areas given the abundant difficulties involved in its planning and execution. Despite the acknowledged importance of this task, it is clear that the universities themselves have not been giving enough attention to this matter. The present report describes the process of a weekly production of a radio program that is aimed at approaching everyday themes using psychological knowledge as one of its references. 20 programs were produced so far with an average duration of 15 minutes each. The importance of the diffusion of science based upon knowledge dialogue is highlighted in this report. Also, here are described the main difficulties faced by the team regarding language and the technical aspects of the production of the radio programs and the solutions found in the search for a format that combines the ethical responsibility in addressing the themes, loyalty to the theoretical assumptions of the interviewees and language accessibility to a public non-specialist in psychology....(AU)

En virtud de las innumerables dificultades involucradas en su proceso de planeación y de ejecución, la divulgación ha representado tradicionalmente un desafío para investigadores de diversas áreas. A despecho de la reconocida importancia de esa tarea, es necesario reconocer que la propia academia ha dedicado a ella insuficiente atención. El relato aquí presentado describe el proceso de producción semanal de un programa de radio que tiene como objetivo, utilizando el saber psicológico como una de sus referencias, abordar temas cotidianos. Han sido producidos, hasta el momento, 20 programas con duración promedio de 15 minutos. Es destacada en el relato la importancia de la actividad de divulgación científica basada en la interlocución de saberes. Son descriptas las principales dificultades enfrentadas por el equipo en cuanto al lenguaje y a los aspectos técnicos de la producción de los programas y las soluciones encontradas en la búsqueda por un formato que conjugue la responsabilidad ética en el tratamiento de los temas, la fidelidad a los presupuestos teóricos de los entrevistados y la accesibilidad del lenguaje para un público no especialista en psicología....(AU)

Divulgando a Psicologia no rádio: relato de uma experiência / Broadcasting Psychology on the radio: an experience report / Divulgando la Psicología en la radio / relato de una experiencia

Em virtude das inúmeras dificuldades envolvidas no seu processo de planejamento e de execução, a divulgação científica tem tradicionalmente representado um desafio para pesquisadores de diversas áreas. Apesar da reconhecida importância dessa tarefa, é preciso admitir que a própria Academia tem dedicado a ela insuficiente atenção. O relato aqui apresentado descreve o processo de produção semanal de um programa de rádio que tem como objetivo, utilizando o saber psicológico como uma de suas referências, abordar temas cotidianos. Foram produzidos, até o momento, 20 programas com duração média de 15 minutos. É destacada no relato a importância da atividade de divulgação científica baseada na interlocução de saberes. São descritas as principais dificuldades enfrentadas pela equipe quanto à linguagem e aos aspectos técnicos da produção dos programas e as soluções encontradas na busca por um formato que conjugue a responsabilidade ética no tratamento dos temas, a fidelidade aos pressupostos teóricos dos entrevistados e a acessibilidade da linguagem para um público não especialista em Psicologia.(AU)

The diffusion of science knowledge has traditionally represented a challenge for researchers from several areas given the abundant difficulties involved in its planning and execution. Despite the acknowledged importance of this task, it is clear that the universities themselves have not been giving enough attention to this matter. The present report describes the process of a weekly production of a radio program that is aimed at approaching everyday themes using psychological knowledge as one of its references. 20 programs were produced so far with an average duration of 15 minutes each. The importance of the diffusion of science based upon knowledge dialogue is highlighted in this report. Also, here are described the main difficulties faced by the team regarding language and the technical aspects of the production of the radio programs and the solutions found in the search for a format that combines the ethical responsibility in addressing the themes, loyalty to the theoretical assumptions of the interviewees and language accessibility to a public non-specialist in psychology.(AU)

En virtud de las innumerables dificultades involucradas en su proceso de planeación y de ejecución, la divulgación ha representado tradicionalmente un desafío para investigadores de diversas áreas. A despecho de la reconocida importancia de esa tarea, es necesario reconocer que la propia academia ha dedicado a ella insuficiente atención. El relato aquí presentado describe el proceso de producción semanal de un programa de radio que tiene como objetivo, utilizando el saber psicológico como una de sus referencias, abordar temas cotidianos. Han sido producidos, hasta el momento, 20 programas con duración promedio de 15 minutos. Es destacada en el relato la importancia de la actividad de divulgación científica basada en la interlocución de saberes. Son descriptas las principales dificultades enfrentadas por el equipo en cuanto al lenguaje y a los aspectos técnicos de la producción de los programas y las soluciones encontradas en la búsqueda por un formato que conjugue la responsabilidad ética en el tratamiento de los temas, la fidelidad a los presupuestos teóricos de los entrevistados y la accesibilidad del lenguaje para un público no especialista en psicología.(AU)