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Although social functioning relies on working memory, whether a social-specific mechanism exists remains unclear. This undermines the characterization of neurodegenerative conditions with both working memory and social deficits. We assessed working memory domain-specificity across behavioral, electrophysiological, and neuroimaging dimensions in 245 participants. A novel working memory task involving social and non-social stimuli with three load levels was assessed across controls and different neurodegenerative conditions with recognized impairments in: working memory and social cognition (behavioral-variant frontotemporal dementia); general cognition (Alzheimer's disease); and unspecific patterns (Parkinson's disease). We also examined resting-state theta oscillations and functional connectivity correlates of working memory domain-specificity. Results in controls and all groups together evidenced increased working memory demands for social stimuli associated with frontocinguloparietal theta oscillations and salience network connectivity. Canonical frontal theta oscillations and executive-default mode network anticorrelation indexed non-social stimuli. Behavioral-variant frontotemporal dementia presented generalized working memory deficits related to posterior theta oscillations, with social stimuli linked to salience network connectivity. In Alzheimer's disease, generalized working memory impairments were related to temporoparietal theta oscillations, with non-social stimuli linked to the executive network. Parkinson's disease showed spared working memory performance and canonical brain correlates. Findings support a social-specific working memory and related disease-selective pathophysiological mechanisms.

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BACKGROUND: Although social cognition is compromised in patients with neurodegenerative disorders such as behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD), research on moral emotions and their neural correlates in these populations is scarce. No previous study has explored the utility of moral emotions, compared to and in combination with classical general cognitive state tools, to discriminate bvFTD from AD patients. OBJECTIVE: To examine self-conscious (guilt and embarrassment) and other-oriented (pity and indignation) moral emotions, their subjective experience, and their structural brain underpinnings in bvFTD (n = 31) and AD (n = 30) patients, compared to healthy controls (n = 37). We also explored the potential utility of moral emotions measures to discriminate bvFTD from AD. METHODS: We used a modified version of the Moral Sentiment Task measuring the participants' accuracy scores and their emotional subjective experiences. RESULTS: bvFTD patients exhibited greater impairments in self-conscious and other-oriented moral emotions as compared with AD patients and healthy controls. Moral emotions combined with general cognitive state tools emerged as useful measures to discriminate bvFTD from AD patients. In bvFTD patients, lower moral emotions scores were associated with lower gray matter volumes in caudate nucleus and inferior and middle temporal gyri. In AD, these scores were associated with lower gray matter volumes in superior and middle frontal gyri, middle temporal gyrus, inferior parietal lobule and supramarginal gyrus. CONCLUSION: These findings contribute to a better understanding of moral emotion deficits across neurodegenerative disorders, highlighting the potential benefits of integrating this domain into the clinical assessment.

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OBJECTIVES: Patients with dementia show dissociations between musical semantic memory (i.e., spared musical lexicon) and other memory modalities, except in some severe cases. We aim to study, from a neuropsychological point of view, the dissociation between musical semantic memory compared to language and verbal memory in a patient with severe Behavioral Variant Frontotemporal Dementia (bvFTD). We hypothesize a single dissociation between these domains will be found, with sparing of musical semantic memory. METHODS: LC, a patient with severe bvFTD, and three matched controls were assessed through language, semantic, and episodic memory, and musical semantic memory tasks. The control group had similar music taste as LC: to participate as controls, tango must be one of their favorite musical genres. RESULTS: LC showed impairment in all Verbal Memory tasks, but not in musical tasks. There was a dissociation between musical semantic memory, and language and verbal semantic memory. CONCLUSIONS: The musical lexicon can be preserved in advanced stages of dementia, which supports the idea that music can be a therapeutic tool in patients with severe dementia.

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BACKGROUND: Processing of linguistic negation has been associated to inhibitory brain mechanisms. However, no study has tapped this link via multimodal measures in patients with core inhibitory alterations, a critical approach to reveal direct neural correlates and potential disease markers. METHODS: Here we examined oscillatory, neuroanatomical, and functional connectivity signatures of a recently reported Go/No-go negation task in healthy controls and behavioral variant frontotemporal dementia (bvFTD) patients, typified by primary and generalized inhibitory disruptions. To test for specificity, we also recruited persons with Alzheimer's disease (AD), a disease involving frequent but nonprimary inhibitory deficits. RESULTS: In controls, negative sentences in the No-go condition distinctly involved frontocentral delta (2-3 Hz) suppression, a canonical inhibitory marker. In bvFTD patients, this modulation was selectively abolished and significantly correlated with the volume and functional connectivity of regions supporting inhibition (e.g. precentral gyrus, caudate nucleus, and cerebellum). Such canonical delta suppression was preserved in the AD group and associated with widespread anatomo-functional patterns across non-inhibitory regions. DISCUSSION: These findings suggest that negation hinges on the integrity and interaction of spatiotemporal inhibitory mechanisms. Moreover, our results reveal potential neurocognitive markers of bvFTD, opening a new agenda at the crossing of cognitive neuroscience and behavioral neurology.

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Frontotemporal lobar degeneration describes a group of progressive brain disorders that primarily are associated with atrophy of the prefrontal and anterior temporal lobes. Frontotemporal lobar degeneration is considered to be equivalent to frontotemporal dementia. Frontotemporal dementia is characterized by progressive impairments in behavior, executive function, and language. There are two main clinical subtypes: behavioral-variant frontotemporal dementia and primary progressive aphasia. The early diagnosis of frontotemporal dementia is critical for developing management strategies and interventions for these patients. Without validated biomarkers, the clinical diagnosis depends on recognizing all the core or necessary neuropsychiatric features, but misdiagnosis often occurs due to overlap with a range of neurologic and psychiatric disorders. In the studies reviewed a very large number of microRNAs were found to be dysregulated but with limited overlap between individual studies. Measurement of specific miRNAs singly or in combination, or as miRNA pairs (as a ratio) in blood plasma, serum, or cerebrospinal fluid enabled frontotemporal dementia to be discriminated from healthy controls, Alzheimer's disease, and amyotrophic lateral sclerosis. Furthermore, upregulation of miR-223-3p and downregulation of miR-15a-5p, which occurred both in blood serum and cerebrospinal fluid, distinguished behavioral-variant frontotemporal dementia from healthy controls. Downregulation of miR-132-3p in frontal and temporal cortical tissue distinguished frontotemporal lobar degeneration and frontotemporal dementia, respectively, from healthy controls. Possible strong miRNA biofluid biomarker contenders for behavioral-variant frontotemporal dementia are miR-223-3p, miR-15a-5p, miR-22-3p in blood serum and cerebrospinal fluid, and miR-124 in cerebrospinal fluid. No miRNAs were identified able to distinguish between behavioral-variant frontotemporal dementia and primary progressive aphasia subtypes. Further studies are warranted on investigating miRNA expression in biofluids and frontal/temporal cortical tissue to validate and extend these findings.

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Objective: To describe the demographic characteristics, initial psychiatric diagnoses, and the time to reach a diagnosis of probable behavioral variant frontotemporal dementia (bvFTD) in a public psychiatric hospital in Cali, Colombia. Methods: We retrospectively reviewed the medical records of 28 patients who were diagnosed with probable bvFTD based on a multidisciplinary evaluation that included a structural MRI, neuropsychological testing, functional assessment, and neurological exam. Prior to this evaluation, all patients were evaluated by a psychiatrist as part of their initial consultation at the hospital. The initial consultation included the Neuropsychiatric Inventory and diagnoses based on the DSM-V. Demographics, clinical features, and initial psychiatric misdiagnoses were extracted from clinical records and summarized in the full sample and by gender. Results: The study sample had a mean education of 10.0 years (SD = 4.9) and 68.0% were female. In the full sample, 28.6% were initially diagnosed with dementia, and 71.4% with a psychiatric disorder. The psychiatric diagnosis at initial consultation differed by gender. Women were most likely to be diagnosed with depression (26.3%) or bipolar disorder (26.3%), while the men were most likely to be diagnosed with anxiety (33.3%) or a psychotic disorder (22.2%). Psychotic symptoms were common (delusions, 60.7% and hallucinations, 39.3%), and the pattern of neuropsychiatric symptoms did not differ by gender. Conclusions: This is one of few case series of bvFTD in a Colombian population, where bvFTD is a recognizable and prevalent disorder. In this psychiatric hospital, the majority of patients with bvFTD were initially diagnosed with a primary psychiatric condition. There was a gender difference in psychiatric diagnosis, but not in neuropsychiatric symptoms. In this sample, the rate of psychiatric misdiagnosis, as well as the psychotic symptoms, were higher compared to rates described in other countries. These results highlight the need for interventions to improve bvFTD diagnosis in under-represented populations.

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The differential diagnosis among the behavioral variant of frontotemporal dementia FTD (bvFTD) and the linguist one primary progressive aphasia (PPA) is challenging. Presentations of dementia type or variants dominated by personality change or aphasia are frequently misinterpreted as psychiatric illness, stroke, or other conditions. Therefore, it is important to identify cognitive tests that can distinguish the distinct FTD variants to reduce misdiagnosis and best tailor interventions. We aim to examine the discriminative capacity of the most frequently used cognitive tests in their Spanish version for the context of dementia evaluation as well as the qualitative aspects of the neuropsychological performance such as the frequency and type of errors, perseverations, and false positives that can best discriminate between bvFTD and PPA. We also described mood and behavioral profiles of participants with mild to moderate probable bvFTD and PPA. A total of 55 subjects were included in this cross-sectional study: 20 with PPA and 35 with bvFTD. All participants underwent standard dementia screening that included a medical history and physical examination, brain MRI, a semistructured caregiver interview, and neuropsychological testing. We found that bvFTD patients had worse performance in executive function tests, and the PPA presented with the lower performance in language tests and the global score of Mini-Mental State Examination (MMSE). After running the linear discriminant model, we found three functions of cognitive test and subtests combination and three functions made by the Montreal Cognitive Assessment (MoCA) language subtest and performance errors that predicted group belonging. Those functions were more capable to classify bvFTD cases rather than PPA. In conclusion, our study supports that the combination of an individual test of executive function and language, MoCA's subtest, and performance errors as well have good accuracy to discriminate between bvFTD and PPA.

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Recent frameworks in cognitive neuroscience and behavioral neurology underscore interoceptive priors as core modulators of negative emotions. However, the field lacks experimental designs manipulating the priming of emotions via interoception and exploring their multimodal signatures in neurodegenerative models. Here, we designed a novel task that involves interoceptive and control-exteroceptive priming conditions followed by post-interoception and post-exteroception facial emotion recognition (FER). We recruited 114 participants, including healthy controls (HCs) as well as patients with behavioral variant frontotemporal dementia (bvFTD), Parkinson's disease (PD), and Alzheimer's disease (AD). We measured online EEG modulations of the heart-evoked potential (HEP), and associations with both brain structural and resting-state functional connectivity patterns. Behaviorally, post-interoception negative FER was enhanced in HCs but selectively disrupted in bvFTD and PD, with AD presenting generalized disruptions across emotion types. Only bvFTD presented impaired interoceptive accuracy. Increased HEP modulations during post-interoception negative FER was observed in HCs and AD, but not in bvFTD or PD patients. Across all groups, post-interoception negative FER correlated with the volume of the insula and the ACC. Also, negative FER was associated with functional connectivity along the (a) salience network in the post-interoception condition, and along the (b) executive network in the post-exteroception condition. These patterns were selectively disrupted in bvFTD (a) and PD (b), respectively. Our approach underscores the multidimensional impact of interoception on emotion, while revealing a specific pathophysiological marker of bvFTD. These findings inform a promising theoretical and clinical agenda in the fields of nteroception, emotion, allostasis, and neurodegeneration.SIGNIFICANCE STATEMENT We examined whether and how emotions are primed by interoceptive states combining multimodal measures in healthy controls and neurodegenerative models. In controls, negative emotion recognition and ongoing HEP modulations were increased after interoception. These patterns were selectively disrupted in patients with atrophy across key interoceptive-emotional regions (e.g., the insula and the cingulate in frontotemporal dementia, frontostriatal networks in Parkinson's disease), whereas persons with Alzheimer's disease presented generalized emotional processing abnormalities with preserved interoceptive mechanisms. The integration of both domains was associated with the volume and connectivity (salience network) of canonical interoceptive-emotional hubs, critically involving the insula and the anterior cingulate. Our study reveals multimodal markers of interoceptive-emotional priming, laying the groundwork for new agendas in cognitive neuroscience and behavioral neurology.

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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.

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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.

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An early stage of behavioral variant frontotemporal dementia (bvFTD) often displays a mix of behavioral disturbances and personality changes hindering a differential diagnosis from elderly bipolar disorder (BD), making this process a big challenge. However, no studies have compared these pathologies from neuropsychological and neuroanatomical perspectives. The aim of the present study was to compare the executive functions (EF) and social cognition profiles as well as the structural neuroimaging of bvFTD and elderly patients with BD. First, we compared the executive and social cognition performances of 16 bvFTD patients, 13 BD patients and 22 healthy controls. Second, we compared grey matter volumes in both groups of patients and controls using voxel-based morphometry. Lastly, we examined the brain regions where atrophy might be associated with specific impairments in bvFTD and BD patients. Compared to controls, bvFTD patients showed deficits in working memory, abstraction capacity, inhibitory control, cognitive flexibility, verbal fluency and theory of mind (ToM). Patients with BD showed lower performance than controls in terms of abstraction capacity and verbal inhibitory control. In bvFTD patients, atrophy of frontal, temporal and insular cortices was related to EF deficits. Atrophy of the amygdala, the hippocampus, the parahippocampal gyrus, the putamen, the insula, the precuneus, the right temporo-parietal junction and superior temporal pole was associated to ToM impairments. No significant associations between atrophy and EF performance were observed in BD patients. BvFTD patients showed greater EF and ToM deficits than BD patients. Moreover, compared to BD, bvFTD patients exhibited a significant decrease in GM volume in frontal, temporal and parietal regions. Our results provide the first comparison of EF, social cognition and neuroanatomical profiles of bvFTD and elderly BD patients. These findings shed light on differential diagnosis of these disorders and may have important clinical implications.

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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.

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BACKGROUND: Previous works highlight the neurocognitive differences between apathetic and disinhibited clinical presentations of the behavioral variant frontotemporal dementia (bvFTD). However, little is known regarding how the early presentation (i.e., first symptom) is associated to the neurocognitive correlates of the disease's clinical presentation at future stages of disease. OBJECTIVES: We analyzed the neurocognitive correlates of patients with bvFTD who debuted with apathy or disinhibition as first symptom of disease. METHODS: We evaluated the neuropsychological, clinical, and neuroanatomical (3T structural images) correlates in a group of healthy controls (n = 30) and two groups of bvFTD patients (presented with apathy [AbvFTD, n = 18] or disinhibition [DbvFTD, n = 16]). To differentiate groups according to first symptoms, we used multivariate analyses. RESULTS: The first symptom in patients described the evolution of the disease. AbvFTD and DbvFTD patients showed increased brain atrophy and increased levels of disinhibition and apathy, respectively. Whole brain analyzes in AbvFTD revealed atrophy in the frontal, insular, and temporal areas. DbvFTD, in turn, presented atrophy in the prefrontal regions, temporoparietal junction, insula, and temporoparietal region. Increased atrophy in DbvFTD patients (compared to AbvFTD) was observed in frontotemporal regions. Multivariate analyses confirmed that a set of brain areas including right orbitofrontal, right dorsolateral prefrontal, and left caudate were enough to distinguish the patients' subgroups.∥Conclusion: First symptom in bvFTD patients described the neurocognitive impairments after around three years of disease, playing an important role in the early detection, disease tracking, and neuroanatomical specification of bvFTD, as well as in future research on potential disease-modifying treatments.

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Las emociones básicas están biológicamente determinadas y ligadas a conductas fundamentales para la supervivencia. Las emociones secundarias son aquellas que para su reconocimiento, requieren la elaboración cognitiva de un contexto social, por lo tanto, de la Teoría de la Mente (TdM). La TdM fue definida como la habilidad de conceptualizar estados mentales de otros individuos para explicar y predecir gran parte de su comportamiento. No es un concepto unitario, existen disociaciones entre los componentes cognitivo y el emocional de la TdM. Han sido establecidas las alteraciones en el reconocimiento facial de emociones básicas (RFEB) y en las tareas de TdM en la variante conductual de la demencia frontotemporal (DFTvc). El objetivo del trabajo que se informa fue estudiar el reconocimiento de emociones básicas o primarias y su relación con la TdM en pacientes con DFTvc. El 81% de los pacientes mostró alteraciones en por lo menos uno de los tests de RFEB y el 35% en el reconocimiento de la prosodia emocional. El subtest Denominación y el Puntaje Total Emociones mostraron correlaciones con el Test Lectura de la Mente en los Ojos, mientras que todas las tareas de RFEB correlacionaron con la tarea de falsa creencia. Se encontraron dobles disociaciones entre TdM emocional y cognitiva, con mayor afectación del componente emocional. Como conclusión se corrobora la presencia de alteraciones en el RFEB con prosodia emocional conservada en la DFTvc. La ausencia de correlaciones entre emociones básicas y secundarias parecería indicar que se trata de procesos independientes entre sí.

Behavioral variant of frontotemporal dementia (bvFTD) is associated with dramatic changes in personality. The behavioral manifestations that show these patients may be due, to one hand, to abnormal emotional processing as a result of the anatomical regions concerned, and on the other, to alterations in Theory of Mind (ToM). Basic emotions are biologically determined. Joy, sadness, anger, disgust and fear are the emotional states that have received most agreement. ToM allows representing, inferring and interpreting mental and emotional states of others. It has been suggested that it is not a unitary concept. Cognitive Theory of Mind refers to the representations regarding the cognitive status of others. Affective ToM refers to the representation of emotional and motivational states. The objectives were to study the presence of alterations in the recognition of basic emotions in the face and voice in patients with bvFTD, aswell as examine the relation ships that exist between the recognition of basic emotions and TdM, to know if there are, or not, independent processes. To study, finally, the presence of dissociations between emotional and cognitive ToM, to know which one of these types of ToM shows greater alteration in our population. 26 bvFTD patients were assessed, diagnosed on the basis of the criteria proposed by the International Consortium Behavioral Variant FTD Criteria, and 23 control subjects. A battery for facial recognition of basic emotions (FRBE) was administered, selecting 60 photographs of the Pictures of Facial Affect. Three tasks were created, comprising 60 sheets each, 10 forevery basic emotion. A test for recognition of emotional prosody was also administered. Among the ToM tests were Reading the Mind in the Eyes (RME), Faux Pas, and Firs Order False Belief Task (FOFBT). 81% of the patients showed alterations in at least one of the tests RFEB, and 35% in emotional prosody recognition. All RFEB tasks showed a significantly moderate statistical correlation with the emotional prosody task. The Naming subtest and the Total Emotions Score (RFEB) showed correlations with RME test, while all tasks RFEB correlated with FC1ºO. Finally, 10 simple dissociations between altered FRBE and preserved emotional prosody were found, and double dissociations between emotional and cognitive ToM, with greater impairment of the emotional component of ToM Discussion: A decreased performance is corroborated in the bvFTD patients group, relative to a control group, in all FRBE tasks. The recognition of emotional prosody is preserved. The presence of correlations between RME and all FRBE tasks may be explained by the neuropsychological structure of the tasks. The correlations found between RME and under standing of emotional prosody may be due to the fact that both tests assess emotional comprehension. As for the correlations found between FC1ºO and all FRBE tasks, taking into account that there seems to be no bibliographic outcome regarding to this correlation, that the neuropsychological tasks structure is completely different, and that FC1ºO showed no correlations with the emotional prosody task, it seems not possible for us to explain this result, leaving open the possibility for the development of a acceptable conclusion. Because of the absence of significant correlations between all of the recognition of basic and complex emotions tasks, we infer that the basic emotions are a qualitatively different kind of emotional state that secondary emotions, this lasts ones processed through ToM.

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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.

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Loss of empathy is an early central symptom and diagnostic criterion of the behavioral variant frontotemporal dementia (bvFTD). Although changes in empathy are evident and strongly affect the social functioning of bvFTD patients, few studies have directly investigated this issue by means of experimental paradigms. The current study assessed multiple components of empathy (affective, cognitive and moral) in bvFTD patients. We also explored whether the loss of empathy constitutes a primary deficit of bvFTD or whether it is explained by impairments in executive functions (EF) or other social cognition domains. Thirty-seven bvFTD patients with early/mild stages of the disease and 30 healthy control participants were assessed with a task that involves the perception of intentional and accidental harm. Participants were also evaluated on emotion recognition, theory of mind (ToM), social norms knowledge and several EF domains. BvFTD patients presented deficits in affective, cognitive and moral aspects of empathy. However, empathic concern was the only aspect primarily affected in bvFTD that was neither related nor explained by deficits in EF or other social cognition domains. Deficits in the cognitive and moral aspects of empathy seem to depend on EF, emotion recognition and ToM. Our findings highlight the importance of using tasks depicting real-life social scenarios because of their greater sensitivity in the assessment of bvFTD. Moreover, our results contribute to the understanding of primary and intrinsic empathy deficits of bvFTD and have important theoretical and clinical implications.

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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.

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ABSTRACT There are few studies describing the functional changes in behavioral variant frontotemporal dementia (bvFTD) and it is not clear which aspects of functionality are affected by the disease. Objective: The aim of the present investigation was to characterize the functional profile of patients previously diagnosed with bvFTD. Methods: The sample consisted of 31 patients diagnosed with bvFTD, who were compared to patients with Alzheimer's disease (AD) (n=31) and to healthy control subjects (NC) (n=34), matched for schooling and age. bvFTD and AD patients were matched by severity of dementia. The protocol included the Mini-Mental State Examination (MMSE), Geriatric Depression Scale (GDS), Direct Assessment of Functional Status (DAFS-BR), Functional Activities Questionnaire (PFAQ), Disability Assessment for Dementia (DAD) and the Clinical Dementia Rating scale (CDR). Results: The group with bvFTD showed worse performance on Initiation and Planning/Organization in the DAD and on ability to feed oneself in the DAFS-BR, as well as higher scores on the PFAQ, suggesting greater dependence in the bvFTD group. Conclusion: The results suggest that individuals with bvFTD display greater functional impairment compared to AD patients with a similar degree of dementia severity and to healthy controls. Direct assessment of functionality proved unable to clearly differentiate between the dementia subtypes.

RESUMO Existem poucos estudos sobre alterações funcionais na variante comportamental da demência frontotemporal (DFTvc). Objetivo: Caracterizar o desempenho funcional de pacientes com diagnóstico prévio de DFTvc. Métodos: Trinta e um pacientes com DFTvc foram comparados a pacientes com doença de Alzheimer (DA) (n=31) e adultos saudáveis (NC) (n=34), pareados para idade e escolaridade. Os pacientes com DFTvc e DA foram pareados pela gravidade da demência. O protocolo incluiu o Mini Exame do Estado Mental, Escala de Depressão Geriátrica (GDS), Direct Assessment of Functional Status (DAFS-BR), Disability Assessment for Dementia (DAD), Functional Activities Questionnaire (PFAQ) e Clinical Dementia Rating scale (CDR). Resultados: O grupo com DFTvc apresentou pior desempenho em Iniciação e Planejamento/Organização na DAD, em Alimentação na DAFS-BR e pontuação mais elevada na PFAQ, sugerindo que a dependência na DFTvc é mais acentuada. Conclusão: Os resultados apresentados sugerem que indivíduos com DFTvc apresentam maior prejuízo funcional, quando comparados com participantes com DA com grau semelhante de gravidade e com adultos saudáveis. A avaliação direta da funcionalidade não ajudou a diferenciar os subtipos de demência de modo significativo.

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There are few studies describing the functional changes in behavioral variant frontotemporal dementia (bvFTD) and it is not clear which aspects of functionality are affected by the disease. OBJECTIVE: The aim of the present investigation was to characterize the functional profile of patients previously diagnosed with bvFTD. METHODS: The sample consisted of 31 patients diagnosed with bvFTD, who were compared to patients with Alzheimer's disease (AD) (n=31) and to healthy control subjects (NC) (n=34), matched for schooling and age. bvFTD and AD patients were matched by severity of dementia. The protocol included the Mini-Mental State Examination (MMSE), Geriatric Depression Scale (GDS), Direct Assessment of Functional Status (DAFS-BR), Functional Activities Questionnaire (PFAQ), Disability Assessment for Dementia (DAD) and the Clinical Dementia Rating scale (CDR). RESULTS: The group with bvFTD showed worse performance on Initiation and Planning/Organization in the DAD and on ability to feed oneself in the DAFS-BR, as well as higher scores on the PFAQ, suggesting greater dependence in the bvFTD group. CONCLUSION: The results suggest that individuals with bvFTD display greater functional impairment compared to AD patients with a similar degree of dementia severity and to healthy controls. Direct assessment of functionality proved unable to clearly differentiate between the dementia subtypes.

Existem poucos estudos sobre alterações funcionais na variante comportamental da demência frontotemporal (DFTvc). OBJETIVO: Caracterizar o desempenho funcional de pacientes com diagnóstico prévio de DFTvc. MÉTODOS: Trinta e um pacientes com DFTvc foram comparados a pacientes com doença de Alzheimer (DA) (n=31) e adultos saudáveis (NC) (n=34), pareados para idade e escolaridade. Os pacientes com DFTvc e DA foram pareados pela gravidade da demência. O protocolo incluiu o Mini Exame do Estado Mental, Escala de Depressão Geriátrica (GDS), Direct Assessment of Functional Status (DAFS-BR), Disability Assessment for Dementia (DAD), Functional Activities Questionnaire (PFAQ) e Clinical Dementia Rating scale (CDR). RESULTADOS: O grupo com DFTvc apresentou pior desempenho em Iniciação e Planejamento/Organização na DAD, em Alimentação na DAFS-BR e pontuação mais elevada na PFAQ, sugerindo que a dependência na DFTvc é mais acentuada. CONCLUSÃO: Os resultados apresentados sugerem que indivíduos com DFTvc apresentam maior prejuízo funcional, quando comparados com participantes com DA com grau semelhante de gravidade e com adultos saudáveis. A avaliação direta da funcionalidade não ajudou a diferenciar os subtipos de demência de modo significativo.

RESUMO

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.

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.