RESUMO
OBJECTIVE: Late-onset amnestic mild cognitive impairment (aMCI) with long disease course and slow progression has been recently recognized as a possible phenotypical expression of a limbic-predominant neurodegenerative disorder. Basic emotion recognition ability crucially depending on temporo-limbic integrity is supposed to be impaired in this group of MCI subjects presenting a selective vulnerability of medio-temporal and limbic regions. However, no study specifically investigated this issue. METHODS: Hereby, we enrolled 30 aMCI with a biomarker-based diagnosis of Alzheimer's disease (i.e., aMCI-AD, n = 16) or a biomarker evidence of selective medio-temporal and limbic degeneration (aMCI-mTLD, n = 14). Ekman-60 Faces Test (Ek-60F) was administered to each subject, comparing the performance with that of 20 healthy controls (HCs). RESULTS: aMCI-mTLD subjects showed significantly lower Ek-60F global scores compared to HC (p = 0.001), whose performance was comparable to aMCI-AD. Fear (p = 0.02), surprise (p = 0.005), and anger (p = 0.01) recognition deficits characterized the aMCI-mTLD performance. Fear recognition scores were significantly lower in aMCI-mTLD compared to aMCI-AD (p = 0.04), while no differences were found in other emotions. CONCLUSIONS: Impaired social cognition, suggested by defective performance in emotion recognition tasks, may be a useful cognitive marker to detect limbic-predominant aMCI subjects among the heterogeneous aMCI population.
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Doença de Alzheimer , Disfunção Cognitiva , Doença de Alzheimer/diagnóstico , Biomarcadores , Cognição , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/psicologia , Diagnóstico Diferencial , Emoções , Humanos , Testes Neuropsicológicos , FenótipoRESUMO
BACKGROUND: Alzheimer's Disease (AD) is a multifactorial disorder driven by genetic and modifiable lifestyle risk factors. Lifestyle primary prevention initiatives may reduce the prevalence and incidence of dementia in older adults. OBJECTIVES: The E.Mu.N.I study is a randomized controlled trial investigating the effect of multilevel non-pharmacologic interventions on cognitive performances (primary outcome) and structural and vascular brain MRI markers (secondary outcome), as well as markers of brain functional connectivity change (exploratory outcome), in older adults with subjective memory decline (SMD). Here, we present the study design and the baseline features of the sample. METHODS: Cognitively intact older adults with SMD, enrolled between February 2016 and June 2017, were randomly assigned to one of the 3 interventions for 1 year: Active Control Intervention (ACI), i.e., educational lessons; Partial Intervention (PI), i.e., homotaurine administration (100 mg/die) and lessons on the Mediterranean diet; Multilevel Intervention (MI), i.e., PI plus computerized cognitive training and physical exercise training. RESULTS: One-hundred and twenty-eight eligible participants were enrolled (66% female; age: 68 ± 5 years). Eighty-two percent of the sample was composed of volunteers with SMD from the community. Participants were randomly allocated to the interventions as follows: ACI (N = 40), PI (N = 44), MI (N = 44). No significant differences among groups emerged on socio-demographic, clinical-neuropsychological variables and MRI markers at baseline. CONCLUSIONS: The outcomes obtained from the E.Mu.N.I. study will clarify the efficacy of multilevel non-pharmacologic interventions on cognitive and neuroimaging markers in SMD individuals. This is a crucial step forward for the development of cost-effective non-pharmacologic primary prevention initiatives for AD.
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Demência/terapia , Transtornos da Memória/terapia , Memória , Idoso , Encéfalo/fisiopatologia , Demência/fisiopatologia , Exercício Físico , Feminino , Humanos , Estilo de Vida , Imageamento por Ressonância Magnética , Masculino , Transtornos da Memória/fisiopatologia , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Limbic encephalitis (LE) is characterized by an acute or subacute onset with memory impairments, confusional state, behavioral disorders, variably associated with seizures and dystonic movements. It is due to inflammatory processes that selectively affect the medial temporal lobe structures. Voltage-gate potassium channel (VGKC) autoantibodies are frequently observed. In this study, we assessed at the individual level FDG-PET brain metabolic dysfunctions and neuropsychological profiles in three autoimmune LE cases seropositive for neuronal VGKC-complex autoantibodies. MATERIALS AND METHODS: LGI1 and CASPR2 potassium channel complex autoantibody subtyping was performed. Cognitive abilities were evaluated with an in-depth neuropsychological battery focused on episodic memory and affective recognition/processing skills. FDG-PET data were analyzed at single-subject level according to a standardized and validated voxel-based Statistical Parametric Mapping (SPM) method. RESULTS: Patients showed severe episodic memory and fear recognition deficits at the neuropsychological assessment. No disorder of mentalizing processing was present. Variable patterns of increases and decreases of brain glucose metabolism emerged in the limbic structures, highlighting the pathology-driven selective vulnerability of this system. Additional involvement of cortical and subcortical regions, particularly in the sensorimotor system and basal ganglia, was found. CONCLUSIONS: Episodic memory and fear recognition deficits characterize the cognitive profile of LE. Commonalities and differences may occur in the brain metabolic patterns. Single-subject voxel-based analysis of FDG-PET imaging could be useful in the early detection of the metabolic correlates of cognitive and non-cognitive deficits characterizing LE condition.
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Autoanticorpos/sangue , Doenças Autoimunes , Medo/fisiologia , Fluordesoxiglucose F18 , Encefalite Límbica , Transtornos da Memória , Memória Episódica , Testes Neuropsicológicos , Tomografia por Emissão de Pósitrons/métodos , Canais de Potássio de Abertura Dependente da Tensão da Membrana/imunologia , Compostos Radiofarmacêuticos , Idoso , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico por imagem , Doenças Autoimunes/fisiopatologia , Feminino , Humanos , Encefalite Límbica/complicações , Encefalite Límbica/diagnóstico por imagem , Encefalite Límbica/fisiopatologia , Masculino , Transtornos da Memória/diagnóstico por imagem , Transtornos da Memória/etiologia , Transtornos da Memória/fisiopatologia , Pessoa de Meia-Idade , Reconhecimento Psicológico/fisiologiaRESUMO
OBJECTIVE: To identify overlapping and unique grey (GM) and white matter (WM) signatures within the frontotemporal lobar degeneration (FTLD) continuum, and discriminate likely FTLD-TAU and FTLD-TDP patients using structural and diffusion tensor (DT) magnetic resonance imaging (MRI). METHODS: T1-weighted and DT MRI were collected from 121 subjects: 35 motor neuron disease (MND), 14 behavioral variant of frontotemporal dementia, 12 semantic and 11 nonfluent primary progressive aphasia, 21 progressive supranuclear palsy syndrome patients, and 28 healthy controls. Patterns of GM atrophy were established using voxel-based morphometry. Tract-based spatial statistics was used to perform a WM voxelwise analysis of mean diffusivity and fractional anisotropy. RESULTS: In all clinical FTLD phenotypes, the pattern of WM damage was more distributed than that of GM atrophy. All patient groups, with the exception of MND cases with a pure motor syndrome, shared a focal GM atrophy centered around the dorsolateral and medial frontal cortex and a largely overlapping pattern of WM damage involving the genu and body of the corpus callosum and ventral frontotemporal and dorsal frontoparietal WM pathways. Surrounding this common area, phenotype (symptom)-specific GM and WM regions of damage were found in each group. CONCLUSIONS: In the FTLD spectrum, WM disruption is more severe than GM damage. Frontal cortex and WM pathways represent the common target of neurodegeneration in these conditions. The topographic pattern of damage supports a "prion-like" protein propagation through WM connections as underlying mechanism of the stereotyped progression of FTLD.
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Degeneração Lobar Frontotemporal/patologia , Substância Cinzenta/patologia , Imageamento por Ressonância Magnética/métodos , Doença dos Neurônios Motores/patologia , Paralisia Supranuclear Progressiva/patologia , Substância Branca/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Atrofia/patologia , Imagem de Tensor de Difusão/métodos , Feminino , Demência Frontotemporal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Afasia Primária Progressiva não Fluente/patologiaRESUMO
PURPOSE: To test a multimodal magnetic resonance (MR) imaging-based approach composed of cortical thickness and white matter (WM) damage metrics to discriminate between variants of primary progressive aphasia (PPA) that are nonfluent and/or agrammatic (NFVPPA) and semantic (SVPPA). MATERIALS AND METHODS: This study was approved by the local ethics committees on human studies, and written informed consent from all patients was obtained before their enrollment. T1-weighted and diffusion-tensor (DT) MR images were obtained from 13 NFVPPA patients, 13 SVPPA patients, and 23 healthy control participants. Cortical thickness and DT MR imaging indices from the long-associative and interhemispheric WM tracts were obtained. A random forest (RF) analysis was used to identify the image features associated with each clinical syndrome. Individual patient classification was performed by using receiver operator characteristic curve analysis with cortical thickness, DT MR imaging, and a combination of the two modalities. RESULTS RF analysis showed that the best markers to differentiate the two PPA variants at an individual patient level among cortical thickness and DT MR imaging metrics were diffusivity abnormalities of the left inferior longitudinal and uncinate fasciculi and cortical thickness measures of the left temporal pole and inferior frontal gyrus. A combination of cortical thickness and DT MR imaging measures (the so-called gray-matter-and-WM model) was able to distinguish patients with NFVPPA and SVPPA with the following classification pattern: area under the curve, 0.91; accuracy, 0.89; sensitivity, 0.92; specificity, 0.85. Leave-one-out analysis demonstrated that the gray matter and WM model is more robust than the single MR modality models to distinguish PPA variants (accuracy was 0.86, 0.73, and 0.68 for the gray matter and WM model, the gray matter-only model, and the WM-only model, respectively). CONCLUSION: A combination of structural and DT MR imaging metrics may provide a quantitative procedure to distinguish NFVPPA and SVPPA patients at an individual patient level. The discrimination accuracies obtained suggest that the gray matter and WM model is potentially relevant for the differential diagnosis of the PPA variants in clinical practice.
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Afasia Primária Progressiva/classificação , Afasia Primária Progressiva/patologia , Córtex Cerebral/patologia , Imagem de Tensor de Difusão , Imageamento por Ressonância Magnética , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: To assess white matter (WM) tract damage in patients with atypical Alzheimer disease (AD), including early-onset AD (EOAD), logopenic variant of primary progressive aphasia (lvPPA), and posterior cortical atrophy (PCA), by using diffusion-tensor magnetic resonance (MR) imaging and to identify similarities and differences across the AD spectrum. MATERIALS AND METHODS: This study was approved by the local ethical committees on human studies, and written informed consent from all subjects was obtained prior to enrollment. WM tract damage and cortical atrophy were assessed by using diffusion-tensor MR imaging and voxel-based morphometry, respectively, in 28 patients with EOAD, 12 patients with lvPPA, and 13 patients with PCA relative to age- and sex-matched healthy subjects. Conjunction and interaction analyses were used to define overlapping and syndrome-specific patterns of brain damage. RESULTS: Patients with EOAD, lvPPA, and PCA shared a common pattern of WM damage that involved the body of the corpus callosum, fornix, and main anterior-posterior pathways (P < .05). They also shared cortical atrophy of the left temporoparietal regions and precuneus (P < .05, family-wise error corrected). Patients with EOAD also had specific damage to the genu and splenium of the corpus callosum and parahippocampal tract bilaterally (P < .05). In all patients with AD, particularly in the two focal forms (lvPPA and PCA), WM damage was more severe and widely distributed than expected on the basis of cortical atrophy. CONCLUSION: In atypical AD clinical phenotypes, the distribution of WM damage exceeds cortical atrophy and may reflect the pathologic dissemination through structural connections from atrophic to unaffected cortical regions. WM degeneration may be an early marker of AD pathologic changes in EOAD and focal AD forms.
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Doença de Alzheimer/patologia , Afasia Primária Progressiva/patologia , Córtex Cerebral/patologia , Imagem de Difusão por Ressonância Magnética , Substância Branca/patologia , Idoso , Idoso de 80 Anos ou mais , Atrofia , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Theory of Mind (ToM), the process by which an individual imputes mental states to himself and others, is presently considered as a multidimensional cognitive domain, with two main facets (i.e., cognitive and affective ToM) accounting, respectively, for the ability to understand others' intention (intention attribution-IA) and emotions (emotion attribution-EA). Despite the large amount of literature investigating the behavioural and neural bases of mentalizing abilities in neurological conditions, there is still a lack of validated neuropsychological tools specifically designed to assess such skills. Here, we report the normative data of the Story-Based Empathy Task (SET), a non-verbal test developed for the assessment of intention and emotion attribution in the neurodegenerative conditions characterized by the impairment of social-emotional abilities. It is an easy-to-administer task including 18 stimuli, sub-grouped into two experimental conditions assessing, respectively, the ability to infer others' intentions (SET-IA) and emotions (SET-EA), compared to a control condition of causal inference (SET-CI). Normative data were collected in 136 Italian subjects pooled across subgroups homogenous for age (range 20-79 years), sex, and education (at least 5 years). The results show a detrimental effect of age and a beneficial effect of education on both the global score and each subscale, for which we provide correction grids. This new task could be a useful tool to investigate both affective and cognitive aspects of ToM in the course of disorders of socio-emotional behaviour, such as the fronto-temporal dementia spectrum.
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Emoções/fisiologia , Empatia , Intenção , Testes Neuropsicológicos/normas , Teoria da Mente/fisiologia , Adulto , Fatores Etários , Idoso , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Valores de Referência , Adulto JovemRESUMO
The Ekman 60-Faces (EK-60F) Test is a well-known neuropsychological tool assessing emotion recognition from facial expressions. It is the most employed task for research purposes in psychiatric and neurological disorders, including neurodegenerative diseases, such as the behavioral variant of Frontotemporal Dementia (bvFTD). Despite its remarkable usefulness in the social cognition research field, to date, there are still no normative data for the Italian population, thus limiting its application in a clinical context. In this study, we report procedures and normative data for the Italian version of the test. A hundred and thirty-two healthy Italian participants aged between 20 and 79 years with at least 5 years of education were recruited on a voluntary basis. They were administered the EK-60F Test from the Ekman and Friesen series of Pictures of Facial Affect after a preliminary semantic recognition test of the six basic emotions (i.e., anger, fear, sadness, happiness, disgust, surprise). Data were analyzed according to the Capitani procedure [1]. The regression analysis revealed significant effects of demographic variables, with younger, more educated, female subjects showing higher scores. Normative data were then applied to a sample of 15 bvFTD patients which showed global impaired performance in the task, consistently with the clinical condition. We provided EK-60F Test normative data for the Italian population allowing the investigation of global emotion recognition ability as well as selective impairment of basic emotions recognition, both for clinical and research purposes.
Assuntos
Emoções , Face , Expressão Facial , Reconhecimento Visual de Modelos/fisiologia , Adulto , Idoso , Escolaridade , Feminino , Humanos , Itália , Masculino , Entrevista Psiquiátrica Padronizada , Pessoa de Meia-Idade , Testes Neuropsicológicos , Estimulação Luminosa , Valores de Referência , Adulto JovemRESUMO
OBJECTIVE: Loss of empathy is a symptom of the behavioral variant of frontotemporal dementia (bvFTD), constituting a clue for early diagnosis. In this study, we directly compared two empathy components (intention attribution [IA] and emotion attribution [EA]), correlating them with possible specific patterns of gray-matter density reduction within the mentalizing network. METHODS: We evaluated IA and EA in 18 mild bvFTD patients compared with 36 healthy controls (HCs) using a single nonverbal test. A subgroup entered a voxel-based morphometry study. RESULTS: Compared with HC, bvFTD patients showed IA and EA impairments. EA performance correlated with gray-matter reduction in the right amygdala, left insula, and posterior-superior temporal sulcus extending into the temporoparietal junction. CONCLUSION: We proved an empathic impairment, with the ability to infer emotional states showing the most severe deficit. These results provide further evidence of selective disease-specific vulnerability of the limbic and frontoinsular network in bvFTD and highlight the usefulness of empathy assessment in early patients.
Assuntos
Mapeamento Encefálico , Encéfalo/patologia , Empatia/fisiologia , Demência Frontotemporal/complicações , Transtornos do Humor/etiologia , Transtornos do Humor/patologia , Adulto , Idoso , Feminino , Demência Frontotemporal/psicologia , Humanos , Processamento de Imagem Assistida por Computador , Imageamento por Ressonância Magnética , Masculino , Entrevista Psiquiátrica Padronizada , Pessoa de Meia-Idade , Testes Neuropsicológicos , Adulto JovemRESUMO
BACKGROUND: Although social cognitive dysfunction is a major feature of the behavioral variant of frontotemporal dementia (bvFTD), quantitative measurement of social behavior changes is poorly available in clinical settings. OBJECTIVE: The aim of the study is to evaluate diagnostic accuracy of social-emotional questionnaires in distinguishing bvFTD from healthy control (HC) subjects and Alzheimer's disease (AD) patients. METHODS: We enrolled 29 bvFTD, 24 AD, and 18 HC subjects matched for age, sex, and education. Two informant-based measures of socio-emotional sensitivity and empathy (i.e., revised Self-Monitoring Scale (rSMS) and Interpersonal Reactivity Index (IRI)) were administered. One-way ANOVA was performed to compare groups, whereas Receiver Operating Characteristics (ROC) curve analysis tested questionnaire ability in distinguishing groups. A short version of IRI (sIRI) was obtained by excluding the non-contributing subscale (i.e., personal distress). RESULTS: Compared to HC and AD, bvFTD showed significantly lower scores in rSMS and IRI scores, except for IRI personal distress subscale. The sIRI showed an excellent performance in early diagnosis (bvFTD versus HCâ=âAUC 0.95). Both sIRI and rSMS showed good performance in distinguishing bvFTD from AD (AUC 0.83). CONCLUSIONS: ROC analyses support the usefulness of informant social questionnaires in memory clinics and their potential value in screening procedures for research eligibility in forthcoming trials. In the timely diagnosis of bvFTD patients, IRI and rSMS may supply crucial information for the early detection of signs and symptoms affecting social-emotional skills, which might otherwise be underrecognized.
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Doença de Alzheimer , Demência Frontotemporal , Humanos , Demência Frontotemporal/diagnóstico , Demência Frontotemporal/psicologia , Testes Neuropsicológicos , Emoções , Doença de Alzheimer/diagnóstico , Doença de Alzheimer/psicologia , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Primary progressive aphasia (PPA) diagnostic criteria underestimate the complex presentation of semantic (sv) and logopenic (lv) variants, in which symptoms partially overlap, and mixed clinical presentation (mixed-PPA) and heterogenous profile (lvPPA +) are frequent. Conceptualization of similarities and differences of these clinical conditions is still scarce. METHODS: Lexical, semantic, phonological, and working memory errors from nine language tasks of sixty-seven PPA were analyzed using Profile Analysis based on Multidimensional Scaling, which allowed us to create a distributed representation of patients' linguistic performance in a shared space. Patients had been studied with [18F] FDG-PET. Correlations were performed between metabolic and behavioral data. RESULTS: Patients' profiles were distributed across a continuum. All PPA, but two, presented a lexical retrieval impairment, in terms of reduced production of verbs and nouns. svPPA patients occupied a fairly clumped space along the continuum, showing a preponderant semantic deficit, which correlated to fusiform gyrus hypometabolism, while only few presented working memory deficits. Adjacently, lvPPA + presented a semantic impairment combined with phonological deficits, which correlated with metabolism in the anterior fusiform gyrus and posterior middle temporal gyrus. Starting from the shared phonological deficit side, a large portion of the space was occupied by all lvPPA, showing a combination of phonological, lexical, and working memory deficits, with the latter correlating with posterior temporo-parietal hypometabolism. Mixed PPA did not show unique profile, distributing across the space. DISCUSSION: Different clinical PPA entities exist but overlaps are frequent. Identifying shared and unique clinical markers is critical for research and clinical practice. Further research is needed to identify the role of genetic and pathological factors in such distribution, including also higher sample size of less represented groups.
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Afasia Primária Progressiva , Semântica , Humanos , Análise de Escalonamento Multidimensional , Linguística , Fluordesoxiglucose F18 , Transtornos da Memória , Afasia Primária Progressiva/diagnóstico por imagemRESUMO
Mutations in progranulin gene (GRN) are the most common cause of autosomal dominant familial frontotemporal lobar degeneration (FTLD). In addition, GRN variability influences the risk to develop the disease in non-carriers (sporadic FTLD). We evaluated progranulin gene (GRN) promoter methylation levels in peripheral blood mononuclear cells isolated from 38 patients with sporadic FTLD compared with 38 controls, and correlate them with GRN mRNA expression rate. The percentage of methylation of the GRN promoter was increased in patients with FTLD compared with controls (61.5 vs. 46.3 %, P < 0.001). A trend towards decreased GRN relative expression was observed in patients compared with controls (threefold decrease over controls, P > 0.05), together with a negative correlation between the degree of GRN promoter methylation and mRNA GRN levels (ρ = -0.1, P > 0.05). GRN promoter methylation was not correlated with age. In conclusion, the degree of methylation of the GRN promoter is increased in patients with FTLD as compared with controls, likely leading to a decreased expression of GRN.
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Degeneração Lobar Frontotemporal/genética , Peptídeos e Proteínas de Sinalização Intercelular/genética , Regiões Promotoras Genéticas/fisiologia , Adulto , Análise Mutacional de DNA , Feminino , Degeneração Lobar Frontotemporal/sangue , Humanos , Peptídeos e Proteínas de Sinalização Intercelular/sangue , Masculino , Metilação , Pessoa de Meia-Idade , Mutação/genética , Progranulinas , RNA Mensageiro/metabolismoRESUMO
BACKGROUND: Assess the impact of an educational Planetary Health Plate (PHP) graphic on meat-related dietary choices of Stanford University dining hall patrons using a randomized controlled trial crossover design. All patrons entering the dining hall during study periods were enrolled as participants. Control, n = 631; PHP, n = 547. METHODS: Compare dietary behavior without signage to behavior while exposed to PHP during four equivalent dinner meals. The primary outcome was total meat-dish weight adjusted for the number of people entering the dining hall. Secondary outcomes included the number of meat-dish servings and average meat-dish serving weight. Analysis using T-tests, Poisson generalized linear model. RESULTS: Differences in total meat-dish weight, (1.54 kg; 95% Confidence Interval [CI] = -4.41,1.33; P = .19) and average meat-dish serving weight (0.03 kg; 95% CI = 0.00, 0.06; P = .07) between PHP and control patrons did not reach significance. The rate at which PHP patrons took meat was significantly lower (Incidence Rate Ratio 0.80; 95% CI = 0.71, 0.91; P < .001). CONCLUSION: Exposure to an educational plate graphic decreased the proportion of patrons taking meat but had no impact on total meat consumption or meat-dish serving weight. Statistical methods used in this study may inform future investigations on dietary change in the dining hall setting. Further research on the role of educational signage in influencing dietary behavior is warranted, with an aim to improve human health and environmental sustainability. TRIAL REGISTRATION: ClinicalTrials.gov, NCT05565859, registered 4 October 2022.
RESUMO
Picture naming tests are widely used to evaluate language impairments in neurodegenerative diseases, especially in Primary Progressive Aphasia (PPA). The available tests differ for many factors affecting the performance, e.g. format of stimuli and their psycholinguistic properties. We aim to identify the most appropriate naming test to be used on PPA according to the clinical and research demands. We investigated the behavioural characteristics, i.e. proportion of correct responses and error type, and their neural correlates in two Italian naming tests, CaGi naming (CaGi) and naming subtest of the Screening for Aphasia in NeuroDegeneration battery (SAND), administered to 52 PPA patients who underwent an FDG-PET scan. We analysed the effectiveness of the tests in distinguishing PPA versus controls and among PPA variants, considering the psycholinguistic variables affecting performance. We explored the brain metabolic correlates of behavioural performance in the tests. SAND, differently from CaGi, has time limits for the response and its items are less frequent and acquired later. SAND and CaGi differed in terms of number of correct responses and error profile, suggesting a higher difficulty to name SAND items compared to CaGi. Semantic errors predominated in CaGi, while anomic and semantic errors were equally frequent in SAND. Both tests distinguished PPA from controls, but SAND outperformed CaGi in discriminating among PPA variants. FDG-PET imaging revealed a shared metabolic involvement of temporal areas associated with lexico-semantic processing, encompassing anterior fusiform, temporal pole, and extending to posterior fusiform in sv-PPA. Concluding, a picture naming test with response time limit and items which are less frequent and acquired later in life, as SAND, may be effective at highlighting subtle distinctions between PPA variants, improving the diagnosis. Conversely, a naming test without time limit for the response, as CaGi, may be useful for a better characterization of the nature of the naming impairment at the behavioural level, eliciting more naming errors than anomia, possibly helping in the development of rehabilitation protocols.
Assuntos
Afasia Primária Progressiva , Encéfalo , Testes Neuropsicológicos , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/psicologia , Humanos , Masculino , Feminino , Idoso , Tomografia por Emissão de Pósitrons , Psicolinguística , Comportamento , Neuroimagem , Encéfalo/diagnóstico por imagemRESUMO
Saitohin (STH) is an intronless gene nested within the human tau gene, which contains a single nucleotide polymorphism (A/G), suggested to be involved in the physiopathology and clinical course of several neurodegenerative and neuropsychiatric diseases. Recently, an association between this polymorphism and frontal hypoperfusion and clinical prognosis in frontotemporal dementia was reported. The present study sought to evaluate the possible role of the STH polymorphism as a concurring factor of cognitive decline in schizophrenia, a disease sharing both early psychotic manifestations, a core deficit of executive functions and hypofrontality with frontotemporal lobe dementia. 220 clinically stabilized patients with schizophrenia were assessed with the Wisconsin Card Sorting Test (WCST) for evaluation of executive functions and compared for STH allele frequency with 48 patients affected by frontotemporal dementia and 47 healthy subjects. There was no significant difference in allelic distribution between the healthy controls and all other groups, while we observed a significantly greater frequency of G allele among both patients with frontotemporal dementia (p = 0.037) and schizophrenia patients with poor performances of WCST (p = 0.044), compared to schizophrenia patients with best WCST performances. Among the patients with schizophrenia, stratified for age and gender, the STH polymorphism resulted in a significant predictor of WCST performance (p = 0.007). These results suggest a possible contribution of STH gene products on the heterogeneity of core frontal executive functions deterioration, probably through complex interactions with mechanism involved in neurodevelopment and neurodegeneration.
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Transtornos Cognitivos/etiologia , Transtornos Cognitivos/genética , Função Executiva/fisiologia , Polimorfismo de Nucleotídeo Único , Esquizofrenia/genética , Proteínas tau/genética , Idoso , Feminino , Predisposição Genética para Doença/genética , Genótipo , Humanos , Masculino , Testes Neuropsicológicos , Esquizofrenia/complicações , Psicologia do EsquizofrênicoRESUMO
BACKGROUND: The Frontal Behavioral Inventory (FBI) is a questionnaire designed to quantify behavioral changes in frontotemporal dementia (FTD). Literature showed heterogeneous FBI profiles in FTD versus Alzheimer's disease (AD) with variable occurrence of positive and negative symptoms. OBJECTIVE: In this study, we constructed a short FBI version (i.e., mini-FBI) with the aim to provide clinicians with a brief tool for the identification of early behavioral changes in behavioral variant of FTD (bvFTD), also facilitating the differential diagnosis with AD. METHODS: 40 bvFTD and 33 AD patients were enrolled. FBI items were selected based on internal consistency and exploratory factor analysis. Convergent validity of mini-FBI was also assessed. A behavioral index (i.e., B-index) representing the balance between positive and negative mini-FBI symptoms was computed in order to analyze its distribution in bvFTD through a cluster analysis and to compare performance among patient groups. RESULTS: The final version of the mini-FBI included 12 items, showing a significant convergent validity with the Neuropsychiatric Inventory scores (rpâ=â0.61, pâ<â0.001). Cluster analysis split patients in four clusters. bvFTD were included in three different clusters characterized by prevalent positive symptoms, both positive and negative symptoms, or prevalent negative behavioral alterations, similar to a subset of AD patients. A fourth cluster included only AD patients showing no positive symptoms. CONCLUSION: The mini-FBI is a valuable easily administrable questionnaire able to early identify symptoms effectively contributing to the bvFTD behavioral syndrome, aiding clinician in diagnosis and management.
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Doença de Alzheimer , Demência Frontotemporal , Doença de Alzheimer/psicologia , Sintomas Comportamentais/diagnóstico , Sintomas Comportamentais/etiologia , Sintomas Comportamentais/psicologia , Demência Frontotemporal/diagnóstico , Demência Frontotemporal/psicologia , Humanos , Testes Neuropsicológicos , FenótipoRESUMO
Primary progressive aphasia (PPA) classification relies on profile characterization of quantitatively impaired/spared performance in language tasks. In this study, we coextracted 8 qualitative types of errors in 67 PPA patients submitted to a comprehensive language assessment. Canonical correlation analysis was applied to simultaneously correlate qualitative errors and brain metabolism, collected with FDG-PET. Results showed the contribution of semantic, syntactic and working memory errors associated with specific correlates of regional metabolic changes. Reduced metabolism in the left fusiform gyrus, anterior-middle and inferior-temporal gyri and middle-temporal pole correlated with an increase of semantic errors. Hypometabolism in the left inferior, middle, and superior frontal gyri, insula and right middle-occipital gyrus was related to syntactic errors. Higher metabolism in the bilateral pallidum, putamen, and left thalamus, as well as hypometabolism in the left angular and supramarginal gyri, inferior-parietal lobule, posterior-middle and inferior-temporal gyri and posterior cingulum predicted the increase of working memory errors. A relevant role of working memory subcomponents was associated with distinct neural systems. Patients' profiles are easily represented in a qualitative multidimensional space, in which mixed PPA overlapped with different phenotypes.
Assuntos
Afasia Primária Progressiva , Humanos , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/complicações , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons/métodos , Idioma , Semântica , Imageamento por Ressonância Magnética/métodos , Encéfalo/diagnóstico por imagemRESUMO
Ultra-processed food (UPF) consumption poses a potential risk to public health and may be related to shelter-in-place orders. This study utilized the level of food processing as a lens by which to examine the relationships between diet, weight change, and lifestyle changes (including cooking, snacking, and sedentary activity) that occurred during regional shelter-in-place orders. This study used a cross-sectional, retrospective survey (n = 589) to assess baseline demographics, changes in lifestyle behaviors using a Likert scale, and changes in dietary behaviors using a modified food frequency questionnaire from mid-March to May 2020; data were collected in the California Bay Area from August to October 2020. Foods were categorized by level of processing (minimally processed, processed, and ultra-processed) using the NOVA scale. Stepwise multiple linear regression and univariate linear regression models were used to determine the associations between these factors. Increased snacking was positively associated with a change in the percent of the calories derived from UPF and weight gain (ß = 1.0, p < 0.001; ß = 0.8 kg, p < 0.001) and negatively associated with the share of MPF calories consumed (ß = -0.9, p < 0.001). These relationships have public health implications as interventions designed around decreased snacking may positively impact diet and weight management and thereby mitigate negative health outcomes.
RESUMO
BACKGROUND: Severe socio-emotional impairments characterize the behavioral variant of frontotemporal dementia (bvFTD). However, literature reports social cognition disorders in other dementias. OBJECTIVE: In this study, we investigated the accuracy of social cognition performances in the early and differential diagnosis of bvFTD. METHODS: We included 131 subjects: 32 bvFTD, 26 Alzheimer's disease (AD), 16 primary progressive aphasia (PPA), 17 corticobasal syndrome (CBS), and 40 healthy control (HC). Each subject completed the Ekman 60 faces (Ek-60F) test assessing basic emotion recognition and the Story-based Empathy Task (SET) assessing attribution of intentions/emotions. A combined social measure (i.e., Emotion Recognition and Attribution (ERA) index) was calculated. One-way ANOVA has been used to compare performances among groups, while receiver operating characteristic (ROC) curve tested measures ability to distinguish subjects with and without bvFTD. RESULTS: Ek-60F and ERA index scores were significantly lower in bvFTD versus HC, AD, and PPA groups. ROC analyses significantly distinguished bvFTD from HC (AUC 0.82-0.92), with the Ek-60F test showing the highest performance, followed by the ERA index. These two social measures showed the best accuracy in detecting bvFTD from AD (AUC 0.78-0.74) and PPA (AUC 0.80-0.76). Investigated measures failed in detecting bvFTD from CBS. CONCLUSION: Accuracy analyses support the advantage of using social cognition tests for bvFTD diagnosis. Short social battery may reduce uncertainties and improve disease identification in clinical settings. We recommend a revision of current clinical criteria considering neuropsychological deficits in emotion recognition and processing tasks as key cognitive markers of this neurodegenerative syndrome.