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Brief cognitive tests (BCTs) are necessary for early detection of cognitive impairment, particularly in primary care settings. Objective: This report describes a systematic review of BCTs evaluated in Peruvian populations. Methods: We used systematic mapping techniques to identify articles on screening tests for cognitive impairment involving Peruvian subjects. We included studies published in English and Spanish up to 2018. We reviewed 6 reference databases within the Virtual Health Library network, as well as the Web of Science, Scopus (MEDLINE), and EMBASE databases. Results: Ten out of 447 articles met the inclusion criteria. Studies included both outpatient (9) and community-based (2) samples. Eligibility criteria of the studies were similar. Although different protocols were applied, the diagnostic criteria were standardized. For discrimination between dementia and controls, IFS (AUC: 0.99) and ACE (AUC: 0.95 to 1.00) showed superior performance, as did the M@T (AUC: 1.00) and CDT-Mv (AUC: 0.94 to 1.00) for discriminating between Alzheimer’s disease (AD) and controls. Conclusion: The available evidence is limited. However, our analysis of national data suggests that the ACE may be a good choice whenever it can be applied to Peruvian patients. Alternatively, the M@T and IFS can be used for screening patients with suspected AD or FTD, respectively. Resumo. Testes cognitivos breves (TCBs) são necessários para a detecção precoce do comprometimento cognitivo, particularmente nos serviços de atenção primária. Objetivo: Este artigo descreve uma revisão sistemática dos TCBs avaliados em populações peruanas. Métodos: Utilizamos técnicas de mapeamento sistemático para identificar artigos sobre testes de triagem para comprometimento cognitivo envolvendo indivíduos peruanos. Incluímos estudos publicados em inglês e espanhol até 2018. Revisamos 6 bancos de dados de referência na rede da Biblioteca Virtual em Saúde e no Web of Science; Scopus (MEDLINE) e banco de dados EMBASE. Resultados: Dez dos 447 artigos preencheram os critérios de inclusão. Os estudos incluíram amostras ambulatoriais (9) e comunitárias (2). Os critérios de elegibilidade entre os estudos foram semelhantes. Embora os diferentes protocolos tenham sido aplicados, os critérios diagnósticos foram padronizados. Para a discriminação entre demência e controles, INECO Frontal Screening (IFS) (AUC: 0.99), Addenbrooke’s Cognitive Examination (ACE) (AUC: 0.95 to 1.00) mostraram desempnho superior, assim como o Memory Alteration Test (M@T) (AUC: 1.00) o Desenho do relógio (CDT-Mv) (AUC: 0,94 a 1,00) para discriminação entre a doença de Alzheimer (DA) e os controles. Conclusão: As evidências disponíveis são limitadas. No entanto, nossa análise com dados nacionais sugere que o ACE pode ser uma boa opção sempre que possível com pacientes peruanos. Alternativamente, o M @ T e o IFS podem ser usados para rastrear pacientes com suspeita de DA ou DFT, respectivamente.

Frontotemporal dementia (FTD) is a progressive neurodegenerative disorder accompanied by behavioral and personality changes and/or language deterioration. Its behavioral variant (bvFTD) is the main clinical presentation. Objective: This study aims to investigate the treatment alternatives for bvFTD available so far. Methods: We conducted a narrative review of bvFTD treatment options. We used PubMed and Lilacs databases with the terms “frontotemporal dementia” or “behavioral variant frontotemporal dementia” combined with “treatment,” “pharmacological treatment,” or “disease-modifying drugs.” Results: The articles retrieved and selected in the research pointed out that there is no specific treatment approved for bvFTD so far. The current proposals are limited to handle the cardinal behavioral symptoms of the disorder. Disease-modifying drugs are under development and may be promising, especially in the monogenic presentations of FTD. Conclusions: There are numerous approaches to treat the core symptoms of bvFTD, most of them based on low-quality research. To date, there are no drugs with a disease-specific therapeutic recommendation for bvFTD. Treatments are often investigated guided by primary psychiatric disorders with similar symptoms and should be chosen by the predominant symptom profile. RESUMO A demência frontotemporal (DFT) é um transtorno neurodegenerativo progressivo acompanhado de deterioração do comportamento e da personalidade e/ou da linguagem. A variante comportamental (DFTvc) é a principal apresentação clínica. Objetivos: Investigar as alternativas de tratamento disponíveis para a DFTvc até o momento. Métodos: Realizou-se uma revisão narrativa das opções de tratamento da DFTvc. Os bancos de dados PubMed e Lilacs foram utilizados com os termos “demência frontotemporal” ou “variante comportamental da demência frontotemporal” combinados com “tratamento”, “tratamento farmacológico” ou “drogas modificadoras de doença”. Resultados: Os artigos recuperados e selecionados na pesquisa indicaram que não há nenhum tratamento específico aprovado até o momento para DFTvc. As propostas atuais são limitadas ao tratamento dos sintomas comportamentais cardinais do transtorno. As drogas modificadoras de doença estão em desenvolvimento e podem ser promissoras, especialmente nas apresentações monogênicas da DFT. Conclusões: Há inúmeras abordagens para tratar os principais sintomas DFTvc, a maioria delas baseada em pesquisas de baixa qualidade. Até o momento, não existem medicamentos com uma recomendação terapêutica específica para a DFTvc. Os tratamentos são frequentemente investigados guiados por distúrbios psiquiátricos primários com sintomas semelhantes e devem ser escolhidos pelo perfil de sintomas predominante.

Judgment is the ability to make sound decisions after consideration of relevant information, possible solutions, likely outcomes, and contextual factors. Loss of judgment is common in patients with mild cognitive impairment (MCI) and dementia. The Test of Practical Judgment (TOP-J) evaluates practical judgment in adults and the elderly, with 15- and 9-item versions that require individuals to listen to scenarios about everyday problems and report their solutions. Objective: Adaptation of TOP-J for a Brazilian sample, preparation of a reduced version and verification of the accuracy of both. Methods: Eighty-five older adults, including 26 with MCI, 20 with Alzheimer’s disease (AD), 15 with frontotemporal dementia behavioral variant (FTDbv) and 24 controls, underwent neuropsychological assessment including the Brazilian adaptation of the TOP-J (TOP-J-Br). Results: On both TOP-J-Br versions, controls outperformed MCI, AD and FTDbv patients (p

Background: Frontotemporal dementia (FTD) is a neurodegenerative disease and is one of the most common causes of dementia in people under 65. There is often a significant diagnostic delay, as FTD can be confused with other psychiatric conditions. A lack of knowledge regarding FTD by health professionals is one possible cause for this diagnostic confusion. Objectives: The aim of this study was to adapt and validate the Frontotemporal Dementia Knowledge Scale (FTDKS) in Spanish. Methods: A translation was done, following cross-cultural adaptation guidelines, which consisted of forward translation, blind back translation, and an analysis by a committee of experts. For the present study, 134 professionals from different health areas responded the Spanish version of the FTDKS. The statistical analysis was performed using R version 4.0.0 “Arbor day” and the Psych, sjPlot packages. Results: The Spanish version of the FTDKS had good reliability and internal consistency (Cronbach alpha 0.74.). The sample's mean score was 19.78 (range = 4-32, SD 6.3) out of a maximum of 36 points. Conclusions: The results obtained show that the Spanish version has good psychometric properties. The FTDKS is applicable in our environment and can be a useful tool to evaluate the knowledge of health professionals regarding frontotemporal dementia. RESUMEN Antecedentes: La demencia frontotemporal (DFT) es una enfermedad neurodegenerativa y es una de las causas más comunes de demencia en personas menores de 65 años. A menudo existe un retraso significativo en el diagnóstico, ya que la FTD puede confundirse con otras afecciones psiquiátricas. La falta de conocimientos sobre la DFT por parte de los profesionales de salud es una posible causa de esta confusión diagnóstica. Objetivos: El presente estudio describe nuestros esfuerzos para adaptar y validar la Escala de Conocimiento de la Demencia Frontotemporal (FTDKS) en español. Métodos: Se realizó una traducción, siguiendo las pautas de adaptación transcultural, que consistió en una traducción directa, una traducción inversa ciega y un análisis por parte de un comité de expertos. Para el presente estudio, 134 profesionales de diferentes áreas de la salud respondieron la versión en español del FTDKS. El análisis estadístico se realizó utilizando la versión 4.0.0 de R "Arbor day" y los paquetes Psych, sjPlot. Resultados: La versión en español del FTDKS tiene una buena fiabilidad y consistencia interna (alfa de Cronbach 0,74.). La puntuación media de la muestra fue de 19,78 (rango = 4-32, SD 6,3) sobre un máximo de 36 puntos. Conclusiones: Los resultados obtenidos muestran que la versión española tiene buenas propiedades psicométricas. El FTDKS es aplicable en nuestro medio y puede ser una herramienta útil para evaluar los conocimientos de los profesionales sanitarios sobre la demencia frontotemporal.

Background: Swallowing and feeding problems may occur with the progression of behavioral variant frontotemporal dementia (bvFTD) and can impair the anticipatory and oral preparatory phases of swallowing. Objective: To characterize swallowing problems and the feeding situation of patients with bvFTD and to correlate the swallowing problems with functionality, executive functions, cognitive and behavioral features. Methods: Consecutive outpatients with bvFTD in mild, moderate and severe dementia stages were recruited along with their caregivers. Patients and caregivers were screened with the following scales: “Mini-Mental State Examination”, “Severe Mini-Mental State Examination”, “FTLD-modified Clinical Dementia Rating”, “Neuropsychiatric Inventory”, “Frontal Assessment Battery”, “Index of Independence in Activities of Daily Living”, “Swallowing Rating Scale” and “Assessment of Feeding and Swallowing Difficulties in Dementia”. Results: Overall, thirty patients with bvFTD were included along with their caregivers. Patients with bvFTD showed feeding and swallowing difficulties such as: messy to eat, passivity, coughing and choking, difficulty with some food consistencies and with specific food. Swallowing problems in bvFTD correlated with impaired functionality (p

Alzheimer’s disease (AD) and frontotemporal dementia (FTD) are neurodegenerative disorders that result in a significant burden to both patients and caregivers. By 2050, the number of people with dementia in Latin America will increase 4-fold. A deep understanding of the relevant genetic factors of AD and FTD is fundamental to tackle this reality through prevention. A review of different genetic variants that cause AD or FTD in Latin America was conducted. We searched Medline and PubMed databases using the keywords “Alzheimer’s disease,” “frontotemporal dementia,” “mutation,” “America,” and “Latin America,” besides specific Latin American countries. Forty-five items were chosen and analyzed. PSEN1 mutations are the commonest cause of genetic early-onset Alzheimer’s disease (EOAD), followed by PSEN2 and APP mutations. Genetic FTD can be mainly explained by GRN and MAPT mutations, as well as C9orf72 G4C2 repeat expansion. APOE ε4 can modify the prevalence and incidence of late-onset Alzheimer’s disease (LOAD), in addition to the cognitive performance in affected carriers. RESUMO. A doença de Alzheimer (DA) e a demência frontotemporal (DFT) são distúrbios neurodegenerativos que causam uma sobrecarga significativa para pacientes e cuidadores. Em 2050, o número de pessoas com demência na América Latina aumentará 4 vezes. Uma compreensão profunda dos fatores genéticos relevantes da DA e da DFT é fundamental para enfrentar essa realidade por meio da prevenção. Foi realizada uma revisão de diferentes variantes genéticas que causam a DA ou a DFT na América Latina. Pesquisamos os bancos de dados Medline e PubMed usando as palavras-chave “doença de Alzheimer”, “demência frontotemporal”, “mutação”, “América” e “América Latina”, além de países latino-americanos específicos. Quarenta e cinco itens foram escolhidos e analisados. As mutações do PSEN1 são a causa mais comum da doença de Alzheimer genética de início precoce (DAIP), seguida pelas mutações do PSEN2 e da APP. A DFT genética pode ser explicada principalmente por mutações no GRN, MAPT e expansões repetidas da C9orf72 G4C2. O APOE ε4 pode modificar a prevalência e a incidência da doença de Alzheimer de início tardio (DAIT), mas também o desempenho cognitivo em portadores afetados.

Neurodegenerative dementias have been described based on their phenotype, in relation to selective degeneration occurring in a particular neuroanatomical system. More recently however, the term proteinopathy has been introduced to describe diseases in which one or more altered proteins can be detected. Neurodegenerative diseases can be produced by more than one abnormal protein and each proteinopathy can determine different clinical phenotypes. Specific biomarkers have now been linked to certain molecular pathologies in live patients. In 2016, a new biomarker-based classification, currently only approved for research in Alzheimer’s disease, was introduced. It is based on the evaluation three biomarkers: amyloid (A) detected on amyloid-PET or amyloid- beta 42 assay in CSF; tau (T) measured in CSF as phosphorylated tau or on tau PET imaging; and neuronal injury/neurodegeneration (N), detected by total T-tau in CSF, FDG PET hypometabolism and on MRI brain scan. Results of clinical research using the ATN biomarkers at FLENI, a Neurological Institute in Buenos Aires, Argentina have, since 2011, contributed to ongoing efforts to move away from the concept of neurodegenerative dementias and more towards one of cognitive proteinopathies. Today, clinical diagnosis in dementia can only tell us “where” abnormal tissue is found but not “what” molecular mechanisms are involved. RESUMO. As demências neurodegenerativas foram descritas com base em seu fenótipo, em relação à degeneração seletiva que ocorre em um sistema neuroanatômico específico. Mais recentemente, no entanto, o termo proteinopatia foi introduzido para descrever doenças nas quais uma ou mais proteínas alteradas podem ser detectadas. As doenças neurodegenerativas podem ser produzidas por mais de uma proteína anormal e cada proteinopatia pode determinar diferentes fenótipos clínicos. Biomarcadores específicos já foram associados a certas patologias moleculares em pacientes vivos. Em 2016, uma nova classificação baseada em biomarcadores, atualmente aprovada apenas para pesquisas na doença de Alzheimer, foi introduzida. É baseado na avaliação de três biomarcadores: amiloide (A) detectado no ensaio amiloide-PET ou amiloide-beta 42 no LCR; tau (T) medida no LCR como tau fosforilada ou em imagem de tau PET; e lesão/neurodegeneração neuronal (N), detectada por T-tau total no LCR, hipometabolismo FDG PET e pela ressonância magnética. Os resultados de pesquisas clínicas usando os biomarcadores ATN no FLENI, um Instituto Neurológico de Buenos Aires, Argentina, desde 2011, contribuíram para os esforços contínuos para se afastar do conceito de demência neurodegenerativa e mover-se mais em direção às proteinopatias cognitivas. Hoje, o diagnóstico clínico da demência só pode nos dizer “onde” o tecido anormal é encontrado, mas não “quais” mecanismos moleculares estão envolvidos.

Pacientes com Transtorno Bipolar (TB) costumam apresentar déficits cognitivos ao envelhecer. No entanto, a correlação com síndromes demenciais é inconclusiva, apesar da similaridade com a variante comportamental da demência frontotemporal (bvFTD). Nós relatamos uma paciente de 78 anos de idade com TB tipo 1 desde a adolescência. Seus sintomas variavam de apatia a mania psicótica. A paciente passou por 3 internações, sendo a última há 10 anos, seguida de estabilização clínica. No entanto, nos últimos 2 anos, ela apresentou sintomas diferentes, como irritabilidade expressada por agressões verbal e física, comprometimento cognitivo, padrão repetitivo de comportamento, perambulação, delírios persecutórios, desorientação e hiporexia. O tratamento com anticolinesterásicos ou estabilizadores de humor não revelou melhora. Apresentou 17/30 pontos no miniexame do estado mental, a avaliação neuropsicológica sugeriu déficit de função executiva, atenção e memória. Os exames de neuroimagem demonstraram atrofia e hipoperfusão fronto-temporal. Abordagens diagnósticas e terapêuticas para este tipo de paciente representam um desafio significativo para os clínicos. Patients with Bipolar Disorder (BD) usually display cognitive deficits with aging. However, the correlation between BD and dementia syndromes is inconclusive, despite the similarity with behavioral variant frontotemporal dementia. We report a 78-year-old female patient who had bipolar type 1 disorder since adolescence. Her symptoms ranged from apathy to psychotic mania. She had had three hospitalizations, and since her last stay 10 years ago, her symptoms had remained stable. However, in the past 2 years, she displayed different symptoms, such as irritability manifested as verbal and physical aggression, cognitive impairment, repetitive pattern of behavior, perambulation, persecutory delusions, disorientation, and hyporexia. Treatment with anticholinesterases or mood stabilizers promoted no improvement. She scored 17/30 points on the Mini-Mental State Examination. Neuropsychological assessment suggested deficits in executive function, attention, and memory. Neuroimaging tests revealed frontotemporal degeneration and hypoperfusion. Diagnostic and therapeutic approaches for this type of patient represent a significant challenge for clinicians.

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 Aβ42, 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.

Patients with Bipolar Disorder (BD) usually display cognitive deficits with aging. However, the correlation between BD and dementia syndromes is inconclusive, despite the similarity with behavioral variant frontotemporal dementia. We report a 78-year-old female patient who had bipolar type 1 disorder since adolescence. Her symptoms ranged from apathy to psychotic mania. She had had three hospitalizations, and since her last stay 10 years ago, her symptoms had remained stable. However, in the past 2 years, she displayed different symptoms, such as irritability manifested as verbal and physical aggression, cognitive impairment, repetitive pattern of behavior, perambulation, persecutory delusions, disorientation, and hyporexia. Treatment with anticholinesterases or mood stabilizers promoted no improvement. She scored 17/30 points on the Mini-Mental State Examination. Neuropsychological assessment suggested deficits in executive function, attention, and memory. Neuroimaging tests revealed frontotemporal degeneration and hypoperfusion. Diagnostic and therapeutic approaches for this type of patient represent a significant challenge for clinicians. RESUMO Pacientes com Transtorno Bipolar (TB) costumam apresentardéficits cognitivosao envelhecer. No entanto, a correlação com síndromes demenciais é inconclusiva, apesar da similaridade com a variante comportamental da demência frontotemporal (bvFTD). Nósrelatamos uma paciente de 78 anos de idade com TB tipo 1 desde a adolescência. Seus sintomas variavam de apatia a mania psicótica. A paciente passou por 3 internações, sendo a última há 10 anos, seguida de estabilização clínica. No entanto, nos últimos 2 anos, ela apresentou sintomas diferentes, como irritabilidade expressada por agressões verbal e física, comprometimento cognitivo, padrão repetitivo de comportamento, perambulação, delírios persecutórios, desorientação e hiporexia. O tratamento com anticolinesterásicos ou estabilizadores de humor não revelou melhora. Apresentou 17/30 pontos no miniexame do estado mental, a avaliação neuropsicológica sugeriu déficit de função executiva, atenção e memória. Os exames de neuroimagem demonstraram atrofia e hipoperfusão fronto-temporal. Abordagens diagnósticas e terapêuticas para este tipo de paciente representam um desafio significativo para os clínicos.

Los trabajos incluidos en esta tesis van sobre el tema de la correlación clínico patológica en la enfermedad neurodegenerativa y más específicamente en la afasia primaria progresiva (APP). En el primer trabajo analizamos la proporción de positividad PET-amiloide en cada variante clínica para probar la hipótesis que clasificación según los criterios diagnósticos actuales (establecidos por consenso en 2011) de afasia primaria progresiva resultaría en grupos patológicamente homogéneos. Esta clasificación resulto ser altamente predictiva del estado del biomarcador de amiloide, en particular, la variante logopénica (vlAPP) se asoció a positividad de PET-amiloide en más del 95% de los casos. Además, todos los casos “amiloide discordantes (variante semántica [vsAPP] y variante no-fluente/agrammatica [vnfAPP])” con datos de autopsia disponible recibieron un diagnostico patológico primario de degeneración lobar frontotemporal (DLFT) con contribución secundaria de patología de enfermedad de Alzheimer (EA), sugiriendo que en casos de vsAPP y vnfAPP, un resultado PET-amiloide positivo puede ser más indicativo de patología mixta DLFT- EA que EA aislada. En el segundo trabajo identificamos rasgos clínicos y neuroanatómicos que pueden ayudar en la predicción en-vivo de patología subyacente en casos de vnfAPP que es la variante más heterogénea en los que respecta al diagnóstico patológico. VnfAPP-paralisis supranuclear progresiva se caracterizó por presentar con mayor disartria y predominio relativo de atrofia de sustancia blanca en la primera visita, y mayor velocidad de atrofia del tronco de encéfalo y aparición de signos clínicos de afectación del tronco de encéfalo en visitas posteriores. VnfAPP-degeneración corticobasal presento más déficit de comprensión de frases, de memoria de trabajo, y más atrofia de sustancia gris en la visita inicial, junto con progresión de atrofia en regiones corticales anteriores y mayor presencia de síntomas conductuales en visitas posteriores. En el tercer trabajo, cuantificamos y evaluamos la habilidad de diferentes medidas clínicas y neuroanatómicas para predecir qué casos de APP son presuntamente debidos a EA (usando PET-amiloide como marcador). Mediante un “data-driven” análisis, pudimos clasificar correctamente al 96% de los casos PET-amiloide negativos y 86% de los positivos. Las medidas de memoria visual y sintomatología conductual mostraron una capacidad predictiva similar a las medidas de lenguaje que más aportaron a la función discriminatoria, que fueron medidas del habla motora y repetición de frases, sugiriendo que medidas clínicas extra-lingüísticas tienen potencial para mejorar el diagnóstico en la APP. Finalmente, en el último trabajo también estudiamos la relación entre el depósito de amiloide (medido por PET-PiB) y la atrofia cerebral. Encontramos que en casos de afasia primaria progresiva generalmente asociados a EA (es decir, vlAPP), la atrofia cerebral era altamente asimétrica y predominante en áreas corticales de lenguaje mientras que la deposición de amiloide estaba distribuida difusamente y simétricamente a través de las áreas corticales de asociación de ambos hemisferios cerebrales, sugiriendo que otro factor está impulsando la progresión de atrofia cerebral., The studies included in this thesis addressed the issue of clinicopathologic correlation in neurodegenerative disease and more specifically in primary progressive aphasia (PPA). In the first study we analyzed rates of amyloid PET positivity to test the hypothesis that classification according to the recently established consensus PPA variant diagnostic criteria would result in groups with largely homogeneous amyloid biomarker profiles. We found that the current classification scheme was highly predictive of amyloid biomarker status with logopenic variant (lvPPA) being associated to amyloid positivity in more than 95% of cases. Furthermore, the amyloid biomarker discordant cases (amyloid positive semantic variant [svPPA] and non-fluent/agrammatic variant [nfvPPA]) that had available autopsy data received a primary pathologic diagnosis of frontotemporal lobar degeneration (FTLD) with presence of contributing Alzheimer’s disease (AD) pathology, suggesting that cases of amyloid biomarker positive svPPA and nvfPPA might be more indicative of mixed FTLD – AD pathology than primary AD. In the second study we identified clinical and neuroimaging features that may help predict underlying pathology in nfvPPA which is the most pathologically heterogeneous of the PPA clinical variants. Greater dysarthria and relative predominance of white-matter atrophy at presentation and greater rate of brainstem atrophy and appearance of brainstem clinical signs at follow-up were characteristic of underlying nfvPPA-progressive supranuclear palsy. NfvPPA-corticobasal degeneration showed more impairment in sentence comprehension, verbal working memory, and greater grey matter atrophy at presentation along with spread of atrophy to anterior cortical structures and greater presence of behavioral symptoms at follow-up. The third study quantified and evaluated the ability of different cognitive and neuroimaging measures to predict which primary progressive aphasia patients have presumptive Alzheimer’s disease pathology (using amyloid-PET as a surrogate marker). A data-driven analysis was able to correctly classify 96% amyloid negative and 86% amyloid positive cases. We found that measures of visual memory and behavioral impairment show similar ability to predict amyloid-PET status as the best performing language measures, which were motor speech and sentence repetition suggesting non-language measures hold potential value for improving differential diagnosis. Finally, the last study also investigated the relationship between amyloid deposition measured by PET-PiB imaging and brain atrophy. We found that, within lvPPA (which is generally due to AD), grey-matter volume loss was highly asymmetric and predominant in language regions whereas amyloid deposition was diffuse throughout association cortices and symmetric between hemispheres suggesting another factor different from amyloid deposition is driving progression of brain atrophy.

The aim of this case study was to perform a cross-sectional analysis of spontaneous speech of a patient with Frontotemporal Dementia (FTD). For this purpose, four speech and language therapy episodes, from 2012 to 2014, were selected, transcribed and analyzed in light of Enunciative-Discursive Neurolinguistics. The analysis showed, as the patient's FTD status progressed, that he used different semiotic strategies, e.g., use of repetition and gesture during speech production. It also highlighted the importance of the interlocutor's role of prompting the patient to express verbal meaning. Thus, it can be concluded that the recognition of the strategies used by the patient in favor of his role as a speaker, during interactions, is what enables and legitimates his role.

The occurrence of dementia in amyotrophic lateral sclerosis (ALS) was only widely recognized in the late 20th century. Hitherto, it was believed that dementia was a rare event due to the fortuitous association with other diseases. In 1924, Kostantin Nikolaevich Tretiakoff and Moacyr de Freitas Amorim reported a case of dementia with features of frontotemporal dementia (FTD) that preceded the motor signs of ALS. Neuropathological examination confirmed ALS and found no signs of other dementia-causing diseases. The authors hypothesized that dementia was part of ALS and recommended the search for signs of involvement of motor neurons in cases of dementia with an ill-defined clinical picture, a practice currently accepted in the investigation of cases of FTD. This was one of the first descriptions of dementia preceding the motor impairments of ALS and was published in Portuguese and French in Memórias do Hospício de Juquery. RESUMO A ocorrência de demência na esclerose lateral amiotrófica (ELA) somente foi amplamente reconhecida no final do século 20. Antes disso, acreditava-se que demência era ocorrência rara que se devia à associação fortuita de outra doença. Em 1924, Kostantin Nikolaevich Tretiakoff e Moacyr de Freitas Amorim relataram caso de demência com as características de demência frontotemporal (DFT) que precedeu os sinais motores da ELA. O exame neuropatológico confirmou ELA e não encontrou sinais de outras doenças que cursam com demência. Os autores do artigo aventaram que a demência fazia parte da ELA e recomendaram a investigação da presença de sinais de comprometimento dos neurônios motores em dementes com alterações cognitivas e comportamentais mal definidas, conduta atualmente aceita na investigação de casos de DFT. Esta foi uma das primeiras descrições de demência precedendo a síndrome motora da ELA e foi publicada em português e em francês em Memórias do Hospício de Juquery.

Frontotemporal dementia (FTD) is one of the most common causes of early-onset dementia with primary progressive aphasia (PPA) being the second-most-frequent form of this degenerative disease. Despite the similarity with progressive dementia (especially in early stages of Alzheimer´s disease), three types of PPA can be differentiated: semantic, agrammatic and logopenic (subtype discussed in this study). To date, no medications have been shown to improve or stabilize cognitive deficits in patients with PPA. We report the case of a 62-year-old woman with difficulty naming objects and planning. An interdisciplinary evaluation, including imaging and lab exams, together with neuropsychological and personality assessments, confirmed that the patient had logopenic PPA on the basis of repetition difficulty, phonemic and semantic paraphasias and absence of agrammatism. The timing of the assessment in this case, along with the resources available and commitment of an integrated interdisciplinary team, allowed a differential diagnosis (from other classical dementias) to be reached. RESUMO A demência frontotemporal (DFT) é uma das causas mais comuns de demências precoces e a Afasia Progressiva Primária (APP) é o segundo tipo mais prevalente desta patologia neurodegenerativa. Apesar das semelhanças entre as demências, especialmente considerando os estágios iniciais da demência de Alzheimer, é possível diferenciar três subtipos de APP: semântico, agramático/não-fluente e logopênico, que é o subtipo discutido no presente estudo. Até o momento não há estudos que tenham evidenciado melhora ou estabilização dos prejuízos cognitivos nos pacientes diagnosticados com APP por meio de controle medicamentoso. No presente estudo descreve-se o caso de uma idosa com 62 anos de idade que apresentou dificuldade para nomear objetos e fazer planejamentos. Após uma avaliação interdisciplinar, incluindo exames de neuroimagem e laboratoriais, associados a avaliações neuropsicológica e de personalidade, foi possível realizar o diagnóstico de APP subtipo logopênico, especialmente por causa da dificuldade de repetição, parafasias semântica e fonêmica e ausência de agramatismo. O momento da avaliação foi propício para realizar um diagnóstico diferencial de outras demências, contando também com os recursos disponíveis e o comprometimento de uma equipe interdisciplinar integrada.