Spanish version of the frontotemporal dementia knowledge scale: adaptation and validation / Versión en español de la escala de conocimiento sobre demencia frontotemporal: adaptación y validación

ABSTRACT 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.

Correlato Entre Hallazgos Neurológicos Y Sintomatología Neuropsiquiátrica En La Demencia Frontotemporal De Variante Conductual: Revisión Sistemática / Correlation Between Neurological Findings And Neuropsychiatric Symptoms In Behavioral Variant Frontotemporal Dementia: Systematic Review

Resumen Introducción. Dentro de las Demencias Frontotemporales (DFT), la variante conductual (DFTvc) es la de mayor prevalencia, estando asociada a una marcada alteración a nivel de comportamiento y regulación emocional. Objetivo. Describir el correlato neuroanatómico en sujetos con DFTvc y las características clínicas neuropsiquiátricas descritas en ellos. Metodología. Se ha realizado una revisión sistemática de artículos publicados entre 2013 y 2018, en relación a la DFTvc, en bases de datos en inglés y español que cumplieran con los criterios de inclusión definidos. Resultados. La DFTvc se asocia a una hipofunción en las zonas de la corteza prefrontal, corteza cingulada, entre otros. La apatía y desinhibición son la principal sintomatología de estudio. Conclusiones. Existe una falta de artículos actualizados que describan las características neuropsiquiátricas junto a su descripción imagenológica en esta población que favorezcan el desarrollo de avances médicos y no médicos de abordaje.

Introduction. Within Frontotemporal Dementia (FTD), Behavioral variant (BvFTD) is the most prevalent, is associated with a marked alteration in behavior and emotional regulation. Objective. Describes the neuroanatomical correlate in subjects with BvFTD and the neuropsychiatric clinical characteristics described in them. Methodology. A systematic review of articles published between 2013 and 2018 has been carried out, in relation to the BvFTD, in databases in english and spanish that meet the inclusion criteria. Results. The BvFTD is associated with a hypofunction in the areas of the prefrontal cortex, cingulate cortex and others. Apathy and disinhibition are the main symptomatology of study. Conclusions. There is a lack of updated articles that describe neuropsychiatric characteristics along with their imaging description in this population that favors the development of medical and non-medical approaches.

Olfactory impairment in frontotemporal dementia: A systematic review and meta-analysis / Alterações olfatórias em Demência Frontotemporal: Revisão sistemática e meta-análise

ABSTRACT. Frontotemporal dementia (FTD) presents clinically in three variants: one behavioral and two with progressive primary aphasia - non-fluent/agrammatic and semantic. Defined by the degenerative process and cerebral atrophy, olfactory dysfunction occurs in up to 96% of previous FTD case series. Objective: the present study aims to critically synthesize data about the relationship between FTD and olfactory impairment to analyze the usefulness of olfactory evaluation tests as a complementary element in early diagnosis. Methods: a database search was performed using the keywords "olfactory OR smell OR olfaction AND frontotemporal dementia". We included studies that evaluated olfactory function in patients diagnosed with frontotemporal dementia, all subtypes, compared with age-matched healthy controls. For comparative purposes, the effect size was calculated using Cohen's D. The studies selected were categorized according to dementia variant and olfactory test type. A meta-analysis was performed using forest plots - homogeneity was evaluated by statistical tests (i2 and Cochran Q). Results: ten articles met the inclusion criteria. Heterogeneity was classified as low for semantic dementia olfactory identification and behavioral variant olfactory discrimination groups (i2 = 0 and 3.4%, respectively) and as moderate for the behavioral variant olfactory identification group (i2 = 32.6%). Conclusion: patients with the frontotemporal dementia behavioral variant seem to present with alterations in odor identification, but with preserved discrimination. Scent identification also seems to be impaired in semantic dementia. Therefore, we conclude that olfactory evaluation in these patients is possibly impacted by cognitive alterations and not by sensory deficits. Application of olfactory tests may prove important in differentiating prodromal states from other types of dementia with more pronounced olfactory impairment.

RESUMO. A demência frontotemporal apresenta-se clinicamente em três variantes: uma comportamental e duas com afasia progressiva primária - não fluente/agramática e semântica. Definida pelo processo degenerativo e atrofia cerebral, apresenta uma prevalência de disfunção olfatória de até 96% em séries anteriores. Objetivo: o presente estudo objetiva sintetizar criticamente dados sobre a relação entre DFT e o comprometimento olfatório para analisar a utilidade dos testes de avaliação olfatória como elemento complementar no diagnóstico precoce. Métodos: uma pesquisa de banco de dados foi realizada usando as palavras-chave "olfactory OR smell OR olfaction AND frontotemporal dementia". Foram incluídos estudos que avaliaram a função olfatória em pacientes com diagnóstico de demência frontotemporal, todos os subtipos, em comparação com controles saudáveis ​​pareados por idade. Para fins de comparação, o tamanho do efeito foi calculado usando D de Cohen. Os estudos selecionados foram separados por variante de demência e tipo de teste olfativo. Uma meta-análise foi realizada utilizando gráficos floresta - sua homogeneidade foi avaliada por testes estatísticos (i2 e Cochran Q). Resultados: dez artigos preencheram os critérios de inclusão. A heterogeneidade foi classificada como baixa para os grupos de identificação olfatória em demência semântica e discriminação olfatória em variante comportamental (i2 = 0 e 3.4%, respectivamente) e moderada para identificação olfatória no grupo de variante comportamental (i2 = 32.6%). Conclusão: pacientes com variante comportamental de demência frontotemporal parecem apresentar alterações na identificação de odores, com discriminação preservada. A identificação de odores parece estar prejudicada, também, na demência semântica. Desta forma, concluímos que a avaliação olfatória nesses pacientes é possivelmente impactada por alterações cognitivas e não por déficits sensoriais propriamente. A aplicação de testes olfatórios pode ser importante na diferenciação de estados prodrômicos de outros tipos de demência com comprometimento olfatório mais pronunciado.

Neural structural imaging in the assessment of cognitive impairment / 医学研究生学报

As our country steps into the aging society gradually, the number of cognitive impairments and the prevalence rate are increasing yearly. The family and society bear a heavy burden. It is more important to explore the more direct and Objective morphological changes of cognitive impairment through neural structural imaging , which is better for early diagnosis, intervention and delay or even prevent its progress. Here we present a review of this topic focusing on neural structural imaging in the assessment of cognitive impairment.

An Autopsy Confirmed Case of Semantic Variant Primary Progressive Aphasia with Frontotemporal Lobar Degeneration-TDP type C

A 62-year-old man presented with a one-year history of word finding difficulty, impaired single word comprehension and personality changes including aggression, apathy and eating change. Brain MRIs showed severe atrophy in the left anterior temporal lobe. The clinical syndromic diagnosis was semantic variant primary progressive aphasia. He died at age 70 of pneumonia. At autopsy, transactive response DNA-binding protein (TDP) immunoreactive long dystrophic neurites were predominantly found in the cerebral cortices, which were compatible with frontotemporal lobar degeneration-TDP type C pathology.

Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy

BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type). METHODS: In total, 94 MRI scans (74 scans from bvFTD and 20 scans from age-matched normal controls) were classified into 35 D- and 39 F-type bvFTD cases based on an axial MRI visual rating scale. We compared baseline clinical characteristics, progression in motor and cognitive symptoms, and survival times between D- and F-types. Survival analyses were performed for 62 of the 74 patients. RESULTS: While D-type performed better on neuropsychological tests than F-type at baseline, D-type had higher baseline scores on the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Evaluations of motor progression showed that the disease duration with motor symptoms was shorter in D-type than F-type. Moreover, the survival time was shorter in D-type (6.9 years) than F-type (9.4 years). Cox regression analyses revealed that a high UPDRS Part III score at baseline contributed to an increased risk of mortality, regardless of the pattern of atrophy. CONCLUSIONS: The prognosis is worse for D-type than for those with F-type. Shorter survival in D-type may be associated with the earlier appearance of motor symptoms.

Clinical, neuroimaging and genetic profiles of amyotrophic lateral sclerosis with frontotemporal lobe degeneration / 中华神经科杂志

Objective To describe the clinical, neuroimaging and genetic profiles of amyotrophic lateral sclerosis with frontotemporal lobe degeneration ( ALS-FTLD).Methods From August 2011 to May 2015, patients with FTLD or other types of neurodegenerative dementia were physically examined in detail and electromyography was performed to those with suspected dysarthria, limb atrophy or weakness.Cognitive and behavioral screenings were performed to all ALS patients.Patients with ALS-FTLD entered further analysis of neuroimaging and genetics.Results Among the 8 patients diagnosed as ALS-FTLD, 4 patients began with personality change or amnesia, while diseases in the remaining 4 cases began with limb weakness or dysarthria.Dementia type of 7 cases was behavioral variant FTLD ( bvFTD) and 1 case was diagnosed as semantic dementia.Electromyography of all the 8 patients showed diffuse neurogenic changes.Constructional neuroimaging of 6 patients showed cerebral atrophy predominantly in frontal and temporal lobes.Fluorodeoxyglucose-positron emission tomography was conducted in 5 patients, indicating hypometabolism mainly in frontal and ( or) temporal lobes.NeuroQ analysis revealed that bilateral frontal lobes were the most hypometabolic areas for ALS-FTLD.Among 4 patients who underwent genetic screening, 1 patient was C9ORF72 mutation carrier.Conclusions bvFTD is the major type of dementia in the context of ALS.Metabolic neuroimaging could assist accurate diagnosis, and it reveals that bilateral frontal lobes are the most hypometabolic areas for ALS-FTLD.C9ORF72 gene mutation is an important pathogenic mutation for ALS-FTLD, although it is rare in Chinese population.

The Genetics of Monogenic Frontotemporal Dementia / Genética da Demência Frontotemporal Monogênica

ABSTRACTAround 10-15% of patients diagnosed with frontotemporal dementia (FTD) have a positive family history for FTD with an autosomal dominant pattern of inheritance. Since the identification of mutations in MAPT(microtubuleassociated protein tau gene) in 1998, over 10 other genes have been associated with FTD spectrum disorders, discussed in this review. Along with MAPT, mutations in GRN(progranulin) and C9orf72(chromosome 9 open reading frame 72) are the most commonly identified in FTD cohorts. The association of FTD and motor neuron disease (MND) can be caused by mutations in C9orf72and other genes, such as TARDBP(TAR DNA-binding protein), FUS(fused in sarcoma), UBQLN2(ubiquilin 2). Multisystem proteinopathy is a complex phenotype that includes FTD, Paget disease of the bone, inclusion body myopathy and MND, and can be due to mutations in VCP(valosing containing protein) and other recently identified genes.

RESUMOCerca de 10-15% dos pacientes diagnosticados com demência frontotemporal (FDFT) têm uma história familiar positiva para DFT com um padrão de herança autossômico dominante. Desde a identificação de mutações em MAPT(proteína tau associada a microtúbulos), em 1998, mais de 10 outros genes já foram associados a doenças do espectro da DFT, que são discutidas nesta revisão. Junto com MAPT, mutações em GRN(progranulina) e C9orf72( chromosome 9 open reading frame 72) são as mais comumente identificadas em casuísticas de DFT. A associação de DFT com doença do neurónio motor (DNM) pode ser causada por mutações em C9orf72e outros genes, tais como TARDBP( TAR DNA-binding protein), FUS( fused in sarcoma), UBQLN2(ubiquilina 2) e outros genes. Proteinopatia multissistêmica é um fenótipo complexo que inclui DFT, doença de Paget óssea, miopatia com corpúsculos de inclusão e DNM, e pode ser devida a mutações em VCP( valosin containing protein) e outros genes recentemente identificados.

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

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

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

Avaliação fonoaudiológica da deglutição na demência frontotemporal / Phonoaudiological swallowing evaluation in frontotemporal dementia

Introdução: A deglutição e suas características principais ainda são desconhecidas na demência frontotemporal. Objetivos: Caracterizar a deglutição e o comportamento alimentar de pacientes com diagnóstico de demência frontotemporal que apresentam a variante comportamental (DFTvc) e a afasia progressiva primária (APP). Caracterizar os pacientes com DFT e seus cuidadores. Descrever aspectos cognitivos e comportamentais, funcionalidade global, comunicação funcional, e a funcionalidade da deglutição na DFT. Descrever os problemas de deglutição e do comportamento alimentar na DFTvc e APP. Correlacionar os aspectos cognitivos e comportamentais, funcionalidade global e a comunicação com as características da deglutição. Identificar fatores preditivos da piora da funcionalidade da deglutição e do comportamento alimentar na DFT. Avaliar o comportamento dos instrumentos empregados. Desenvolver a versão reduzida do Questionário de Habilidades de Alimentação e Deglutição nas Demências e do Questionário de Comunicação Funcional na Afasia. Método: Este estudo incluiu 46 indivíduos com DFT nas fases leve, moderada e grave, e seus 46 cuidadores. O Mini exame do estado mental (MEEM) e o Mini exame do estado mental grave (MEEM-g) foram usados para avaliar os aspectos cognitivos. A Escala de estadiamento da demência (CDR-DLFT) foi usada para confirmar a fase da doença. O Inventário Neuropsiquiátrico (INP) foi aplicado para investigar os problemas comportamentais. A Bateria de Avaliação Frontal (BAF) investigou as funções executivas. O Índice das Atividades de Vida Diária (Katz), Questionário para Avaliação da Comunicação Funcional na Afasia (QACFA) e a Escala de funcionalidade da deglutição (EFD) avaliaram as habilidades funcionais. O Questionário de Habilidades de Alimentação e Deglutição nas Demências (QHADD) avaliou as dificuldades na deglutição e alimentação. Resultados: Os grupos DFTvc e APP não mostraram diferença estatisticamente significante no MEEM,...

Introduction: Swallowing and its main characteristics are still unknown in frontotemporal dementia. Objectives: To characterize swallowing and feeding behavior of patients with frontotemporal dementia who have behavioral variant (bvFTD) and primary progressive aphasia (PPA). To characterize patients with FTD and their caregivers.To describe cognitive and behavioral aspects, functionalstatus, functional communication, and swallowing function in FTD.To describe swallowing problems and feeding behavior in bvFTD and PPA. To correlate cognitive and behavioral aspects, functional status, and communication with swallowing. To identify predictive factors associated with worsening of functionality of swallowing and feeding behavior in FTD. To evaluate the instruments used. To develop reduced versions of: "Assessment of Feeding and Swallowing Difficulties in Dementia" and "Functional Outcome Questionnaire Aphasia". Method: This study included 46 individuals with FTD in mild, moderate and severe phases, and their 46 caregivers. The Mini mental state examination (MMSE) and the Severe Mini mental state examination (SMMSE) were used to assess the cognitive aspects. The FTLD-modified Clinical Dementia Rating scale (FTLD-CDR) was used to confirm the stage of the disease. The Neuropsychiatric Inventory (NPI) was applied to investigate the behavioral problems. The Frontal Assessment Battery (FAB) investigated executive functions. The Index of Activities of Daily Living (Katz), Functional Outcome Questionnaire- Aphasia and Swallowing rating scale (SRE) evaluated the functional abilities. The Assessment of Feeding and Swallowing Difficulties in Dementia (QHADD) evaluated the difficulties in swallowing and feeding. Results: bvFTD and PPA groups showed no statistically significant difference in MMSE, CDR and FAB. Caregivers of patients with bvFTD had more hours of care per day compared to patients with PPA (p < 0.05). The groups differed in SRE (p < 0.05). The behavioral...

Non-literal language and semantic dementia / Linguagem não literal e demência semântica

Semantic dementia is characterized by fluent, phonologically adequate speech with various anomias and semantic paraphasias. Performance on semantic tasks is well documented in these patients, although little is known regarding performance on more complex language tasks, such as those involving non-literal language (interpretation of metaphors and proverbs and recognition of irony). OBJECTIVE: To report the investigation of non-literal language in cases of semantic dementia. METHODS: Two cases of semantic dementia were investigated for the presence of deficits in non-literal language abilities using the screening test for Alzheimer's disease with proverbs, metaphor test and irony test. RESULTS: Both patients were found to have low performance on the tests applied, particularly for interpretation of proverbs. CONCLUSION: This poor performance was attributed largely to the characteristic semantic changes of the disease, but some frontal symptoms inherent to other forms of frontotemporal lobar degeneration were also observed which interfered in the testing, such as negativism, reduced attention span, concretism and perseverations.

A demência semântica é caracterizada por fala fluente e adequada fonologicamente e com diversas anomias e parafasias semânticas. O desempenho em tarefas semânticas é bem documentado nestes pacientes, porém pouco se sabe acerca do desempenho em tarefas linguísticas mais complexas, como naquelas que envolvem linguagem não literal (interpretação de metáforas e provérbios e reconhecimento de ironias). OBJETIVO: Relatar a investigação da linguagem não-literal em casos de demência semântica. MÉTODOS: Dois casos de demência semântica foram investigados para a presença de déficits em habilidades de linguagem não-literal, usando o teste de triagem para a doença de Alzheimer com provérbios, teste de metáforas e teste de ironia.RESULTADOS: Verificou-se que ambas as pacientes apresentaram baixo desempenho nos testes aplicados, principalmente na interpretação de provérbios. CONCLUSÃO: O baixo desempenho foi atribuído especialmente às alterações semânticas características da doença, porém também foram observados sintomas frontais característicos de outras formas de degeneração lobar frontotemporal que interferiram na testagem como negativismo, redução do fôlego atencional, concretismo e perseverações.

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

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

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

Prevalence of frontotemporal dementia in community-based studies in Latin America: a systematic review / Prevalência de demência frontotemporal em estudos de base comunitária na America Latina: uma revisão sistemática

Latin America (LA) is experiencing a rise in the elderly population and a consequent increase in geriatric problemssuch as dementia. There are few epidemiological studies assessing the magnitude of dementia and dementia subtypesin LA. Objective: To identify published community-based studies on the prevalence of FTD in LA countries. Methods: Adatabase search for door-to-door studies on FTD prevalence in LA was performed. The search was carried out on Medline,Embase, and LILACS databases for research conducted between 1994 and 2012. The main inclusion criteria were: use ofany internationally accepted diagnostic criteria and investigation of community samples. Results: Four hundred and ninetytwo articles were found, of which 26 were initially pre-selected by title or abstract review. Five studies from 3 differentcountries were included. The FTD prevalence rates in community-dwelling elderly were 1.2 (Venezuela), 1.3 (Peru) and1.7-1.8 (Brazil) per thousand persons, depending on age group. Conclusion: The FTD prevalence in LA studies showedvalues mid-way between those observed in western and in oriental populations. Despite the magnitude of this problem,epidemiological information on FTD remains scarce in LA.

A América Latina (AL) está experimentando um aumento na população de idosos e um consequente aumento nosproblemas geriátricos, como demência. Existem poucos estudos epidemiológicos avaliando a magnitude de demência edemência subtipos na AL Objetivo: Identificar publicações baseadas em estudos sobre a prevalência da FTD em países daAL. Métodos: A pesquisa realizada foi por estudos de prevalência de FTD em comunidade na AL. A pesquisa foi realizada emMed-line, Embase, e LILACS no período entre 1994 e 2012. Os principais critérios de inclusão foram: utilização de quaisquercritérios internacionalmente aceitos de diagnóstico e investigação de amostras em comunidade. Resultados: Quatrocentose noventa e dois artigos foram encontrados, dos quais 26 foram inicialmente pré-selecionados pelo título ou fiscalização doabstract. Cinco estudos de 3 países diferentes foram incluídos. As taxas de prevalência na comunidade em idosos com FTDeram 1,2 (Venezuela), 1,3 (Peru) e 1,7-1,8 (Brasil) por mil pessoas, dependendo da faixa etária. Conclusão: A prevalênciaFTD em estudos da AL, apresentaram valores intermediários entre os observados em populações ocidentais e orientais. Apesarda magnitude do problema, informações epidemiológicas sobre FTD permanecem escassas em AL.

Neuropathology of frontotemporal lobar degeneration: a review / Neuropatologia da degeneração lobar frontotemporal: uma revisão

Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Three main clinical variants are widely recognized within the FTLD spectrum: the behavioural variant of frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD represents a highly heterogeneous group of neurodegenerative disorders which are best classified according to the main protein component of pathological neuronal and glial inclusions. The most common pathological class of FTLD is associated with the TDP-43 protein (FTLD-TDP), while FTLD-Tau is considered slightly less common while the FTLD-FUS (Fused in sarcoma protein) pathology is rare. In this review, these three major pathological types of FTLD are discussed.

A degeneração lobar frontotemporal (DLFT) é a segunda principal causa de demência pré-senil. Sob o diagnóstido de DLFT, há três principais diagnósticos clínicos: demência frontotemporal variante comportamental (DFTvc), demência semântica (DS) e a afasia progressiva não Fluente (APNF). A DLFT representa um grupo heterogêneo de desordens degenerativas que são classificadas de acordo com o componente proteico patológico das inclusões neuronais e gliais. A classe patológica mais comum das DLFT é associada com a proteína TDP-43 (DLFT-TDP), seguida pela DLFT-Tau, enquanto a DLFT-FUS é rara. Nesta revisão, nós iremos discutir os três principais subtipos patológicos da DLFT.

Frontotemporal dementia and neurocysticercosis: a case report / Demência frontotemporal e neurocisticercose: um relato de caso

We report a case of a 67-year-old woman with frontotemporal dementia (FTD) and a history of neurocysticercosis. After her retirement she showed progressive behavioral changes and neuropsychiatric symptoms with relative preservation ofcognitive functioning. During the next three years, the patient manifested progressive deterioration of verbal communication gradually evolving to mutism, a hall mark of cases of progressive nonfluent aphasia.

Caso de uma mulher de 71 anos com demência frontotemporal e historia de neurocisticercose. Após sua aposentadoria ela apresentou progressivas mudanças comportamentais e sintomas neuropsiquiátricos, com relativa preservação do funcionamento cognitivo. Durante os seguintes três anos, a paciente foi desenvolvendo uma deteriorização progressiva da comunicação verbal evoluindo gradualmente a mutismo, marca que descreve um caso de afasia progressiva não fluente.

Pharmacological treatment of frontotemporal lobar degeneration: systematic review / Tratamento farmacológico da degeneração lobar frontotemporal: revisão sistemática

OBJECTIVE: To identify the therapeutic options available for treatment of cognitive and behavioral symptoms in frontotemporal lobar degeneration. METHOD: Systematic review using the descriptors "frontotemporal lobar degeneration" OR "frontotemporal dementia" OR "fronto-temporal dementia" OR "fronto-temporal degeneration" OR "Pick's disease" OR "Pick's atrophy" OR "semantic dementia" OR "progressive aphasia" AND "pharmacotherapy" OR "treatment" OR "efficacy" OR "effects" OR "management" was performed in the Medline and Lilacs databases. Selection criteria: Quality A - randomized clinical trials. Quality B - open studies or reports of six or more cases. Quality C - reports of five or fewer cases. Two reviewers independently assessed the clinical studies. Information collected included diagnostic criteria used, sample size, duration, efficacy and tolerability measures used and results obtained. RESULTS: From the 532 studies found, 29 complied with the inclusion criteria. All studies worked with a small sample, had short duration of treatment and used non-uniform measures in evaluating efficacy and tolerability. Studies showed disparate results with respect to behavior and cognition. CONCLUSION: There is still little, and poor, evidence available for treatment of frontotemporal lobar degeneration and studies with better methodological background are needed.

OBJETIVO: Identificar as opções terapêuticas disponíveis para tratamento dos sintomas cognitivos e comportamentais da degeneração lobar frontotemporal. MÉTODO: Revisão sistemática utilizando os descritores "frontotemporal lobar degeneration OR frontotemporal dementia OR fronto-temporal dementia OR fronto-temporal degeneration OR Pick's disease OR Pick's atrophy OR semantic dementia OR progressive aphasia AND pharmacotherapy OR treatment OR efficacy OR effects OR management" nas bases Medline e Lilacs. Critérios de seleção: Qualidade A - Estudos clínicos randomizados. Qualidade B - Estudos abertos ou relatos de seis ou mais casos. Qualidade C - Relatos de cinco ou menos casos. Dois revisores avaliaram independentemente os estudos clínicos. As informações coletadas incluíram critérios de diagnóstico utilizados, número da amostra, duração, medidas de eficácia e tolerabilidade utilizadas e os resultados obtidos. RESULTADOS: Encontraram-se 532 estudos e 29 preenchiam os critérios. Todos os estudos incluíam uma amostra pequena, com curta duração de tratamento, com utilização de medidas não uniformes na avaliação da eficácia e da tolerabilidade. O comportamento e a cognição apresentaram resultados díspares entre os estudos. CONCLUSÃO: São poucas as evidências disponíveis para tratamento da degeneração lobar frontotemporal e de qualidade insatisfatória, sendo necessários estudos com maior rigor metodológico.

The Pathogenetic Role of TAR DNA Binding Protein (TDP-43) in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

The recent identification of the transactive response DNA binding protein with a molecular weight of 43 kDa (TDP-43) as the major pathological protein, in both amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin positive inclusions (FTLD-U), provides the new insight into understanding disease processes. The pathogenesis of both diseases is unclear, although they are related by having some overlap of symptoms and now by the shared histopathology of TDP-43 deposition. The number of degenerative diseases associated with TDP-43 has increased, leading to the new designation "TDP-43 proteinopathy". TDP-43 is a highly conserved protein ubiquitously expressed in many tissues including the central nervous system where it is present in neuronal and glial nuclei and to a lesser extent in the cytoplasm. Currently, TDP-43 has been implicated in regulating gene transcription and alternative splicing, in addition to maintaining mRNA stability. However, we still need to investigate the effects of posttranslational modifications of TDP-43, including phosphorylation, ubiquitination, and cleavage, on its regulation of various cellular processes. We review recently published studies of TDP-43 and its relationship to human disease with a special focus on ALS and FTLD-U. We conclude that the TDP-43 proteinopathies represent a novel class of neurodegenerative disorders and both ALS and FTLD-U are closely related conditions linked to similar mechanism of neurodegeneration.

Fronotemporal lobar degeneration: An update / 第二军医大学学报

Frontotemporal lobar degeneration (FTLD) is a non-Alzheimer dementia syndrome characterized by focal atrophy of the frontal and/or temporal lobes. Recently, it has been found that FTLD is related to the degeneration of tau protein, and is closely associated with corticobasal ganglionic degeneration, progressive supranuclear palsy, and motor neuron disease. This article reviews the progress in etiology, genetics, pathology, clinical features, and diagnostic criteria of FTLD.

Frontotemporal Lobar Degeneration

Frontotemporal lobar degeneration (FTLD) is a progressive dementia with prominent neuropsychiatric features, aphasia or both. FTLD predominantly affects the frontal and anterior part of temporal cortex. FTLD is classified into frontotemporal dementia (FTD), progressive nonfluent aphasia (PA), and semantic dementia (SD). FTLD is estimated to account for 20% of cases of degenerative dementia with presenile onset. This disease typically has onset in the mid- or early fifties. FTD is characterized by behavioral change and executive dysfunction, PA features a progressive nonfluent aphasia. SD is characterized by a progressive semantic aphasia and associative agnosia. Structural imaging shows atrophy of the frontal lobe and the anterior portion of the temporal lobe, bilaterally symmetric or asymmetric. Pathologically, FTLD can be classified into tau-positive pathology, tau-negative, ubiquitin positive pathology, dementia lacking distinctive histology. At present, there are no specific pharmacological therapies approved for use in any of the FTLD syndrome.

Temporal Variant of Frontotemporal Dementia: A Case of Semantic Dementia

Semantic dementia is a rare, distinct form of frontotemporal lobar degeneration, characterized by a deficit in semantic memory with relative preservation of attention and executive functions. We report a case of semantic dementia that pre-sented with a 3-year history of progressive word-finding difficulty and prosopagnosia. Brain MRI showed prominent atrophic changes in the left temporal region and neuropsychological tests demonstrated semantic memory deficits.