Dementia with Lewy bodies (DLB) is the second commonest cause of neurodegenerative dementia in older people. It is part of the range of clinical presentations that share a neuritic pathology based on abnormal aggregation of the synaptic protein alpha-synuclein. DLB has many of the clinical and pathological characteristics of the dementia that occurs during the course of Parkinson's disease. Here we review the current state of scientific knowledge on DLB. Accurate identification of patients is important because they have specific symptoms, impairments, and functional disabilities that differ from those of other common types of dementia. Severe neuroleptic sensitivity reactions are associated with significantly increased morbidity and mortality. Treatment with cholinesterase inhibitors is well tolerated by most patients and substantially improves cognitive and neuropsychiatric symptoms. Clear guidance on the management of DLB is urgently needed. Virtually unrecognised 20 years ago, DLB could within this decade be one of the most treatable neurodegenerative disorders of late life.

Objectives: To present the methods and baseline characteristics of the Pietà study, a population-based survey investigating successful brain aging in the oldest-old. Method:The study was conducted in Caeté (MG), Brazil. In 2007, 1,251 individuals aged 75+ years were living in the city and were invited to participate. Participants responded to a general health questionnaire and were submitted to clinical, neurological, cognitive, psychiatric and functional evaluations. A subgroup was submitted to neuropsychological testing, blood tests and magnetic resonance of the skull. Individuals were classified as having cognitive impairment-no dementia, dementia, parkinsonism, psychiatric disorders or successful brain aging. Results: We evaluated 639 individuals (51.1% of the target population; 64% women), aged 81.4±5.2 years and with 2.7±2.6 years of schooling. Almost 30% of the elderly were illiterates and 82.1% belonged to middle/middle-low socioeconomic levels. Almost 50% were widows, but only 14.3% were living alone. Conclusion: The Pietà cohort is representative of the oldest-old Brazilian population. We believe the results of the study may contribute to increase our knowledge about healthy and pathological brain aging in the oldest-old. Key words: aging, epidemiology, brain, cognitive impairment, dementia, depression.Estudo Pietà: investigação epidemiológica sobre envelhecimento cerebral bem sucedido em Caeté (MG), Brasil. Métodos e características de base da coorte RESUMO Objetivos: Apresentar os métodos e as características sociodemográficas do projeto Pietà, estudo de base populacional que investiga o envelhecimento cerebral bem sucedido em uma coorte de idosos muito idosos. Método: O estudo foi conduzido em Caeté (MG). Em 2007, 1.251 indivíduos com 75+ anos residiam no município e foram convidados a participar. Os idosos responderam a um questionário de saúde geral e foram submetidos à avaliação clínica, neurológica, cognitiva, psiquiátrica e funcional. Um subgrupo realizou avaliação neuropsicológica, exames laboratoriais e ressonância magnética de crânio. Os participantes foram classificados em grupos com comprometimento cognitivo-não demência, demência, parkinsonismo, transtornos psiquiátricos ou envelhecimento cerebral bem sucedido. Resultados: Foram avaliados 639 idosos (51,1% da população-alvo; 64% mulheres), com idade 81,4±5,2 anos e escolaridade 2,7±2,6 anos. Quase 30%

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Background Mutations in GRN (progranulin) and MAPT (microtubule-associated protein tau) are among the most frequent causes of monogenic Frontotemporal Dementia (FTD), but data on the frequency of those mutations in regions such as Latin America is still lacking. Objective We aimed to investigate the frequencies of GRN and MAPT mutations in FTD cohorts from two Brazilian dementia research centers, from University of Sao Paulo (USP) and Federal University of Minas Gerais (UFMG) medical schools. Methods We included 76 probands diagnosed with behavioral variant FTD (n=55), semantic variant Primary Progressive Aphasia (PPA) (n=11), or nonfluent variant PPA (n=10). Twenty-five percent of the cohort had at least one relative affected with FTD. Results Mutations in GRN were identified in seven probands, and in MAPT, in two probands. We identified three novel GRN mutations (p.Q130X, p.317Afs*12, and p.K259Afs*23), in patients diagnosed with nfvPPA or bvFTD. Plasma progranulin levels were measured and a cutoff value of 70ng/ml was found with 100% sensitivity and specificity to detect null GRN mutations. Conclusions The frequency of GRN mutations was 9.6% and of MAPT mutations, was 7.1%. Among familial cases of FTD, the frequency of GRN mutations was 31.5%, and of MAPT mutations was 10.5%.

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