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Dementia & Neuropsychologia

Print version ISSN 1980-5764

Dement. neuropsychol. vol.7 no.1 São Paulo Jan./Mar. 2013

http://dx.doi.org/10.1590/S1980-57642013DN70100003 

VIEWS & REVIEWS

Behavioural-variant frontotemporal dementia: An update

Demência frontotemporal-variante comportamental: uma revisão

Olivier Piguet1 

John R. Hodges1 

1Neuroscience Research Australia, Barker St, Randwick NSW 2031, Australia. School of Medical Sciences, the University of New South Wales, Sydney, Australia. ARC Centre of Excellence in Cognition and its Disorders, the University of New South Wales, Sydney, Australia.

ABSTRACT

Behavioural-variant frontotemporal dementia (bvFTD) is characterised by insidious changes in personality and interpersonal conduct that reflect progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation and decision making. The underlying pathology is heterogeneous and classified according to the presence of intraneuronal inclusions of tau, TDP-43 or occasionally FUS. Biomarkers to detect these histopathological changes in life are increasingly important with the development of disease-modifying drugs. Gene mutations have been found which collectively account for around 10-20% of cases including a novel hexanucleotide repeat on chromosome 9 (C9orf72). The recently reviewed International Consensus Criteria for bvFTD propose three levels of diagnostic certainly: possible, probable and definite. Detailed history taking from family members to elicit behavioural features underpins the diagnostic process with support from neuropsychological testing designed to detect impairment in decision-making, emotion processing and social cognition. Brain imaging is important for increasing the level of diagnosis certainty. Carer education and support remain of paramount importance.

Key words: frontotemporal dementia; genetics; cognition; social cognition; neuroimaging

RESUMO

A demência frontotemporal-variante comportamental (DFTvc) é caracterizada por mudanças insidiosas de personalidade e conduta interpessoal, que refletem a desintegração progressiva de circuitos neurais envolvidos em cognição social, regulação emocional, motivação e tomada de decisão. O substrato patológico é heterogêneo e classificado de acordo com a presença de inclusões intraneuronais de proteína tau, TDP-43 ou, ocasionalmente, de FUS. Biomarcadores capazes de detectar estas alterações histopatológicas durante a vida vêm ganhando importância com o desenvolvimento de drogas específicas modificadoras da doença. Algumas mutações genéticas já foram encontradas, sendo em conjunto responsáveis por 10-20% dos casos, incluindo a recentemente descrita repetição de hexanucleotídeo no cromossomo 9 (C9orf72). A versão revisada dos Critérios Internacionais do Consenso em DFTvc propõe três níveis de certeza diagnóstica: possível, provável e definida. História clínica detalhada obtida com familiares, para identificar as alterações de comportamento características, auxilia no diagnóstico, juntamente com o apoio de avaliação neuropsicológica dirigida à detecção de comprometimento em tarefas de tomada de decisão, processamento emocional e cognição social. A neuroimagem é importante para aumentar o grau de certeza diagnóstica. Educação e suporte dos cuidadores continuam sendo medidas de extrema relevância.

Palavras-chave: demência frontotemporal; genética; cognição; cognição social; neuroimagem

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Received: December 27, 2012; Accepted: February 28, 2013

John Hodges. Neuroscience Research Australia. Barker St,Randwick NSW 2031, Australia. E-mail: j.hodges@neura.edu.au

Disclosure: The authors report no conflicts of interest.

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