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Key emerging issues in frontotemporal dementia

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Abstract

Frontotemporal dementia (FTD) encompasses the syndromes of behavioural variant FTD (bvFTD) and primary progressive aphasia (PPA) and refers to those neurodegenerative diseases characterised by predominant pathological involvement of the frontal and temporal lobes. Recent years have witnessed major advances in the clinical characterisation of FTD, reflected in the publication of updated diagnostic criteria for bvFTD and PPA, and the discovery of new pathogenic mutations has added to the understanding of genotype–phenotype interactions and of disease mechanisms. Emerging results from longitudinal studies of familial FTD show that imaging and cognitive changes occur years before symptom onset and such studies may yield biomarkers of early disease that in turn will facilitate earlier diagnosis. The hope and (guarded) expectation is that these advances may together herald the beginning of the end of the chapter in which FTD is considered an inexorably progressive and untreatable condition.

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Acknowledgments

Dr Chan is funded by the Cambridge NIHR Biomedical Research Centre and receives grant income from the UK Medical Research Council, Technology Strategy Board and the Cambridge Isaac Newton Trust.

The images shown in Fig. 1 are acquired by Dr Kuven Moodley, Brighton and Sussex Medical School, on the integrated PET–MRI scanner at the Institute of Nuclear Medicine, University College London, UK.

Table 1 is reproduced with permission of Oxford University Press. Table 2 is reproduced with permission of Wolters Kluwer Health Inc. Table 3 is reproduced with permission of Elsevier.

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Authors and Affiliations

  1. Department of Medicine for the Elderly, Addenbrooke’s Hospital, Hills Road, Cambridge, CB2 0QQ, UK

    Sarah A. A. Hopkins

  2. Department of Clinical Neurosciences, Herchel Smith Building for Brain and Mind Sciences, University of Cambridge, Forvie Site, Robinson Way, Cambridge, CB2 0SZ, UK

    Dennis Chan

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Correspondence to Dennis Chan.

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Hopkins, S.A.A., Chan, D. Key emerging issues in frontotemporal dementia. J Neurol 263, 407–413 (2016). https://doi.org/10.1007/s00415-015-7880-7

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