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Nat Rev Neurol. 2017 Jul;13(7):406-419. doi: 10.1038/nrneurol.2017.75. Epub 2017 Jun 16.

Imaging and fluid biomarkers in frontotemporal dementia.

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Department of Neurology, Erasmus Medical Center, 's Gravendijkwal 230, 3015 CE Rotterdam, Netherlands.
Department of Clinical Genetics, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, Netherlands.
Dementia Research Centre, Department of Neurodegenerative diseases, Institute of Neurology, Queen Square, University College London, London WC1N 3BG, UK.
Department of Clinical Genetics, VU University Medical Center, De Boelelaan 1118, 1081 HZ Amsterdam, Netherlands.


Frontotemporal dementia (FTD), the second most common type of presenile dementia, is a heterogeneous neurodegenerative disease characterized by progressive behavioural and/or language problems, and includes a range of clinical, genetic and pathological subtypes. The diagnostic process is hampered by this heterogeneity, and correct diagnosis is becoming increasingly important to enable future clinical trials of disease-modifying treatments. Reliable biomarkers will enable us to better discriminate between FTD and other forms of dementia and to predict disease progression in the clinical setting. Given that different underlying pathologies probably require specific pharmacological interventions, robust biomarkers are essential for the selection of patients with specific FTD subtypes. This Review emphasizes the increasing availability and potential applications of structural and functional imaging biomarkers, and cerebrospinal fluid and blood fluid biomarkers in sporadic and genetic FTD. The relevance of new MRI modalities - such as voxel-based morphometry, diffusion tensor imaging and arterial spin labelling - in the early stages of FTD is discussed, together with the ability of these modalities to classify FTD subtypes. We highlight promising new fluid biomarkers for staging and monitoring of FTD, and underline the importance of large, multicentre studies of individuals with presymptomatic FTD. Harmonization in the collection and analysis of data across different centres is crucial for the implementation of new biomarkers in clinical practice, and will become a great challenge in the next few years.


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