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Nuclear Imaging in Frontotemporal Dementia

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Abstract

Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative syndrome, predominantly affecting the frontal and temporal lobes. Most patients present with behavioural deficits, executive dysfunction and language difficulties. FTD presents as two clinically recognized subtypes: the behavioural manifestation (FTD-b) and primary progressive aphasia (PPA), which can be divided into semantic dementia (SD) and progressive nonfluent aphasia (PNFA). FTD is second to Alzheimer’s disease (AD) as the major cause of young-onset dementia. Neuropathological characteristics of FTD roughly can be divided in tauopathy (FTD-TAU) and ubiquitin pathology (FTD-U). Almost half of FTD occurs familial, and genetic heterogeneity is reflected by the identification of mutations in causative genes. Diagnostic criteria have modest sensitivity, and it may be challenging to differentiate FTD from other types of dementia, especially AD. Functional imaging, especially FDG-PET, improves early diagnosis, and frontotemporal hypometabolism correlates with clinical symptoms. Besides functional markers, nuclear imaging techniques may be helpful to detect specific markers of pathology or deficits of different neurotransmitter systems, depending on degeneration of subcortical nuclei, and may provide valuable insight in the pathophysiology of FTD. Although currently no effective treatment is available for FTD, early and correct diagnosis is necessary for adequate clinical management, because of prognostic implications and for genetic counselling.

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Authors and Affiliations

  1. Department of Neurology, Alzheimer Research Center, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen, 9713 GZ, The Netherlands

    Fransje E. Reesink & Peter Paul De Deyn MD, PhD

  2. Department of Nuclear Medicine and Molecular Imaging, Alzheimer Research Center, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen, 9713 GZ, The Netherlands

    Gilles N. Stormezand

  3. Department of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, Hanzeplein 1, Groningen, 9713GZ, The Netherlands

    Rudi A. J. O. Dierckx

  4. Department of Radiology and Nuclear Medicine, Ghent University, De Pintelaan 185 K12, Ghent, 9000, Belgium

    Rudi A. J. O. Dierckx

  5. Laboratory of Neurochemistry and Behavior, Department of Biomedical Sciences, Institute Born-Bunge, University of Antwerp, Born Bunge Foundation, Antwerp (Wilrijk), Belgium

    Peter Paul De Deyn MD, PhD

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  1. Fransje E. Reesink
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  4. Peter Paul De Deyn MD, PhD
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Correspondence to Fransje E. Reesink .

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Editors and Affiliations

  1. Dept. of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands

    Rudi A.J.O. Dierckx

  2. Faculty of Electrical Engineering and Information Technology, University of Applied Sciences Offenburg, Offenburg, Germany

    Andreas Otte

  3. Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands

    Erik F.J. de Vries

  4. Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, Groningen, The Netherlands

    Aren van Waarde

  5. Neurology, University Medical Center Groningen, Groningen, The Netherlands

    Klaus L. Leenders

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Reesink, F.E., Stormezand, G.N., Dierckx, R.A.J.O., De Deyn, P.P. (2014). Nuclear Imaging in Frontotemporal Dementia. In: Dierckx, R., Otte, A., de Vries, E., van Waarde, A., Leenders, K. (eds) PET and SPECT in Neurology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54307-4_15

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