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Frontotemporal Dementia

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Neuropsychiatric Symptoms of Cognitive Impairment and Dementia

Part of the book series: Neuropsychiatric Symptoms of Neurological Disease ((NSND))

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Abstract

Frontotemporal lobar degeneration (FTLD) includes the behavioral variant of frontotemporal lobar degeneration (bvFTD), also called frontotemporal dementia (FTD), and two language variants, namely, semantic dementia (SD) and primary progressive agrammatic aphasia (PPA). There is a large degree of overlap between the three FTLD variants, as well as between FTLD and motor neurone disease or parkinsonism. Neuropsychiatric features, the earliest and prominent manifestations of bvFTD, are also commonly encountered in the language variants. FTLD is a pathologically heterogeneous entity, divided into several subtypes characterized by protein aggregates of different nature and distribution. The discovery of mutations on different genes made the classification of FTLD even more complex because of weak genotype-phenotype correlations, even within the same family. And a same phenotype can be caused by different mutations. Since neuropsychiatric symptoms are inaugural, bvFTD is often misdiagnosed as psychiatric conditions such as depression, bipolar disorder, obsessive-compulsive disorder, or schizophreniform psychosis, although atypical features are usually present. The main behavioral symptoms are early behavioral disinhibition; apathy or inertia; loss of sympathy or empathy; perseverative, stereotyped, or ritualistic behavior; and hyperorality and dietary changes. Psychosis is less frequent, except in some familial cases, raising the possibility of a link between FTD, schizophrenia, and bipolar disorders. There is, as yet, no specific pathophysiological treatment for FTLD. As for symptomatic treatments, few randomized placebo-controlled studies have been performed. However, serotonergic agents are recommended to treat FTD behavioral disorders.

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Authors and Affiliations

  1. Department of Neurology, Universitaire de Lille, CHU Lille, Memory Clinic, Inserm U1171, Labex DISTALZ, F-59000, Lille, France

    Florence Pasquier, Thibaud Lebouvier & Florence Lebert

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  1. Florence Pasquier
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  2. Thibaud Lebouvier
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  3. Florence Lebert
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Correspondence to Florence Pasquier .

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Editors and Affiliations

  1. Department of Neurosciences and Mental Health, Centro Hospitalar Lisboa Norte-Hospital de Santa Maria, Instituto de Medicina Molecular (IMM) and Instituto de Saúde Ambiental (ISAMB), Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal

    Ana Verdelho

  2. CEDOC, Chronic Diseases Research Center, Nova Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal

    Manuel Gonçalves-Pereira

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Pasquier, F., Lebouvier, T., Lebert, F. (2017). Frontotemporal Dementia. In: Verdelho, A., Gonçalves-Pereira, M. (eds) Neuropsychiatric Symptoms of Cognitive Impairment and Dementia. Neuropsychiatric Symptoms of Neurological Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-39138-0_13

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  • DOI: https://doi.org/10.1007/978-3-319-39138-0_13

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