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Behavior Symptoms in Primary Progressive Aphasia Variants

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Neuropsychiatric Symptoms of Cognitive Impairment and Dementia

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

Abstract

Primary progressive aphasia (PPA) is a clinical syndrome characterized by an isolated and progressive deterioration of language abilities, in the context of otherwise preserved cognition and activities of daily living. Prominent behavior changes at symptom onset represent exclusionary criteria for PPA. However, in the last years, it has been acknowledged that early, mild behavior changes may accompany or precede language deficits in this condition. The frequency of these neuropsychiatric symptoms across the three variants of PPA is an area still being explored. Here we present a revision on this topic and additional research results conducted in our PPA clinical series. Overall, studies indicate a high frequency of behavior changes in PPA (above 80 %), with depression, apathy, anxiety, agitation, irritability, as well as abnormal appetite, lack of insight, and disinhibition being frequently endorsed by these patients. The semantic variant is characterized by decreased empathy, disinhibition-like behaviors, changes in appetite/food preferences, and obsessive-compulsive behaviors. Negative behaviors, such as apathy, are more commonly associated with the nonfluent and logopenic variants, being significantly more frequent in the former. Delusions and hallucinations have also been reported in association with logopenic variant. These differences are likely to reflect distinct underlying neurodegenerative diseases, with implications for disease management.

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Acknowledgments

The authors thank the patients for their participation and also acknowledge the facilities provided by Memoclínica. The authors thank Professor Isabel Pavão Martins, head of the Laboratory of Language Research, as well as speech therapists of this department involved in the language assessments and in SLT of some patients (Luísa Farrajota, Gabriela Leal, and José Fonseca). The authors also thank Sara Madeira and Telma Pereira from INESC-ID and Instituto Superior Técnico (IST) for their contribution to the discussion on new sophisticated data analysis applied to PPA clinical datasets. C. M. was supported by Fundação para a Ciência e Tecnologia (FCT) PhD Fellowship (SFRH/BD/75710/2011). A. d. M. and M. G. also receive funding from FCT.

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Authors and Affiliations

  1. Faculty of Medicine, Laboratory of Language Research, Department of Neurology, University of Lisbon, Av. Prof Egas Moniz, 1649-028, Lisboa, Portugal

    Carolina Maruta

  2. Faculty of Medicine, Laboratory of Neurosciences, Institute of Molecular Medicine, University of Lisbon, Av. Prof Egas Moniz, 1649-028, Lisboa, Portugal

    Carolina Maruta, Alexandre de Mendonça & Manuela Guerreiro

  3. Psychology and Health Research Unit, Instituto Superior de Psicologia Aplicada, Rua Jardim do Tabaco, n°34, Lisboa, Portugal

    João Maroco

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Correspondence to Carolina Maruta .

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  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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Maruta, C., Maroco, J., de Mendonça, A., Guerreiro, M. (2017). Behavior Symptoms in Primary Progressive Aphasia Variants. 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_3

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