Cognitive and Behavioral Profiles of Left and Right Semantic Dementia: Differential Diagnosis with Behavioral Variant Frontotemporal Dementia and Alzheimer’s Disease

Article type: Research Article

Authors: Pozueta, Ana^{a; *} | Lage, Carmen^a | García-Martínez, María^a | Kazimierczak, Martha^a | Bravo, María^a | López-García, Sara^a | Riancho, Javier^b | González-Suarez, Andrea^a | Vázquez-Higuera, José Luis^a | de Arcocha-Torres, María^c | Banzo, Ignacio^c | Jiménez-Bonilla, Julio^c | Berciano, José^a | Rodríguez-Rodríguez, Eloy^a | Sánchez-Juan, Pascual^a

Affiliations: [a] Neurology Service and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), ‘Marqués de Valdecilla’ University Hospital, University of Cantabria, Institute for Research ‘Marqués de Valdecilla’ (IDIVAL), Santander, Spain | [b] Neurology Service and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Sierrallana, Institute for Research ‘Marqués de Valdecilla’ (IDIVAL), Santander, Spain | [c] Department of Nuclear Medicine, University Hospital ‘Marqués de Valdecilla’, University of Cantabria, Molecular imaging Group - IDIVAL, Santander, Spain

Correspondence: [*] Correspondence to: Ana Pozueta Cantudo, PsyS, Servicio de Neurología Hospital Universitario ‘Marqués de Valdecilla’, Planta Baja Valdecilla Sur, Avda Marqués de Valdecilla s/n, 39011 Santander, Cantabria, Spain. Tel.: +34 942202520 (EXT 73427); E-mail: anapozueta@gmail.com.

Abstract: Background:Semantic dementia (SD) is a subtype of frontotemporal dementia (FTD) characterized by semantic memory loss and preserved abilities of other cognitive functions. The clinical manifestations of SD require a differential diagnosis with Alzheimer’s disease (AD), especially those with early onset, and behavioral variant FTD (bvFTD). Objective:The present study aimed to compare cognitive performances and neuropsychiatric symptoms in a population of AD, bvFTD, and left and right SD defined with the support of molecular imaging (amyloid and 2-[18F] fluoro-2-deoxy-D-glucose positron emission tomography) and assessed the accuracy of different neuropsychological markers in distinguishing these neurodegenerative diseases. Methods:Eighty-seven participants (32 AD, 20 bvFTD, and 35 SD (17 Left-SD and 18 Right-SD) completed a comprehensive neuropsychological battery that included memory, language, attention and executive functions, visuospatial function, visuoconstructional skills, and tasks designed specifically to evaluate prosopagnosia and facial emotions recognition. The Neuropsychiatric Inventory was administered to assess neuropsychiatric symptoms. Results:An episodic memory test that included semantic cues, a visuospatial test (both impaired in AD), a naming test and a prosopagnosia task (both impaired in SD) were the four most valuable cognitive metrics for the differential diagnosis between groups. Several behavioral abnormalities were differentially present, of which aggression, self-care (both more frequent in bvFTD), and eating habits, specifically overeating and altered dietary preference (more frequent in SD), were the most valuable in group discrimination. Conclusion:Our study highlights the value of a comprehensive neuropsychological and neuropsychiatric evaluation for the differential diagnosis between FTD syndromes and AD.

Keywords: Alzheimer’s disease, behavior, behavioral variant frontotemporal dementia, cognition, semantic dementia

DOI: 10.3233/JAD-190877

Journal: Journal of Alzheimer's Disease, vol. 72, no. 4, pp. 1129-1144, 2019