Language, executive function and social cognition in the diagnosis of frontotemporal dementia syndromes

Abstract

Frontotemporal dementia (FTD) represents a spectrum of non-Alzheimer’s degenerative conditions associated with focal atrophy of the frontal and/or temporal lobes. Frontal and temporal regions of the brain have been shown to be strongly involved in executive function, social cognition and language processing and, thus, deficits in these domains are frequently seen in patients with FTD or may even be hallmarks of a specific FTD subtype ( i.e., relatively selective and progressive language impairment in primary progressive aphasia). In this review, we have attempted to delineate how language, executive function, and social cognition may contribute to the diagnosis of FTD syndromes, namely the behavioral variant FTD as well as the language variants of FTD including the three subtypes of primary progressive aphasia (PPA): non-fluent/agrammatic, semantic, and logopenic. This review also addresses the extent to which deficits in these cognitive areas contribute to the differential diagnosis of FTD versus AD. Finally, early clinical determinants of pathology are briefly discussed and contemporary challenges to the diagnosis of FTD are presented.

Impulsivity, decreased social exploration, and executive dysfunction in a mouse model of frontotemporal dementia

“…Increased aggression could also be explained by the higher arousal in male Tg mice as observed by the touch escape and Preyer reflex. In later stages of the disease, a decline in executive function is often seen in FTD patients (Eslinger et al, 2011;Harciarek and Cosentino, 2013;Huey et al, 2009;Johns et al, 2009;Seltman and Matthews, 2012;Shea et al, 2014). To evaluate executive function, the animals were first subjected to the Y-maze test, which depends on their intrinsic motivation to explore a novel environment.…”

“…BvFTD presents with changes in social behavior and conduct, such as loss of social awareness and social withdrawal, restlessness and poor impulse control leading to compulsive behaviors including stereotyped hair-pulling and skin picking (Eslinger, Moore, Anderson, & Grossman, 2011;Lindau et al 2000;Mendez & Perryman, 2002;Pressman & Miller, 2014;Snowden et al, 2001Snowden et al, , 2003. At later stages, FTD patients develop deficits in executive function: they have problems planning, coordinating and executing simple tasks (Harciarek and Cosentino, 2013;Huey et al, 2009;Johns et al, 2009;Moy et al, 2004;Stopford et al, 2012). In addition to the characteristic behavioral changes, the clinical features of FTD can be complicated by neurological signs, such as motor neuron signs, parkinsonism, and gait disturbances, with some patients developing motor problems resulting from motor neuron pathology (Devenney et al, 2015;Merrilees et al, 2010).…”

Early Cognitive/Social Deficits and Late Motor Phenotype in Conditional Wild-Type TDP-43 Transgenic Mice

“…Post-weaning 1 month induction of the Tg caused early deficits in social behavior, a characteristic early symptom of FTD patients (Shinagawa et al, 2006; Harciarek and Cosentino, 2013). A battery of cognitive tasks revealed early alterations in object recognition and working memory tests, while aversive memory was spared.…”

Social cognition in Alzheimer's disease: A separate construct contributing to dependence

“…Even in cases that fit the typical AD profile with amnestic deficits being prominent, individuals have been shown to demonstrate impairment on objective social cognitive tests including Theory of Mind (ToM) and emotion recognition (Freedman, Binns, Black, Murphy, & Stuss, 2012; Miller et al, 2012), and in some cases these impairments can be commensurate with those in FTD. See (Harciarek & Cosentino, in press) for a review.…”