Individuals with different subtypes of primary progressive aphasia (PPA), a common clinical presentation of frontotemporal dementia characterized by language disturbances, often show overlapping...Show moreIndividuals with different subtypes of primary progressive aphasia (PPA), a common clinical presentation of frontotemporal dementia characterized by language disturbances, often show overlapping clinical symptoms, such that accurate diagnosis can be difficult. Prior studies indicate that social cognitive abilities, in particular emotion recognition, may improve the diagnostic process. Therefore, this study investigated emotion recognition deficits across different emotions and emotional intensities by means of the Emotion Recognition Task (ERT) among patients with a PPA subtype (semantic (svPPA), non-fluent (nfvPPA) or logopenic (lvPPA)), and behavioural frontotemporal dementia (bvFTD). The ERT is a neuropsychological test consisting of dynamic, naturalistic videos of the six basic facial emotional expressions (anger, disgust, fear, happiness, sadness and surprise) across different intensity levels (40, 60, 80 and 100%). In total, emotion recognition abilities were assessed in 152 participants (10 svPPA, 20 nfvPPA, 6 lvPPA, 63 bvFTD, 53 controls). Results of an ANCOVA controlling for age, sex, and education level showed emotion recognition deficits in patients with a PPA subtype and bvFTD compared to controls. Among the patient groups, patients with bvFTD were poorer in overall emotion recognition than patients with nfvPPA. Among the PPA subtypes emotion recognition did not differ. Patients with svPPA were worse in recognizing anger than patients with bvFTD, nfvPPA and lvPPA. In addition, patients with svPPA and bvFTD were worse in recognizing disgust than patients with nfvPPA. Besides, patients with svPPA and controls were better in recognizing happiness, than patients with bvFTD, nfvPPA and lvPPA. Results of a three-way mixed ANOVA showed that emotion recognition abilities improved with higher emotional intensity and group differences were not the largest at the lowest intensity. In conclusion, our findings demonstrate emotion recognition deficits in PPA and bvFTD, and hint towards specific impairments for recognizing anger and disgust in patients with svPPA. These insights can help to improve clinical distinction among the PPA subtypes and bvFTD, which could ultimately lead to better differential diagnosis.Show less
Frontotemporal dementia (FTD) is a neurodegenerative disorder with a heterogeneous profile. It is a highly heritable disorder with an autosomal dominant pattern of inheritance. The three most...Show moreFrontotemporal dementia (FTD) is a neurodegenerative disorder with a heterogeneous profile. It is a highly heritable disorder with an autosomal dominant pattern of inheritance. The three most common mutations are found in the microtubule-associated protein tau (MAPT) gene, progranulin (GRN) gene and hexanucleotide repeat expansion in the chromosome 9 open reading frame 72 (C9orf72) gene. Sensitive outcome measures in early stage genetic FTD are necessary for upcoming disease-modifying therapeutic trials. It is known that social cognition declines in genetic mutation carriers of FTD. The Dutch version of the Social Norms Questionnaire (SNQ-NL) measures the degree to which subjects understand and identify accepted social norms and is able to differentiate FTD patients from healthy controls. The aim of this study was to identify if the SNQ-NL can already indicate social cognitive changes in the presymptomatic phase of FTD. We examined group differences between patients with FTD (n = 34), presymptomatic mutation carriers (n = 55), prodromal mutation carriers (n = 20), and control participants (n = 51) in Total score, Break errors and Overadhere errors of the SNQ-NL, associations with other cognitive functions, and longitudinal data in a subset were further explored. Results showed that the SNQ-NL Total Score and Overadhere errors differed between patients and the other participant groups, but not between presymptomatic or prodromal mutation carriers and control participants. Differences between patients and the other participant groups were also found for the SNQ-NL Direct/indirect ratio. The SNQ-NL Total score and Overadhere scores significantly correlated with cognitive flexibility, theory of mind and confrontation naming. None of the SNQ-NL variables correlated significantly with emotion recognition. No interaction effect is found between groups over time for one of the SNQ-NL variables. In conclusion, present results showed that the SNQ-NL can differentiate between FTD patients, presymptomatic and prodromal mutation carriers. No evidence is found for the SNQ-NL as an indicator of social cognitive changes in the presymptomatic phase of genetic FTD.Show less
