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Neuroimage Clin. 2017 Aug 26;16:447-454. doi: 10.1016/j.nicl.2017.08.020. eCollection 2017.

Emotion detection deficits and changes in personality traits linked to loss of white matter integrity in primary progressive aphasia.

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Division of Neurology, Krembil Neuroscience Centre, Toronto Western Hospital, University Health Network, 399 Bathurst St., Toronto, ON M5T2S8, Canada.
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, 60 Leonard Avenue, Toronto, ON M5T2S8, Canada.
Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina, 58, 20132 Milan, Italy.
Memory and Aging Center, Department of Neurology, University of California San Francisco, 1500 Owens Suite 320, San Francisco, CA 94158, USA.
Department of Speech Language and Hearing Sciences, University of Arizona, 1131 E. 2nd Street, Tucson, AZ 85721, USA.
Department of Psychology, University of Southern California, 3620 South McClintock Ave., Los Angeles, CA 90089, USA.


Non-cognitive features including personality changes are increasingly recognized in the three PPA variants (semantic-svPPA, non fluent-nfvPPA, and logopenic-lvPPA). However, differences in emotion processing among the PPA variants and its association with white matter tracts are unknown. We compared emotion detection across the three PPA variants and healthy controls (HC), and related them to white matter tract integrity and cortical degeneration. Personality traits in the PPA group were also examined in relation to white matter tracts. Thirty-three patients with svPPA, nfvPPA, lvPPA, and 32 HC underwent neuropsychological assessment, emotion evaluation task (EET), and MRI scan. Patients' study partners were interviewed on the Clinical Dementia Rating Scale (CDR) and completed an interpersonal traits assessment, the Interpersonal Adjective Scale (IAS). Diffusion tensor imaging of uncinate fasciculus (UF), superior longitudinal fasciculus (SLF) and inferior longitudinal fasciculus (ILF), and voxel-based morphometry to derive gray matter volumes for orbitofrontal cortex (OFC), anterior temporal lobe (ATL) regions were performed. In addition, gray matter volumes of white matter tract-associated regions were also calculated: inferior frontal gyrus (IFG), posterior temporal lobe (PTL), inferior parietal lobe (IPL) and occipital lobe (OL). ANCOVA was used to compare EET performance. Partial correlation and multivariate linear regression were conducted to examine association between EET and neuroanatomical regions affected in PPA. All three variants of PPA performed significantly worse than HC on EET, and the svPPA group was least accurate at recognizing emotions. Performance on EET was related to the right UF, SLF, and ILF integrity. Regression analysis revealed EET performance primarily relates to the right UF integrity. The IAS subdomain, cold-hearted, was also associated with right UF integrity. Disease-specific emotion recognition and personality changes occur in the three PPA variants and are likely associated with disease-specific neuroanatomical changes. Loss of white matter integrity contributes as significantly as focal atrophy in behavioral changes in PPA.


Diffusion tensor imaging; Emotion detection; Personality; Primary progressive aphasia; Social cognition

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