Degradation of emotion processing ability in corticobasal syndrome and Alzheimer's disease

Brain. 2014 Nov;137(Pt 11):3061-72. doi: 10.1093/brain/awu246. Epub 2014 Sep 16.


Disturbed emotion processing and difficulty with social interactions are present to variable degrees in dementia. They are characteristic features of frontotemporal dementia, whereas these deficits tend to be mild in Alzheimer's disease, reflecting the different patterns of neurodegeneration seen in these disorders. Corticobasal syndrome is an atypical parkinsonian disorder clinically and pathologically related to frontotemporal dementia. Corticobasal syndrome typically presents as a motor disturbance, although cognitive and behavioural changes are now recognized. Pathological changes are found in frontoparietal cortical regions and in the basal ganglia; regions that are heavily involved in emotion processing. Despite the overlap with frontotemporal dementia and the observed regions of brain atrophy, emotion processing has not been systematically explored in corticobasal syndrome. This study aimed to (i) comprehensively examine emotion processing in corticobasal syndrome in comparison to Alzheimer's disease, to determine whether emotion processing deficits exist in this syndrome, beyond those seen in Alzheimer's disease; and (ii) identify the neural correlates underlying emotion processing in corticobasal syndrome and Alzheimer's disease. Sixteen patients with corticobasal syndrome, 18 patients with Alzheimer's disease and 22 matched healthy control subjects were assessed on a comprehensive battery of face and emotion processing tasks. Behavioural analyses revealed deficits in both basic face processing and high-level emotion processing tasks in patients with corticobasal syndrome. Notably, the emotion processing disturbance persisted even after controlling for face processing deficits. In contrast, patients with Alzheimer's disease were impaired on high-level complex and cognitively demanding emotion recognition tasks (Ekman 60, The Awareness of Social Inference Test) only. Neuroimaging analyses using FreeSurfer revealed that emotion processing deficits in corticobasal syndrome were associated with basal ganglia volume loss as well as cortical thinning of the left paracentral gyrus/precuneus region. In Alzheimer's disease, however, emotion processing deficits were associated with atrophy in a different set of brain regions, including the right cingulate and the bilateral insulae, as well as the hippocampi, right amygdala and nucleus accumbens bilaterally. Our results demonstrate that patients with corticobasal syndrome experience widespread deficits in emotion processing, and these deficits are related to changes in brain regions known to be crucial for emotion processing. These findings have important clinical implications for the treatment and management of these patients.

Keywords: basal ganglia; dementia; face processing; striatum.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Alzheimer Disease / pathology
  • Alzheimer Disease / physiopathology*
  • Amygdala / pathology
  • Amygdala / physiopathology
  • Atrophy / pathology
  • Basal Ganglia Diseases / pathology
  • Basal Ganglia Diseases / physiopathology*
  • Cerebral Cortex / pathology
  • Cerebral Cortex / physiopathology*
  • Emotions / physiology*
  • Facial Expression*
  • Female
  • Humans
  • Limbic System / pathology
  • Limbic System / physiopathology*
  • Male
  • Middle Aged
  • Neurodegenerative Diseases / pathology
  • Neurodegenerative Diseases / physiopathology*
  • Social Perception
  • Syndrome