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Research paper
Impaired affective and cognitive theory of mind and behavioural change in amyotrophic lateral sclerosis
  1. Egberdina-Józefa van der Hulst1,
  2. Thomas H Bak1,2,3,4,
  3. Sharon Abrahams1,2,3,4
  1. 1Human Cognitive Neuroscience—Psychology, University of Edinburgh, Edinburgh, UK
  2. 2Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh, UK
  3. 3Centre for Cognitive Aging and Epidemiology, University of Edinburgh, Edinburgh, UK
  4. 4Anne Rowling Regenerative Neurology Clinic, University of Edinburgh, Edinburgh, UK
  1. Correspondence to Professor Sharon Abrahams, 7 George Square, Edinburgh, EH8 9JZ, UK; s.abrahams{at}


Objectives Executive and behavioural changes are well-recognised in classical amyotrophic lateral sclerosis (ALS), indicating a subclinical behavioural-variant frontotemporal dementia (bvFTD) in some patients. Social cognitive deficits in ALS have been recently described and an impairment was identified on a simple Theory of Mind (ToM) test, which assesses the judgement of the preference of another through direction of eye gaze. The present study further delineated this deficit, by distinguishing between Affective and Cognitive subcomponents, and determining the relationship to behavioural change, levels of empathy and self-awareness.

Methods The Cognitive–Affective Judgement of Preference Test was administered to 33 patients with ALS and 26 controls. Furthermore, a comprehensive neuropsychological battery and detailed behavioural assessment, with measures of empathy and awareness, were included.

Results Patients with ALS showed a significant impairment in Affective ToM only when compared with healthy controls, with a deficit in 36% of patients; 12% showed an isolated Affective ToM deficit while 24% showed more generic ToM dysfunction. A Cognitive ToM deficit was found in 27% of patients, with 3% showing an isolated Cognitive ToM deficit. The patients with ALS showed reduced empathy (Fantasy scale) and increased behavioural dysfunction with high levels of apathy. In addition, patients with either an Affective and/or Cognitive ToM deficit exhibited poor self-awareness of their performance and abnormalities on verbal fluency, while those with an Affective ToM deficit also displayed higher levels of apathy and a naming deficit.

Conclusions Dysfunctional ToM is a prominent feature of the cognitive profile of ALS. This specific difficulty in identifying and distinguishing the feelings and thoughts of another from a self-perspective may underpin the social behavioural abnormalities present in some patients with ALS, manifest as apathy and loss of awareness.

  • ALS

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