Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of the motor system with recognised extra-motor and cognitive involvement. This cross-sectional study examined ALS patients’ performance on measures requiring social inference, and determined the relationship between such changes and variations in mood, behaviour, personality, empathy and executive function. Fifty-five ALS patients and 49 healthy controls were compared on tasks measuring social cognition and executive function. ALS patients also completed measures examining mood, behaviour and personality. Regression analyses explored the contribution of executive function, mood, behaviour and personality to social cognition scores within the ALS sample. A between-group MANOVA revealed that, the ALS group was impaired relative to controls on two composite scores for social cognition and executive function. Patients also performed worse on individual tests of executive function measuring cognitive flexibility, response inhibition and concept formation, and on individual aspects of social cognition assessing the attribution of emotional and mental states. Regression analyses indicated that ALS-related executive dysfunction was the main predictor of social cognition performance, above and beyond demographic variables, behaviour, mood and personality. On at least some aspects of social cognition, impaired performance in ALS appears to be secondary to executive dysfunction. The profile of cognitive impairment in ALS supports a cognitive continuum between ALS and frontotemporal dementia.
Motor neuron disease Executive function Social cognition Dementia
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We thank the patients with ALS who participated in the research. In addition to the listed authors, we thank the following individuals who made a significant contribution to the work reported in this paper: Dr Rachael Burman; Catherine Knights; Andrew Dougherty; Dr Naomi Martin; Rachel Tuck; Dr Robin Howard; Jan Clarke; Christine Batts; Hazel Watts; Joanna Sasson; Helen Copesy; Trish Cutts; Lucy Adamzyck. This study was funded by the Medical Research Council; The National Institute of Health Research (NIHR) Dementias and Neurodegenerative Diseases Research Network; the Motor Neurone Disease Association. The work leading up to this publication was funded by the European Community’s Health Seventh Framework Programme (AAC & CES, grant number 259867). AAC & CES are involved in two EU Joint Programmes—Neurodegenerative Disease Research (JPND) projects (STRENGTH and ALS-CarE). These projects are supported through the following funding organisations under the aegis of JPND—www.jpnd.eu: United Kingdom, Medical Research Council and Economic and Social Research Council. RGB, AAC, CES and LHG receive salary support from the NIHR Dementia Biomedical Research Unit and/or the NIHR Biomedical Research Centre for Mental Health, both at the South London and Maudsley NHS Foundation Trust and King’s College London. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.
Conflicts of interest
The authors declare that they have no conflict of interest.
Ethics approval was obtained from the National Research Ethics South East London Research Ethics Committee 4 (11/H0807/1). Informed written consent was obtained from all participants. Ethical standards were consistent with the 1964 Declaration of Helsinki and its later amendments.
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