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PLoS One. 2017 Apr 6;12(4):e0173467. doi: 10.1371/journal.pone.0173467. eCollection 2017.

Social cognition in Wilson's disease: A new phenotype?

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Genopsy-Center for the Diagnosis and Management of Genetic Psychiatric Disorders, Le Vinatier Hospital, Lyon, France.
University Department of Rehabilitation (SUR/CL3R), Le Vinatier Hospital, Lyon, France.
Lumière-Lyon 2 University, Lyon, France.
Department of Neurology C, Pierre Wertheimer Neurological Hospital, Hospices Civils de Lyon, Lyon, France.
Center of Cognitive Neurosciences, UMR 5229, CNRS, Lyon, France.
Claude Bernard-Lyon 1 University, Lyon, France.
French National Center for Wilson's Disease, Hospices Civils de Lyon, Lyon, France.
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Lyon, Hospices Civils de Lyon, Lyon, France.
Laboratory of Inherited Metabolic Diseases, CBPE, Lyon, France.
Laboratory of Trace Element and Toxic Metal Analysis, Edouard Herriot Hospital, Lyon, France.


Studies focusing on neuropsychological impairments in Wilson's disease (WD) have highlighted that patients showing neurological signs present significant deficits in a wide range of cognitive domains. Attentional and executive impairments have also been described in people with hepatic WD. However, social cognition abilities, i.e. cognitive processes required to perceive the emotions, intentions and dispositions of other people, have not been clearly investigated in WD. In this study we examined the social cognitive functioning in 19 patients with WD depending on their clinical status-Neurological versus Non-Neurological ("hepatic") forms-compared to 20 healthy controls. For the very first time, results highlighted that patients with WD had significant impairments in the three major components of social cognition: emotion recognition, Theory of Mind and attributional style. However, these deficits differ depending on the form of the disease: patients with neurological signs showed a wide range of deficits in the three components that were assessed-results notably revealed impairments in recognizing "fear", "anger", and "disgust", a significant Theory of Mind deficit and an "aggression bias"-whereas Non-Neurological patients only showed deficits on test assessing attributional bias, with a trend to react more "aggressively" to ambiguous social situations than healthy controls, as observed in Neurological WD patients, and a specific impairment in "anger" recognition. Our findings are discussed in the light of both neurocognitive impairments and brain damages, and especially those affecting the basal ganglia, as observed in people with WD.

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