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

Abstract

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.

Deficits of facial emotion recognition and visual information processing in adult patients with classical galactosemia

“…Deficits in emotion recognition have also been described in other inborn errors of metabolism, such as Wilson’s disease [28] and tyrosinemia type I [29]. As in both studies different tests were used, conclusions by comparing the results can only be drawn cautiously.…”

“…In the study with patients with Wilson’s disease, the most significant deficit was found in recognizing “anger”, while in our galactosemia patients, the most important difficult emotion was “fear”. The authors argue that patients with Wilson’s disease tend to react more aggressively to ambiguous social situations than healthy controls [28]. In contrast, galactosemia patients, as mentioned before, tend to be shy and withdrawn.…”

The Patient-Reported Experience of Living with Wilson Disease

“…Some patients may also present with musculoskeletal abnormalities such as premature osteoarthritis, skeletal deformities or bone fractures [10]. Common neurological symptoms include motor impairments, problems with memory, visuospatial processing, executive function, and personality and behavioral disorders [8,11,12]. Neurological symptoms have also been shown to include reacting with increased aggression to certain social situations [11].…”

“…Common neurological symptoms include motor impairments, problems with memory, visuospatial processing, executive function, and personality and behavioral disorders [8,11,12]. Neurological symptoms have also been shown to include reacting with increased aggression to certain social situations [11]. This and other psychiatric symptoms seen in patients with Wilson disease have been linked with decreased quality of life [13].…”

Social cognition remediation interventions: A systematic mapping review

“…Several neurological disorders such as behavioral-variant frontotemporal dementia [44], traumatic brain injury [45], or Parkinson’s disease [46] are characterized by impairments in social cognition [47]. Other disorders with central nervous system involvement such as tuberous sclerosis [48], fragile X syndrome [49], Prader Willi syndrome [50], or Wilson’s disease [51] have also been related to social cognition impairments. Therefore, it seems interesting to study which areas of knowledge are developing research on social cognition remediation, including medicine and related fields such as psychology and neuroscience.…”