Social cognition in myotonic dystrophy type 1: Specific or secondary impairment?

Abstract

AimsThe cognitive profile of Myotonic Dystrophy type 1 (DM1) has been described in recent decades. Moreover, DM1 patients show lowered social engagement and difficulties in social-cognitive functions. The aim of the present study is to explore whether social cognition impairment is present in DM1 taking into account the overall cognitive condition.Method38 patients and a control group paired in age and gender participated in the study. All the participants had an IQ within the normal range. Subjects were administered an abbreviated neuropsychological battery which comprised a facial emotion recognition test (POFA) and Faux Pas Test, as well as a self-report questionnaire on cognitive and affective empathy (TECA).ResultsStatistically significant differences were found only for facial emotion recognition (U = 464.0, p = .006) with a moderate effect size (.31), with the controls obtaining a higher score than the patients. Analyzing each emotion separately, DM1 patients scored significantly lower than controls on the recognition of anger and disgust items. Emotion recognition did not correlate with genetic load, but did correlate negatively with age. No differences were found between patients and controls in any of the other variables related to Theory of Mind (ToM) and empathy.ConclusionDM1 does not manifest specific impairments in ToM since difficulties in this area predominantly rely on the cognitive demand of the tasks employed. However, a more basic process such as emotion recognition appears as a core deficit. The role of this deficit as a marker of aging related decline is discussed.

Abnormal Cortical Thickness Is Associated With Deficits in Social Cognition in Patients With Myotonic Dystrophy Type 1

“…We have indeed confirmed the presence of difficulties in emotion processing (mainly for negative emotions) with our patients scoring below the normality cutoff when required to recognize not only sadness, embarrassment, and anger, but also happiness. Nevertheless, our findings are also partially different compared to those reported by Labayru et al (19), who found difficulties mainly in the recognition of anger and disgust. In our opinion, such differences are likely due to the different material used to assess emotions; indeed, Labayru et al (19) used a facial emotion recognition test, whereas in our patients, we evaluated the emotion attribution processing using brief verbal stories.…”

“…Nevertheless, our findings are also partially different compared to those reported by Labayru et al (19), who found difficulties mainly in the recognition of anger and disgust. In our opinion, such differences are likely due to the different material used to assess emotions; indeed, Labayru et al (19) used a facial emotion recognition test, whereas in our patients, we evaluated the emotion attribution processing using brief verbal stories. This test, which is part of the Social Cognition Battery, includes 58 brief stories that are read by the clinician.…”

“…More recently, face emotion recognition was found impaired in a cohort of DM1 patients with prominent difficulties for faces showing negative emotions such as anger and disgust, and crucially deficits were more extensive than expected (19). In our current work, we confirmed the conclusions drawn by Labayru et al (19), and we contributed to clarify the neurobiological substrates of SC deficits in DM1 patients, especially for their emotion processing and moral/non-moral judgments abilities. We have indeed confirmed the presence of difficulties in emotion processing (mainly for negative emotions) with our patients scoring below the normality cutoff when required to recognize not only sadness, embarrassment, and anger, but also happiness.…”

“…Theory of Mind deficits were previously documented in DM1 patients and associated with abnormal functional connectivity in specific brain networks (16). More recently, face emotion recognition was found impaired in a cohort of DM1 patients with prominent difficulties for faces showing negative emotions such as anger and disgust, and crucially deficits were more extensive than expected (19). In our current work, we confirmed the conclusions drawn by Labayru et al (19), and we contributed to clarify the neurobiological substrates of SC deficits in DM1 patients, especially for their emotion processing and moral/non-moral judgments abilities.…”

“…In DM1 patients, we recently identified functional brain abnormalities within networks implicated in ToM ability (16). Emotion recognition-wise, specific deficits have also been reported in patients with DM1 (19), although there are no available studies exploring their neurobiological substrate.…”