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

• DOI: 10.1371/journal.pone.0204227
• Publication Date: 2018-09-24T17:32:32Z
• Authors: Garazi Labayru; Irati Arenzana; Jone Aliri; Miren Zulaica; Adolfo López de M...; Andone Sistiaga A.
• Keywords: Adult; Male; Science; prevalence; Emotions; Neuropsychological Tests; spectrum; 03 medical and health sciences; expansion; Cognition; 0302 clinical medicine; Humans; Myotonic Dystrophy; Social Behavior; 10. No inequality; facial emotion recognition; disease; Q; R; Age Factors; muscular-dy frontotemporal dementia; Middle Aged; 3. Good health; quality-of-life; personality; Case-Control Studies; Medicine; muscular-dystroph; Female; Self Report; Empathy; Facial Recognition; metaanalysis; Research Article

The 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.38 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).Statistically 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.DM1 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.