Abstract

Background: Spino-bulbar muscular atrophy is a rare genetic X-linked disease caused by testosterone insensitivity. An inverse correlation has been described between testosterone levels and empathic responses. The present study explored the profile of neural empathic responding in spino-bulbar muscular atrophy patients. Methods: Eighteen patients with spino-bulbar muscular atrophy and eighteen healthy male controls were enrolled in the study. Their event-related potentials were recorded during an “Empathy Task” designed to distinguish neural responses linked with experience-sharing (early response) and mentalizing (late response) components of empathy. The task involved the presentation of contextual information (painful vs. neutral sentences) and facial expressions (painful vs. neutral). An explicit dispositional empathy-related questionnaire was also administered to all participants, who were screened via neuropsychological battery tests that did not reveal potential cognitive deficits. Due to electrophysiological artefacts, data from 12 patients and 17 controls were finally included in the analyses. Results: Although patients and controls did not differ in terms of dispositional, explicit empathic self-ratings, notably conservative event-related potentials analyses (i.e., spatio-temporal permutation cluster analyses) showed a significantly greater experience-sharing neural response in patients compared to healthy controls in the Empathy-task when both contextual information and facial expressions were painful. Conclusion: The present study contributes to the characterization of the psychological profile of patients with spino-bulbar muscular atrophy, highlighting the peculiarities in enhanced neural responses underlying empathic reactions.

Highlights

  • Spino-bulbar muscular atrophy is a rare genetic X-linked disease caused by testosterone insensitivity

  • The samples for the analyses concerning the neuropsychological battery screening and the explicit dispositional empathy questionnaires were paired for age and education

  • The findings of the present study revealed enhanced neural empathic responses in painful faces preceded by painful contexts elicited significantly more positive event-related potential (ERP) compatients with X-linked SBMA disease

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Summary

Introduction

Spino-bulbar muscular atrophy is a rare genetic X-linked disease caused by testosterone insensitivity. Methods: Eighteen patients with spino-bulbar muscular atrophy and eighteen healthy male controls were enrolled in the study. Their event-related potentials were recorded during an “Empathy Task” designed to distinguish neural responses linked with experiencesharing (early response) and mentalizing (late response) components of empathy. Results: patients and controls did not differ in terms of dispositional, explicit empathic self-ratings, notably conservative event-related potentials analyses (i.e., spatio-temporal permutation cluster analyses) showed a significantly greater experiencesharing neural response in patients compared to healthy controls in the Empathy-task when both contextual information and facial expressions were painful. Conclusion: The present study contributes to the characterization of the psychological profile of patients with spino-bulbar muscular atrophy, highlighting the peculiarities in enhanced neural responses underlying empathic reactions

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