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Journal of Neurology
Published in: Journal of Neurology 2/2015

01-02-2015 | Original Communication

Theory of mind, empathy and neuropsychological functioning in X-linked Spinal and Bulbar Muscular Atrophy: a controlled study of 20 patients

Authors: Elisa Di Rosa, Gianni Sorarù, Johann Roland Kleinbub, Vincenzo Calvo, Antonino Vallesi, Giorgia Querin, Sonia Marcato, Irene Grasso, Arianna Palmieri

Published in: Journal of Neurology | Issue 2/2015

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Abstract

Recent studies have described brain involvement, mainly at frontal level, in patients with spinal and bulbar muscular atrophy (SBMA), a rare adult-onset motor neuron disease caused by a CAG repeat in the androgen receptor (AR) gene. The aim of our research was to investigate the poorly characterized neuropsychological and psychological profile of these patients, on the basis of previous literature. We administered a neuropsychological screening and tests relating to cognitive and affective empathy, attributed to the theory of mind (ToM) framework, to 20 males with SBMA, and to age- and education-matched controls. Although patients’ neuropsychological performance was unimpaired, a clear dissociation emerged between their cognitive and affective empathy. Patients had distinctive deficits in mentalizing, as assessed with the Faux Pas Test, whilst affective empathy (i.e., sharing experience), assessed with the Reading the Mind in the Eyes test, appeared to be preserved. The likely implications of subtle frontal lobe impairments on the one hand, and a protective influence of androgen insensitivity in these patients on the other, are discussed in the light of our results.
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Metadata
Title
Theory of mind, empathy and neuropsychological functioning in X-linked Spinal and Bulbar Muscular Atrophy: a controlled study of 20 patients
Authors
Elisa Di Rosa
Gianni Sorarù
Johann Roland Kleinbub
Vincenzo Calvo
Antonino Vallesi
Giorgia Querin
Sonia Marcato
Irene Grasso
Arianna Palmieri
Publication date
01-02-2015
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 2/2015
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-014-7567-5

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