Social cognition in DMD and BMD dystrophinopathies: A cross-sectional preliminary study

Abstract

Objective: The dystrophinopathies called Duchenne and Becker muscular dystrophies (DMD/BMD) are rare, progressive, incurable, and life-limiting paediatric-onset neuromuscular diseases. These diseases have long been associated with specific neuropsychological deficits. However, the performance of these patients in the social cognition domain has not been properly investigated. Thus, the main objective of this study was to compare the performance on social cognition between DMD/BMD patients and healthy age-matched boys. Method: This cross-sectional study included 20 DMD/BMD children and adolescents and 20 healthy controls. The protocol included the Social Perception Domain of the NEPSY-II, the Reading the Mind in the Eyes Test – Child and Happé’s Strange Stories test. General intelligence was controlled to eliminate the possible influence of covariables. All the assessments were performed remotely. Results: Most social cognition tasks were worse in patients with DMD/BMD than in matched healthy controls. These differences remained even after controlling for the general intelligence variable, with the exception of Total Disgust Errors (F = 1.462, p = .234, η²_p= .038) and Verbal task (F = 1.820, p = .185, η²_p= .047) scores from the NEPSY-II. Conclusions: This is the first study to demonstrate that the neuropsychological domain of social cognition is impaired in DMD/BMD patients, independent of the level of general intelligence. Screening assessments in DMD/BMD patients should be promoted to allow social cognition difficulties to be detected at an early stage to enhance patients’ quality of life and social development.

Keywords:

• DMD
• BMD
• dystrophinopathies
• social cognition
• general intelligence

Acknowledgments

We thank all of the participants for their involvement in the study; especially, we are grateful to DPP Spain and ASEM for their collaboration.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

This study was funded by a grant from the Deusto International Research School (DIRS) ‘Programa de Ayudas para Formación de Personal Investigador de la Universidad de Deusto’ (FPI UD_2021_04 to Irune García).