Music therapy and Sanfilippo syndrome: an analysis of psychological and physiological variables of three case studies

P Pérez-Núñez,S Berrocoso,J F López-Paz,I Amayra,E Lázaro,P Caballero,A A Rodríguez,M Al-Rashaida,M Pérez,O Martínez,P M Luna,M García

doi:10.1186/s13023-021-02123-6

Abstract

IntroductionMucopolysaccharidosis type III (MPS III) or Sanfilippo syndrome is a neurodegenerative disease caused by the accumulation of mucopolysaccharides in the body. As the symptoms are wide ranging, it is a challenge to provide a diagnosis and psychological treatment for affected children.MethodThe main objective of this study was to describe a form of music therapy treatment applied to three children diagnosed with MPS III. The psychological variables were evaluated by an ad hoc observation recording template, and the physiological variables were measured with a digital meter before and after each session. The perception of the parents was also considered through a semi-structured interview.ResultsAn improvement in the psychological variables was shown in all cases. Changes in the physiological variables were also noted, although they varied according to each child. The parents report some benefit of music therapy and they share difficulty in assessing the extent of benefits of the music therapy.DiscussionFindings indicate that music therapy can be a useful form of treatment with multiple benefits for children with conditions such as MPS III or similar conditions. However, further research is needed in this area and in the development of specific ways of evaluating music therapy.

Highlights

Mucopolysaccharidosis type III (MPS III) or Sanfilippo syndrome is a neurodegenerative disease caused by the accumulation of mucopolysaccharides in the body
Findings indicate that music therapy can be a useful form of treatment with multiple benefits for chil‐ dren with conditions such as MPS III or similar conditions
Music therapy is presented as an appropriate intervention model for this particular pathology due to its clinical profile, there are hardly any studies that go into detail on the effects of its application [28,29,30,31,32,33,34]. In response to these shortcomings, this study aims to describe a music therapy programme applied to children with MPS III and to analyse the psychological and physiological variables related to the symptoms of the disease through quantitative and qualitative observational analysis

Summary

Introduction

Mucopolysaccharidosis type III (MPS III) or Sanfilippo syndrome is a neurodegenerative disease caused by the accumulation of mucopolysaccharides in the body. The disease is inherited in an autosomal recessive manner and is mainly characterised by severe degeneration of the central nervous system [6, 7]. It was first described in 1963 by paediatrician Sylvestre Sanfilippo, who studied a case of eight children with intellectual disability and elevated urinary excretion of a single GAG, heparan sulphate [8]

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