Association between Reported Sleep Disorders and Behavioral Issues in Children with Myotonic Dystrophy Type 1—Results from a Retrospective Analysis in Italy

<b>Background:</b> Sleep disorders have been poorly described in congenital (CDM) and childhood (ChDM) myotonic dystrophy despite being highly burdensome. The aims of this study were to explore sleep disorders in a cohort of Italian CDM and ChDM and to assess their association with motor and respiratory function and disease-specific cognitive and behavioral assessments. <b>Methods:</b> This was an observational multicenter study. Reported sleep quality was assessed using the Pediatric Daytime Sleepiness Scale (PDSS) and Pediatric Sleep Questionnaire (PSQ). Sleep quality was correlated to motor function (6 min walk test, 6MWT and grip strength; pulmonary function (predicted Forced Vital Capacity%, FVC% pred.); executive function assessed by BRIEF-2; autism traits assessed by Autism Spectrum Screening Questionnaire (ASSQ) and Repetitive Behavior Scale-revised (RBS-R); Quality of life (PedsQL) and disease burden (Congenital Childhood Myotonic Dystrophy Health Index, CCMDHI). <b>Results:</b> Forty-six patients were included, 33 CDM and 13 ChDM, at a median age of 10.4 and 15.1 years. Daytime sleepiness and disrupted sleep were reported by 30% children, in both subgroups of CDM and ChDM. Daytime sleepiness correlated with autism traits in CDM (<i>p</i> < 0.05). Disrupted sleep correlated with poorer executive function (<i>p</i> = 0.04) and higher disease burden (<i>p</i> = 0.03). <b>Conclusions:</b> Sleep issues are a feature of both CDM and ChDM. They correlate with behavioral issues and impact on disease burden.

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Publication Details

Journal
Journal of Clinical Medicine
Published
2024-09-14
DOI
https://doi.org/10.3390/jcm13185459
Citations
7
Primary Topic
Genetic Neurodegenerative Diseases
Type
article
Field-Weighted Citation Impact
1.88

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article

Association between Reported Sleep Disorders and Behavioral Issues in Children with Myotonic Dystrophy Type 1—Results from a Retrospective Analysis in Italy

Federica Trucco, Gemma Marinella, Susanna Pozzi, Adele D’Amico et al.
7 citations
Journal of Clinical Medicine
Genetic Neurodegenerative Diseases
1.88
article

Association between Reported Sleep Disorders and Behavioral Issues in Children with Myotonic Dystrophy Type 1—Results from a Retrospective Analysis in Italy

Federica Trucco, Gemma Marinella, Susanna Pozzi, Adele D’Amico, Elisabetta Roma, Nicholas E. Johnson, Valeria Sansone, Stefano Becchiati, Federica Ricci, Anna Salvalaggio, F. Bruschi, Eugenio Maria Mercuri, Stefano Parravicini, Roberta Battini, Marco Veneruso, Marika Pane, Antonella Pini, Guja Astrea, Alessandra Di Bari, Federica Rachele Danti, Chiara Fiorillo, Angela Lucia Berardinelli, Isabella Moroni, Andrea Lizio, Francesca Salmin, Emilio Albamonte, Michela Catteruccia, Jacopo Casiraghi, Michele Tosi, Laura Antonaci
article en
7 citations

Abstract

<b>Background:</b> Sleep disorders have been poorly described in congenital (CDM) and childhood (ChDM) myotonic dystrophy despite being highly burdensome. The aims of this study were to explore sleep disorders in a cohort of Italian CDM and ChDM and to assess their association with motor and respiratory function and disease-specific cognitive and behavioral assessments. <b>Methods:</b> This was an observational multicenter study. Reported sleep quality was assessed using the Pediatric Daytime Sleepiness Scale (PDSS) and Pediatric Sleep Questionnaire (PSQ). Sleep quality was correlated to motor function (6 min walk test, 6MWT and grip strength; pulmonary function (predicted Forced Vital Capacity%, FVC% pred.); executive function assessed by BRIEF-2; autism traits assessed by Autism Spectrum Screening Questionnaire (ASSQ) and Repetitive Behavior Scale-revised (RBS-R); Quality of life (PedsQL) and disease burden (Congenital Childhood Myotonic Dystrophy Health Index, CCMDHI). <b>Results:</b> Forty-six patients were included, 33 CDM and 13 ChDM, at a median age of 10.4 and 15.1 years. Daytime sleepiness and disrupted sleep were reported by 30% children, in both subgroups of CDM and ChDM. Daytime sleepiness correlated with autism traits in CDM (<i>p</i> < 0.05). Disrupted sleep correlated with poorer executive function (<i>p</i> = 0.04) and higher disease burden (<i>p</i> = 0.03). <b>Conclusions:</b> Sleep issues are a feature of both CDM and ChDM. They correlate with behavioral issues and impact on disease burden.

Journal of Clinical MedicineVol. 13(18)
Università Cattolica del Sacro Cuore (IT), University of Pisa (IT), Virginia Commonwealth University (US), University of Milan (IT), University of Pavia (IT), Istituto Giannina Gaslini (IT), Agostino Gemelli University Polyclinic (IT), Centro Clinico Nemo (IT), Istituto delle Scienze Neurologiche di Bologna (IT), Bambino Gesù Children's Hospital (IT), Fondazione Stella Maris (IT), Istituti di Ricovero e Cura a Carattere Scientifico (IT), Fondazione IRCCS Istituto Neurologico Carlo Besta (IT), University of Turin (IT)
University of Rochester
No poverty
Openalex Percentile: Top 14%
Genetic Neurodegenerative Diseases
1.88
DOIOpen Access PDFOpenAlex
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