Language Development in Preschool Duchenne Muscular Dystrophy Boys

Daniela Pia Rosaria Chieffo, Federica Moriconi, Ludovica Mastrilli, Federica Lino, Claudia Brogna, Giorgia Coratti, Michela Altobelli, Valentina Massaroni, Giulia Norcia, Elisabetta Ferraroli, Simona Lucibello, Marika Pane, Eugenio Maria Mercuri

Research output: Contribution to journalArticle

Abstract

Background: the present study aims to assess language in preschool-aged Duchenne muscular dystrophy (DMD) boys with normal cognitive quotients, and to establish whether language difficulties are related to attentional aspects or to the involvement of brain dystrophin isoforms. Methods: 20 children aged between 48 and 72 months were assessed with language and attention assessments for preschool children. Nine had a mutation upstream of exon 44, five between 44 and 51, four between 51 and 63, and two after exon 63. A control group comprising 20 age-matched boys with a speech language disorder and normal IQ were also used. Results: lexical and syntactic comprehension and denomination were normal in 90% of the boys with Duchenne, while the articulation and repetition of long words, and sentence repetition frequently showed abnormal results (80%). Abnormal results were also found in tests assessing selective and sustained auditory attention. Language difficulties were less frequent in patients with mutations not involving isoforms Dp140 and Dp71. The profile in Duchenne boys was different form the one observed in SLI with no cognitive impairment. Conclusion: The results of our observational cross-sectional study suggest that early language abilities are frequently abnormal in preschool Duchenne boys and should be assessed regardless of their global neurodevelopmental quotient.
Original languageEnglish
Pages (from-to)1252-1252
Number of pages1
JournalBrain Sciences
Volume12
DOIs
Publication statusPublished - 2022

Keywords

  • Duchenne muscular dystrophy
  • auditory attention
  • dystrophin isoforms
  • language development
  • visual attention

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