Prevalence of Neurodevelopmental Disorders in Children with Duchenne Muscular Dystrophy in Srinagarind Hospital
Keywords:
Duchenne Muscular Dystrophy (DMD), Intellectual disabilities (ID), Intellectual quotient (IQ), Autistic spectrum disorder (ASD), Attention deficit hyperactivity disorder (ADHD)Abstract
Background: Duchenne Muscular Dystrophy (DMD) has an association with neurodevelopmental disorders, such as Intellectual disabilities (ID), Autistic spectrum disorder (ASD) and Attention deficit hyperactivity disorder (ADHD). These comorbidities are an important factor affects DMD patient’s quality of life.
Objectives: To define the prevalence of neurodevelopmental disorders and genotypephenotype correlation in Thai children with DMD.
Methods: A descriptive cohort study, Neurodevelopmental disorders were evaluated in Thai DMD children under 18 years old in Srinagarind hospital. Development and behavioral problems were evaluated. ASD and ADHD were screened. IQ was evaluated by clinical psychologist. Making a diagnosis of neurodevelopmental disorders was established by developmental-behavioral pediatrician using DSM-5 criteria. Genotype-phenotype correlation was evaluated.
Results: Twenty Thai boys with DMD were enrolled. The median age was 9.5 years. Twelve patients (60%) had ID, one patient had both ASD and ID, one patient had both ADHD and ID. Seven patients (35%) had mild ID (IQ 50-69), 5 patients (25%) had moderate ID (IQ 36-49), 4 patients (20%) had borderline IQ (IQ 70-79), 3 patients (15%) had low normal IQ (IQ 80-89), and 1 patient had normal IQ (IQ>90). Median IQ of all patients was 68.0 (IQR 62.0, 81.0). Eleven patients (64.7%) have deletion/duplication of DMD gene. No statistically significant correlations between genotypes and level of IQ and types of neurodevelopmental disorders were identified.
Conclusions: The prevalence of ID in children with DMD is high while ASD and ADHD are less common. The association of genotype and cognitive functions and behavioral problems in children with DMD remains unclear.
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