Neurodevelopmental, Behavioral, and Emotional Symptoms in Becker Muscular Dystrophy

Lambert JT, Darmahkasih AJ, Horn PS, et al. Neurodevelopmental, Behavioral, and Emotional Symptoms in Becker Muscular Dystrophy. Muscle & nerve 2019

Abstract

INTRODUCTION: Becker muscular dystrophy (BMD) results in decreased dystrophin with implications for mental health.

METHODS: This is a retrospective case series of neurodevelopmental, behavioral, and emotional symptoms and respective pharmacotherapies of 70 BMD patients.

RESULTS: Fifty-four patients (77.1%) exhibited at least one symptom, and 19 patients (27.1%) exhibited four or more. The most prevalent symptoms were specific learning disabilities or special education needs (31.4%), inattention/hyperactivity (35.7%), language/speech delays (35.7%), and emotional or behavioral dysregulation (38.6%). Fisher’s exact tests indicated that anxiety was more prevalent with mutations upstream of exon 30 (p = 0.049), but the prevalence of other symptoms did not differ with respect to mutation sites. Similarly, the number of symptoms individual BMD subjects exhibited did not differ with respect to mutation sites. Seventeen (24.3%) patients required pharmacotherapy to manage symptoms.

DISCUSSION: Neurodevelopmental, behavioral, and emotional symptoms are prevalent in patients with BMD regardless of dystrophin gene mutation site.

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