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Highlights

  • Behavioral problems as reported in children with DS are not frequently seen in adulthood.
  • Hyperactivity was no longer a clinical feature in the adult patients.
  • The use of psychoactive medication in adulthood was limited.
  • On the other hand, self-mutilation rates were higher than reported in the literature.
  • Autism or autistic-like behaviors are common in adults with DS, though these may be ID-related rather than DS-related.

Abstract

Introduction

Autism and behavioral characteristics in adults with Dravet syndrome (DS) have rarely been systematically studied.

Method

Three scales were used to assess the outcomes of DS in adulthood in terms of autism and behavior. All the adult patients with DS, nine male and four female, aged between 18 and 60 years, living at the Epilepsy Center Kempenhaeghe in The Netherlands were included in the study. In addition, the past medical history of each patient was systematically screened for diagnoses like autism, Pervasive Development Disorder-Not Otherwise Specified (PDD-NOS), autism spectrum disorder (ASD), hyperactivity, Attention Deficit Hyperactivity Disorder (ADHD), and self-mutilation. Information concerning past and current use of psychoactive drugs was also evaluated.

Results

Eight patients (61.5%) were classified as having autism spectrum disorder (ASD) according to the AVZ-R or according to the medical record. Self-mutilation was seen in four patients (30.8%), hyperactivity in none. Three patients (23.1%) currently used psychoactive drugs.

Conclusion

Autism spectrum disorders persist in adult patients with DS, while certain characteristics associated with behavioral problems, such as hyperactivity or use of psychoactive medication, seem to be less prominent than in childhood.

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