Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome

Gonzalo Laje, Rebecca Bernert, Rebecca Morse, Maryland Pao, Ann C.M. Smith

Research output: Contribution to journalArticlepeer-review

Abstract

Smith-Magenis syndrome (SMS) is a complex genetic syndrome caused by an interstitial deletion of chromosome 17p11.2. Children and adults with SMS appear to have unique neurobehavioral problems that include: sleep disturbance, self-injurious and maladaptive behaviors, stereotypies, and sensory integration disorders. We gathered retrospective psychotropic use information from parents or other caregivers of 62 individuals with SMS who were asked about use of psychotropic medication from a list of commonly used psychiatric medications. For those drugs identified, respondents were asked to rate the experience with the particular medication using a likert-type scale. Drugs were grouped into seven main categories: (1) stimulants; (2) antidepressants; (3) antipsychotics; (4) sleep aides; (5) mood stabilizers; (6) alpha 2 agonists; and (7) benzodiazepines. Relative frequencies, means and standard deviations pertaining to age and medication effect were derived for each medication category. Six of the seven medication categories examined showed no meaningful deviations from the "no change" score. The benzodiazepine group showed a mild detrimental effect. There were no gender differences in efficacy. Use of psychotropic medication started early in life (mean age 5 years), particularly with sleep aides. Although no medication category was identified as efficacious in SMS, all the categories reported herein may be considered as an option for brief symptomatic relief. Published 2010 Wiley-Liss, Inc.

Original languageEnglish (US)
Pages (from-to)463-468
Number of pages6
JournalAmerican Journal of Medical Genetics, Part C: Seminars in Medical Genetics
Volume154
Issue number4
DOIs
StatePublished - Nov 2010
Externally publishedYes

Keywords

  • Aggression
  • Autism
  • Genetics
  • Melatonin
  • Mental retardation
  • Pharmacogenetics
  • Pharmacogenomics
  • Pharmacology
  • Self-injurious behavior
  • Sleep
  • Smith-Magenis syndrome (SMS)
  • Treatment

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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