title: Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome.
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.
authors.affiliation: Intramural Research Program at the National Institute of Mental Health, NIH, Bethesda, MD 20892-3719, USA. gonzalo.laje@nih.gov
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Laje
authors.forename: Rebecca
authors.initials: R
authors.lastname: Bernert
authors.forename: Rebecca
authors.initials: R
authors.lastname: Morse
authors.forename: Maryland
authors.initials: M
authors.lastname: Pao
authors.forename: Ann C M
authors.initials: AC
authors.lastname: Smith
mesh_terms: D000328:Adult; D000367:Age Factors; D000704:Analysis of Variance; D019958:Attention Deficit and Disruptive Behavior Disorders / Q000188:drug therapy* / Q000503:physiopathology*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D011619:Psychotropic Drugs / Q000145:classification / Q000627:therapeutic use*; D012189:Retrospective Studies; D058496:Smith-Magenis Syndrome / Q000503:physiopathology*; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: D011619:Psychotropic Drugs