title: Phenotypic psychiatric characterization of children with Williams syndrome and response of those with ADHD to methylphenidate treatment.
abstract: Williams syndrome (WS) is associated with cognitive deficits, special behavioral phenotype, and high rates of psychiatric disorders. The aims of the present study were: (1) To compare the rates of psychiatric disorders and repetitive behaviors in children with WS to children with idiopathic developmental disability (DDs); (2) To longitudinally assess the change in psychiatric disorders during adolescence in WS; (3) To assess retrospectively the effectiveness and safety of methylphenidate (MPH) treatment in WS children with ADHD. The study consisted of a cohort of 38 children and adolescents (age 13.1 ± 5.2 years) with WS and a sample of age-matched DDs (age 15.0 ± 3.1 years). A current follow-up evaluation was conducted after 5.6 ± 1.6 years for 25 subjects (65.8%) of the WS cohort. The rate of most psychiatric disorders was found similar in children with WS and DD controls. Specific phobia, especially from noises, obsessive-compulsive symptoms (e.g., aggressive obsessions and repetitive questions), and stereotypic behaviors (e.g., glancing), were more common in WS than DDs. In a longitudinal follow-up of the WS children, we found a decrease in the rate of anxiety disorders. In addition, a clinically significant improvement was reported in 72.2% of WS children with ADHD following MPH treatment. Sadness/unhappiness was the most common side effect associated with MPH treatment in WS, occurring in 2/3 of treated individuals. The present study further elucidates the neuropsychiatric phenotype of WS. Our results also suggest that MPH treatment for ADHD in WS warrants future prospective controlled trials.
authors: Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: Tamar
authors: T
authors: Green
authors: Sarit
authors: S
authors: Avda
authors: Inbar
authors: I
authors: Dotan
authors: Omer
authors: O
authors: Zarchi
authors: Lina
authors: L
authors: Basel-Vanagaite
authors: Gil
authors: G
authors: Zalsman
authors: Abraham
authors: A
authors: Weizman
authors: Doron
authors: D
authors: Gothelf
mesh_terms: D000293:Adolescent; D001289:Attention Deficit Disorder with Hyperactivity / Q000150:complications* / Q000188:drug therapy*; D016022:Case-Control Studies; D000697:Central Nervous System Stimulants / Q000494:pharmacology / Q000627:therapeutic use; D002648:Child; D002658:Developmental Disabilities / Q000150:complications / Q000523:psychology; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008774:Methylphenidate / Q000494:pharmacology / Q000627:therapeutic use*; D009771:Obsessive-Compulsive Disorder / Q000150:complications; D010641:Phenotype; D017367:Selective Serotonin Reuptake Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D013239:Stereotyped Behavior / Q000187:drug effects; D018980:Williams Syndrome / Q000150:complications* / Q000188:drug therapy / Q000523:psychology*
chemical_list: D000697:Central Nervous System Stimulants; D008774:Methylphenidate; D017367:Selective Serotonin Reuptake Inhibitors