Buspirone for the treatment of anxiety in Williams syndrome: a retrospective chart review study.

BACKGROUND: Williams syndrome (WS) is a rare genetic disorder associated with a high prevalence of anxiety disorders. Evidence-based pharmacologic treatments for anxiety in WS are lacking. The purpose of this study is to provide naturalistic data on the use of buspirone for the treatment of anxiety in WS. RESEARCH DESIGN AND METHODS: Medical records of 24 individuals with Williams syndrome (ages 7-47 years) and anxiety who received treatment with buspirone were reviewed. Treatment response to buspirone was rated by assigning a retrospective Clinical Global Impression Improvement subscale (CGI-I) score. RESULTS: Twenty-three of 24 (96%) patients completed at least a 16-week treatment course with buspirone. Sixteen patients (67%; 95% CI 47%, 82%) were treatment responders (CGI-I ≤ 2). Only 1 (4%) patient discontinued buspirone due to a treatment-emergent side effect (nausea and vomiting). The most common side effect was nausea (13%). Twenty (84%) patients remained on buspirone at the time of their most recent follow-up visit. CONCLUSIONS: In this retrospective study, the majority of patients responded to a 16-week course of buspirone. Prospective studies are warranted to further assess the efficacy and tolerability of buspirone for anxiety in WS.

Body mass index variation in adults with Williams syndrome: associations with predicted dietary intake and food behaviors.

Background: Dietary intake and body weight are important predictors of long-term health. However, few studies have focused on these topics in adults with genetic syndromes that have associated intellectual disability, such as Williams syndrome (WS). Objective: In adults with WS, describe predicted dietary intake, food-related problems, and associations between body mass index (BMI) and possible factors contributing to differences in weight status. Design: In this study of 82 participants (median age of 30 years, range 18-69), we cross sectionally investigated associations between BMI, predicted dietary intakes (Dietary Screener Questionnaire), food-related behaviors (Food-Related Problem Questionnaire), and anxiety (Spence Children's Anxiety Scale). Longitudinal patterns of weight change were further studied in a subset (n = 41). Results: BMI variation was observed with median BMI of 27.3 kg/m2 (range 16.7-55.5 kg/m2). Several components of dietary intake deviated from recommendations in the WS cohort. When compared with WS participants with either normal or overweight BMI, WS participants with obesity had reduced daily intake of fruits and vegetables of 0.15 cup equivalents (P = 0.049), while participants with underweight BMI had reduced daily intake of fruits and vegetables of 0.44 cup equivalents (P = 0.026) and additionally had reduced intake of dietary fiber of 2.12 grams per day (P = 0.019). A one-point increase in the 'preoccupation with food' sub-score was associated with a 0.57 unit increase in BMI (P = 0.16), while a one-point increase in the 'takes and stores food' sub-score was associated with a 0.72 unit increase in BMI. In the longitudinal weight subset, a weight gain group and a weight stable group were identified. The former was associated with increased 'takes and stores food' sub-score but not with dietary intakes. Conclusion: We observed considerable BMI variability. While few dietary intakes were associated with BMI, increased BMI and weight gain were associated with 'preoccupation with food' and with 'takes and stores food' behavior sub-scores.

Diversity of Participants in Williams Syndrome Intervention Studies.

PURPOSE: This study describes participant diversity in Williams syndrome (WS) intervention studies. METHODS: A literature search was conducted to identify prospective treatment studies including participants with WS. Data was extracted on the reporting of and information provided on age, sex, cognitive ability, socioeconomic status, race, and ethnicity. RESULTS: Eleven eligible articles were identified. Reporting rates of demographic factors varied considerably, with the highest rates for age and sex (100%) and the lowest reporting rates for race (18%) and ethnicity (9%). Combining demographic data from the two studies that reported on race and/or ethnicity (n = 33), 88% of participants were White. The combined participant mean age was 20.9 years. CONCLUSION: There is a low frequency of reporting on several demographic factors including socioeconomic status, race, and ethnicity in WS intervention studies. There is a need for increased representation of racial and ethnic minority groups, older participants, and more cognitively impaired patients in WS research.