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Neuropsychiatric and Behavioral Profiles of 2 Adults With Williams Syndrome: Response to Antidepressant Intake

Diego Urgeles, MD; Victoria Alonso, MSc; and Tania Ramos-Moreno, PhD

Published: August 1, 2013

Article Abstract

Background: Individuals with Williams syndrome, a rare genetic disorder, are characterized by specific medical, cognitive, and behavioral phenotypes and often have high anxiety levels as well as phobia. Studies of the psychiatric phenotype in adults affected by Williams syndrome or literature on the management of their mental pathologies are lacking.

Method: In this article, we report the neuropsychiatric profile of 2 adult patients with Williams syndrome who also have generalized anxiety disorder and depressive symptoms (DSM-IV-TR criteria), along with their anxiety profiles and the strategies that were adopted for pharmacologic intervention.

Results: Neuropsychiatric profiles revealed a prefrontal cortex affliction that includes an alteration in executive functions. The patients had high scores for trait-anxiety and responded to treatment with a low-potency antipsychotic. A selective serotonin reuptake inhibitor (SSRI) was coadministered with the antipsychotic to alleviate the depressive symptoms. The treatment led to an improvement in self-control, mental concentration, and social skills, as well as decreased irritability and aggressiveness and stabilization of mood.

Conclusions: The combination of SSRIs and low doses of low-potency antipsychotics seems to be the most suitable medication to treat generalized anxiety disorder and related disorders in individuals with Williams syndrome. Manic reactions and increase in anxiety must be closely monitored during treatment. Control of anxiety and sleep should be a priority in these patients, even as a preventative measure.

Prim Care Companion CNS Disord 2013;15(4):doi:10.4088/PCC13m01504

Submitted: January 17, 2013; accepted March 26, 2013.

Published online: August 1, 2013.

Corresponding author: Tania Ramos-Moreno, PhD, Department of Clinical Sciences, Division of Neurology, Experimental Epilepsy Group, Wallenberg Neuroscience Center, Lund University, Lund, Sweden (

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