Johanson-Blizzard syndrome--a case study, behavioral manifestations, and successful treatment strategies

Mental retardation has multiple causes, including genetic, and is often accompanied by severe behavioral difficulties. These behavioral problems frequently disrupt care, reduce quality of life, and represent risk of injury. In treating special populations where biologic interventions are most important, the psychiatrist must often combine appropriate pharmacologic interventions with behavioral strategies, employing staff support and using a multidisciplined team approach. This article reviews a case in which a person with Johanson-Blizzard syndrome, a rare genetic disorder whose physical manifestations have been described in the literature, is successfully treated using a combination of psychotropic medication and behavioral programming. Target behaviors of severe obsessive compulsive disorder and aggression were ameliorated using these strategies.Currently, specific pharmacologic interventions in combination with structured behavioral programming represents the most successful method of dealing with various forms of behavioral problems linked to mental retardation.