Behavioral and psychiatric features of Sturge-Weber syndrome.

Sturge-Weber syndrome (SWS) is characterized by seizures, port-wine birthmarks, vascular malformations, and rarely studied psychobehavioral features. This study describes a small group of outpatients (N = 16, age, 3-34 years) with Sturge-Weber syndrome seeking medical services (due to seizures, ophthalmological, and dermatological problems among others). The patients were screened for psychiatric diagnoses. The most frequent diagnoses were mood disorder (31%), disruptive behavior disorder (25%), and adjustment disorder (25%). A substance-related disorder was the most frequent in adults (67%). A significant association was found between disruptive behavior disorder not otherwise specified and more left frontal and left parietal involvement. A trend toward significant association of having a seizure in the past 3 months with disruptive behavior disorder not otherwise specified was observed. Problems with mood, attention, sleep, learning, and substance use were common. Disruptive behavior disorders and their association with medical conditions should be further investigated.