A 17 year-old-boy with a diagnosis of 48, XXYY syndrome, presented with suicidal ideation and auditory hallucinations. He has had history of problematic sexual behaviors since age six. We discuss his clinical presentation and highlight his behavioral issues, which may be due to low intelligence, sexual abuse, neglect or a behavior pattern of a person with 48, XXYY syndrome. Scientific literature suggests that children at high risk for problematic sexual behavior are those with intellectual disability, history of sexual abuse, neglect, or genetic syndromes. Little is known about the sexual behavior pattern in a case of XXYY syndrome. We discuss this case report to bring forward the sexual presentation of this rare syndrome and how a clinician’s diagnosis-oriented approach fails to address the risky sexual behavior. An important aspect is the lack of training to screen, diagnose, and most importantly intervene optimally.
Subhash Chandra, Cathryn A Galanter, Kenneth J Weiss and Lenore Engel