Topiramate in the treatment of Prader-Willi syndrome: A case report.

Background: Prader-Willi syndrome (PWS), a neurologic disorder caused by a mutation of chromosome 15, is characterized by such symptoms as hypotonia, hypogonadism, hyperphagia, cognitive impairment, and difficult behaviors. One of the most concerning symptoms is hyperphagia, which can lead to uncontrolled obesity. Obesity is a major cause of increased morbidity and mortality in patients with PWS; however, diagnosing PWS early in life improves the prognosis.

Case Summary: An 11-year-old African American boy with a past medical history significant for PWS, attention deficit/hyperactivity disorder, oppositional defiant disorder, obesity, and asthma was admitted after he became violent and destructive at his foster home while trying to get food. The patient had a 28-day stay on the children's crisis intervention unit where quetiapine was discontinued and he was maintained on clonidine 0.1 mg 3 times daily, hydroxyzine 25 mg in the morning and 50 mg at bedtime, montelukast 5 mg daily and titrated on methylphenidate 10 mg in the morning and 5 mg in the afternoon, topiramate 100 mg twice daily, and aripiprazole 10 mg twice daily. The patient displayed improved behavior control and less food-related aggression; he denied any side effects of medications.

Discussion: This case demonstrates the positive effects of topiramate for reducing aggression and demand for food in a child with PWS most likely due to an increase in satiety. It is hard to definitively attribute the positive response directly to topiramate. Further research should be conducted to determine if topiramate is an effective treatment option in these individuals.