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Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
Danyon J Anderson1, Mokshal H Porwal1, Jay I Sandlow1
1Urology, Medical College of Wisconsin, Wauwatosa, USA.
Abstract:
Angelman syndrome (AS) is a rare genetic imprinting disorder characterized by a maternal microdeletion of the 15q11q13 locus. It is traditionally associated with intellectual disability, inappropriate laughing, and a happy demeanor. Here, we report a patient with AS who presented with aggression and hypersexuality and was successfully treated with leuprolide injections for nine years until a definitive orchiectomy was performed. To the best of our knowledge, this is the first report of castration as a treatment for refractory behavioral symptoms in a patient with AS.
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