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Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy

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...

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Detalles Bibliográficos
Autores principales: Anderson, Danyon J, Porwal, Mokshal H, Sandlow, Jay I
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9184178/
https://www.ncbi.nlm.nih.gov/pubmed/35698716
http://dx.doi.org/10.7759/cureus.24865
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author Anderson, Danyon J
Porwal, Mokshal H
Sandlow, Jay I
author_facet Anderson, Danyon J
Porwal, Mokshal H
Sandlow, Jay I
author_sort Anderson, Danyon J
collection PubMed
description 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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spelling pubmed-91841782022-06-12 Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy Anderson, Danyon J Porwal, Mokshal H Sandlow, Jay I Cureus Neurology 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. Cureus 2022-05-09 /pmc/articles/PMC9184178/ /pubmed/35698716 http://dx.doi.org/10.7759/cureus.24865 Text en Copyright © 2022, Anderson et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Neurology
Anderson, Danyon J
Porwal, Mokshal H
Sandlow, Jay I
Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
title Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
title_full Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
title_fullStr Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
title_full_unstemmed Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
title_short Testosterone-Mediated Aggression in Angelman Syndrome Treated With Leuprolide and Orchiectomy
title_sort testosterone-mediated aggression in angelman syndrome treated with leuprolide and orchiectomy
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9184178/
https://www.ncbi.nlm.nih.gov/pubmed/35698716
http://dx.doi.org/10.7759/cureus.24865
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