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Aripiprazole treatment for temper outbursts in Prader–Willi syndrome

BACKGROUND: Prader–Willi syndrome (PWS) is a rare neurodevelopmental disorder based on a loss of paternally expressed genes in chromosome segment 15q11-13. Behavioral traits such as temper outbursts, stereotypic, and ritualistic behavior, as well as an increased risk of psychosis accompany the syndr...

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Autores principales: Deest, Maximilian, Wieting, Jelte, Jakob, Maximilian Michael, Deest-Gaubatz, Stephanie, Groh, Adrian, Seifert, Johanna, Toto, Sermin, Bleich, Stefan, Frieling, Helge, Eberlein, Christian K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9419314/
https://www.ncbi.nlm.nih.gov/pubmed/36028863
http://dx.doi.org/10.1186/s13023-022-02470-y
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author Deest, Maximilian
Wieting, Jelte
Jakob, Maximilian Michael
Deest-Gaubatz, Stephanie
Groh, Adrian
Seifert, Johanna
Toto, Sermin
Bleich, Stefan
Frieling, Helge
Eberlein, Christian K.
author_facet Deest, Maximilian
Wieting, Jelte
Jakob, Maximilian Michael
Deest-Gaubatz, Stephanie
Groh, Adrian
Seifert, Johanna
Toto, Sermin
Bleich, Stefan
Frieling, Helge
Eberlein, Christian K.
author_sort Deest, Maximilian
collection PubMed
description BACKGROUND: Prader–Willi syndrome (PWS) is a rare neurodevelopmental disorder based on a loss of paternally expressed genes in chromosome segment 15q11-13. Behavioral traits such as temper outbursts, stereotypic, and ritualistic behavior, as well as an increased risk of psychosis accompany the syndrome, representing a major issue in the treatment of adults with PWS. Up to now, no treatment guideline for these conditions in PWS exist. This study aimed to retrospectively analyze the effect and adverse effects of treatment with aripiprazole for temper outbursts in 10 adults with PWS. RESULTS: Aripiprazole was prescribed for temper outbursts (n = 10). Treatment outcome was assessed using the Clinical Global Impression-Severity (CGI-S) and -Improvement Scale (CGI-I). Treatment success (CGI-I < 3) was observed in 70% of cases, with adverse effects from mild to partly serious extent in 60% of cases. The major adverse effect observed was increased daytime sleepiness. In total, 50% of the individuals were treated successfully for temper outbursts. The BMI did not change significantly in the successfully treated group after 6 months of treatment. CONCLUSIONS: Aripiprazole can be a treatment option for temper outbursts in people with PWS. Although a high rate of side effects was detected, their severity led to discontinuation in only 20% of the cases. Furthermore, the absence of weight gain makes aripiprazole interesting especially for the PWS population.
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spelling pubmed-94193142022-08-28 Aripiprazole treatment for temper outbursts in Prader–Willi syndrome Deest, Maximilian Wieting, Jelte Jakob, Maximilian Michael Deest-Gaubatz, Stephanie Groh, Adrian Seifert, Johanna Toto, Sermin Bleich, Stefan Frieling, Helge Eberlein, Christian K. Orphanet J Rare Dis Research BACKGROUND: Prader–Willi syndrome (PWS) is a rare neurodevelopmental disorder based on a loss of paternally expressed genes in chromosome segment 15q11-13. Behavioral traits such as temper outbursts, stereotypic, and ritualistic behavior, as well as an increased risk of psychosis accompany the syndrome, representing a major issue in the treatment of adults with PWS. Up to now, no treatment guideline for these conditions in PWS exist. This study aimed to retrospectively analyze the effect and adverse effects of treatment with aripiprazole for temper outbursts in 10 adults with PWS. RESULTS: Aripiprazole was prescribed for temper outbursts (n = 10). Treatment outcome was assessed using the Clinical Global Impression-Severity (CGI-S) and -Improvement Scale (CGI-I). Treatment success (CGI-I < 3) was observed in 70% of cases, with adverse effects from mild to partly serious extent in 60% of cases. The major adverse effect observed was increased daytime sleepiness. In total, 50% of the individuals were treated successfully for temper outbursts. The BMI did not change significantly in the successfully treated group after 6 months of treatment. CONCLUSIONS: Aripiprazole can be a treatment option for temper outbursts in people with PWS. Although a high rate of side effects was detected, their severity led to discontinuation in only 20% of the cases. Furthermore, the absence of weight gain makes aripiprazole interesting especially for the PWS population. BioMed Central 2022-08-26 /pmc/articles/PMC9419314/ /pubmed/36028863 http://dx.doi.org/10.1186/s13023-022-02470-y Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Deest, Maximilian
Wieting, Jelte
Jakob, Maximilian Michael
Deest-Gaubatz, Stephanie
Groh, Adrian
Seifert, Johanna
Toto, Sermin
Bleich, Stefan
Frieling, Helge
Eberlein, Christian K.
Aripiprazole treatment for temper outbursts in Prader–Willi syndrome
title Aripiprazole treatment for temper outbursts in Prader–Willi syndrome
title_full Aripiprazole treatment for temper outbursts in Prader–Willi syndrome
title_fullStr Aripiprazole treatment for temper outbursts in Prader–Willi syndrome
title_full_unstemmed Aripiprazole treatment for temper outbursts in Prader–Willi syndrome
title_short Aripiprazole treatment for temper outbursts in Prader–Willi syndrome
title_sort aripiprazole treatment for temper outbursts in prader–willi syndrome
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9419314/
https://www.ncbi.nlm.nih.gov/pubmed/36028863
http://dx.doi.org/10.1186/s13023-022-02470-y
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