Orphanet Journal of Rare Diseases (Aug 2022)

Aripiprazole treatment for temper outbursts in Prader–Willi syndrome

  • Maximilian Deest,
  • Jelte Wieting,
  • Maximilian Michael Jakob,
  • Stephanie Deest-Gaubatz,
  • Adrian Groh,
  • Johanna Seifert,
  • Sermin Toto,
  • Stefan Bleich,
  • Helge Frieling,
  • Christian K. Eberlein

DOI
https://doi.org/10.1186/s13023-022-02470-y
Journal volume & issue
Vol. 17, no. 1
pp. 1 – 8

Abstract

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Abstract 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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