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Chapter 9 - Agitation and Aggression in Prader-Willi Syndrome

Published online by Cambridge University Press:  26 May 2022

Deepan Singh
Affiliation:
Maimonides Medical Center in Brooklyn
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Summary

Agitation and aggression are common behavioral manifestations in patients with PWS. This chapter reviews how to recognize possible triggers and psychological mechanisms behind them. Caregivers are made aware of operant conditioning and the stages of moral development. Additionally the role of externalization is described. The underlying cause of the agitation can vary from person to person and similarly the management strategy differs considerably. The importance of ensuring the safety of the patient and others is emphasized. Behavioral management techniques can be very effective when started at an early age. Medications are effective options to supplement behavioral strategies as long as they are prescribed under the close monitoring of a medical provider. Finally, remember that most patients with PWS do not have malicious intent when they are exhibiting aggression. Their aggression is rooted in poor impulse control and hence is reactive in nature. Their aggression should be treated as a symptom rather than an inherent character flaw.

Type
Chapter
Information
Neuro-behavioral Manifestations of Prader-Willi Syndrome
A Guide for Clinicians and Caregivers
, pp. 77 - 89
Publisher: Cambridge University Press
Print publication year: 2022

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References

Powis, L, Oliver, C. The prevalence of aggression in genetic syndromes: A review. Res Dev Disabil 2014;35(5):1051–71.CrossRefGoogle ScholarPubMed
Widom, CS. Does violence beget violence? A critical examination of the literature. Psychol Bull 1989;106(1):328.Google Scholar
Yeager, CA, Lewis, DO. Mental illness, neuropsychologic deficits, child abuse, and violence. Child Adolesc Psychiatr Clin N Am 2000;9(4):793813.Google Scholar
Gillett, ES, Perez, IA. Disorders of sleep and ventilatory control in Prader-Willi syndrome. Diseases 2016;4(3):23. doi: 10.3390/diseases4030023CrossRefGoogle ScholarPubMed
Merk, W, Orobio de Castro, B, Koops, W, Matthys, W. The distinction between reactive and proactive aggression: Utility for theory, diagnosis and treatment? European Journal of Developmental Psychology 2005;2(2):197220.Google Scholar
Urben, S, Habersaat, S, Pihet, S, Suter, M, de Ridder, J, Stéphan, P. Specific contributions of age of onset, callous-unemotional traits and impulsivity to reactive and proactive aggression in youths with conduct disorders. Psychiatr Q 2018;89(1):110.CrossRefGoogle ScholarPubMed
Skinner, BF. Operant behavior. American Psychologist 1963;18(8):503–15.Google Scholar
Kohlberg, L. The Philosophy of Moral Development: Moral Stages and the Idea of Justice. First edition. San Francisco: Harper & Row, 1981.Google Scholar
Parent, J, McKee, LG, Anton, M, Gonzalez, M, Jones, DJ, Forehand, R. Mindfulness in parenting and coparenting. Mindfulness (NY) 2016;7(2):504–13.Google Scholar
Kay, SR, Wolkenfeld, F, Murrill, LM. Profiles of aggression among psychiatric patients. I. Nature and prevalence. J Nerv Ment Dis 1988;176(9):539–46.CrossRefGoogle ScholarPubMed
Repple, J, Pawliczek, CM, Voss, B, Siegel, S, Schneider, F, Kohn, N, et al. From provocation to aggression: the neural network. BMC Neurosci 2017;18(1):73. Available at https://rdcu.be/cDzVsGoogle Scholar
Manning, KE, Tait, R, Suckling, J, Holland, AJ. Grey matter volume and cortical structure in Prader-Willi syndrome compared to typically developing young adults. Neuroimage Clin 2018;17:899909.Google Scholar
Singh, D, Wakimoto, Y, Filangieri, C, Pinkhasov, A, Angulo, M. Guanfacine extended release for the reduction of aggression, attention-deficit/hyperactivity disorder symptoms, and self-injurious behavior in Prader-Willi syndrome: A retrospective cohort study. J Child Adolesc Psychopharmacol 2019;29(4):313–17.Google Scholar
Fernández de la Cruz, L, Simonoff, E, McGough, JJ, Halperin, JM, Arnold, LE, Stringaris, A. Treatment of children with attention-deficit/hyperactivity disorder (ADHD) and irritability: Results from the multimodal treatment study of children with ADHD (MTA). J Am Acad Child Adolesc Psychiatry 2015;54(1):62–70.e3.CrossRefGoogle ScholarPubMed
Deest, M, Jakob, MM, Seifert, J, Bleich, S, Frieling, H, Eberlein, C. Sertraline as a treatment option for temper outbursts in Prader-Willi syndrome. Am J Med Genet A 2021;185(3):790–7.Google Scholar
Peet, M. Induction of mania with selective serotonin re-uptake inhibitors and tricyclic antidepressants. Br J Psychiatry 1994;164(4):549–50.Google Scholar
Manning, KE, Beresford-Webb, JA, Aman, LCS, Ring, HA, Watson, PC, Porges, SW, et al. Transcutaneous vagus nerve stimulation (t-VNS): A novel effective treatment for temper outbursts in adults with Prader-Willi syndrome indicated by results from a non-blind study. PLoS ONE 2019;14(12):e0223750.Google Scholar
Knani, I, Earley, BJ, Udi, S, Nemirovski, A, Hadar, R, Gammal, A, et al. Targeting the endocannabinoid/CB1 receptor system for treating obesity in Prader-Willi syndrome. Mol Metab 2016;5(12):1187–99.CrossRefGoogle ScholarPubMed
Kendrick, JG, Goldman, RD, Carr, RR. Pharmacologic management of agitation and aggression in a pediatric emergency department: A retrospective cohort study. J Pediatr Pharmacol Ther 2018;23(6):455–9.Google Scholar
Kang, M, Galuska, MA, Ghassemzadeh, S. Benzodiazepine Toxicity. Treasure Island, FL: StatPearls, 2021.Google Scholar

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