Published online by Cambridge University Press: 26 May 2022
This chapter is intended to provide a neurobiologically based framework to understand the behavioral problems seen in PWS. These abnormalities are as a direct result of the loss of function of chromosome 15q11-13. The main brain abnormalities in PWS arise from a dysfunction of areas of higher functioning such as the hypothalamus, insula, orbitofrontal, and anterior cingulate cortices. Additionally, there is abnormal functioning of the serotonin, dopamine, and norepinephrine systems. These dysfunctions collectively lead to the failure of the response monitoring or model-based learning function of the brain. Inadequate or impaired functioning of the response monitoring system in patients with PWS leads to multiple issues including impulsivity and response perseveration. Finally, in place of utilizing the response monitoring system, patients with PWS resort to habit-based learning, which leads to inflexibility. Reminding ourselves of the complex neurobiology that drives our patients and loved ones with PWS might lead to better tolerance for their maladaptive behaviors and more useful management strategies.
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