Individuals with Prader-Willi Syndrome (PWS) have been described by parents and clinicians as having difficulty with regulation of sensory stimuli from the environment. It is suspected that this problem may be related to other symptoms of PWS such as poor satiety recognition, decreased sensitivity to pain, tendency to self-injure and sleep issues. However, there are no research studies that have evaluated sensory functioning in PWS.
Our overarching research question is: Do individuals with PWS have signs and symptoms of Sensory Processing Disorder?
Our proposed project includes three aims:
Aim 1) describing sensory behaviors using parent interview and parent-report scales.
Aim 2) describing the autonomic nervous system function in reaction to sensory stimuli.
Aim 3) studies the relation between behavioral and physiologic measures of Sensory Processing.
We propose to evaluate 30 individuals, ages 5-21. Fifteen with PWS will be recruited through our local PWS chapter, our state chapter and nearby states if necessary. In addition, we will recruit 15 typically developing individuals who will be age and gender matched to the individuals with PWS.
All participants will complete a) the Short Sensory Profile, a parent or self-report of sensory stimulation that affects daily functioning and a detailed interview and b) our psychophysiology laboratory paradigm, called the Sensory Challenge Protocol. This protocol has been well-studied with individuals who have a variety of developmental disabilities. The information derived from the Sensory Challenge Protocol provides detailed information about the functioning of the autonomic nervous system, one part of the brain used for regulation.
Achieving these aims will help further the goals of the Foundation for Prader-Willi Research by establishing a better understanding of the underlying brain mechanisms in PWS. In addition this study may suggest new therapeutic interventions that may be effective for individuals with PWS.
Lucy Jane Miller, Ph.D., OTR