New insights into behavior and learning in PWS

Two recent papers address the very important areas of behavior and learning in PWS, and may be helpful in teaching people with PWS how to better cope and learn as they navigate through the world.

The first paper is the work of recent FPWR-grant recipient, Dr. Kate Woodcock.  Dr. Woodcock has been interested in better defining problematic PWS behaviors and understanding their underlying causes.  More importantly, she has taken on the challenge of using that information to develop interventions to minimize the challenging behaviors and improve coping skills. Much of her work has focused on temper outbursts in PWS, and the genetic, neural, and environmental factors that contribute to those outbursts.

As anyone familiar with PWS knows, routine is very important to a person with PWS.  In earlier work [Woodcock, 2010, see also here], Dr. Woodcock and colleagues found that this strong affinity for routine may be related to deficits in a particular kind of higher order thinking - “task switching”.  Brain imaging studies revealed that those with PWS weren’t able to activate and utilize the brain circuits that normally allow a person to switch from one task to another.  This finding may explain why repetitive questioning is so common in PWS (the brain gets “stuck”) and why changes to routine or unmet expectations can trigger temper outbursts.  Dr. Woodcock’s new study explores this problem on a more practical level. Many parents and caregivers have received advice to 'make a routine and stay with it' when dealing with their loved one with PWS. This can be a great help at times - if you can get a good routine going, a person with PWS will stay with it like nobody’s business. But, the new study from Dr. Woodcock’s group looks the potential downside of a well-entrenched routine. In the paper [Increased Exposure to Rigid Routines can Lead to Increased Challenging Behavior Following Changes to Those Routines, full paper here], Dr Woodcock and colleagues engaged people with PWS in games and allowed a routine to be established for a short or longer time period. Then (brave souls that they are!), they broke the routine. They found that the longer and more rigid the routine had been, the worse the consequences when the routine was broken (i.e., longer, more extreme temper outbursts). This finding brings into question the concept that establishing routine is always the best in PWS, and suggests the need to investigate how our kids might be taught to cope with inevitable changes to routine. To this end, Dr. Woodcock’s FPWR-supported project seeks to develop a computer training program to help kids with PWS learn to improve their task switching skills.  Dr. Woodcock is currently recruiting children with PWS between the ages of 8-15 years to participate in this study (see recruiting video).  This group is also conducting related studies on this important topic.  If your child is over 5 years old, please consider participating in a phone interview designed to help researchers better understand how resistance to change develops in PWS.  Or, consider participating in the PREDICTORS project to help test and optimize strategies to reduce temper outbursts in PWS.  More information and links to videos describing all three studies can be found here.

The second area that has recently been investigated looks at how individuals with PWS learn. [Learning by observation and learning by doing in Prader-Willi syndrome]. Dr. Francesca Foti at the Sapienza University of Rome examined the effectiveness of learning by active experience (learning by doing) compared to a different learning approach (learning by observing someone else). The learning task was a computer-based spatial task that involved linking together a sequence of blocks. Strikingly, individuals with PWS had real difficulties when they were asked to learn the sequence by observing someone else perform the task. Not only were they much less able to do this than typically developing children, matched for mental age, but they also did poorly compared to individuals with a different genetic disorder (Williams syndrome), who were matched for IQ (intelligence quotient). The good news is that individuals with PWS were proficient in their ability to learn by doing. In fact, their scores were comparable to the typically developing children when using this mode of learning. The authors suggest that while it is common practice to teach a person something by first showing them what to do, a more effective method for PWS may be to first allow the person to actually perform a task, and then show them how to refine the task that they have just experienced.

Topics: Research

Theresa Strong

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Theresa V. Strong, Ph.D., received a B.S. from Rutgers University and a Ph.D. in Medical Genetics from the University of Alabama at Birmingham (UAB). After postdoctoral studies with Dr. Francis Collins at the University of Michigan, she joined the UAB faculty, leading a research lab focused on gene therapy for cancer and directing UAB’s Vector Production Facility. Theresa is one of the founding members of FPWR and has directed FPWR’s grant program since its inception. In 2016, she transitioned to a full-time position as Director of Research Programs at FPWR. She remains an Adjunct Professor in the Department of Genetics at UAB. She and her husband Jim have four children, including a son with PWS.

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