Educational Staff and Students Wear Orange for PWS Awareness Month

May 7, 2015

PWS FACT 3:

Why does Prader-Willi syndrome occur?

Most cases of PWS are attributed to a spontaneous genetic error that occurs at or near the time of conception for unknown reasons. In a very small percentage of cases (2 percent or less), a genetic mutation that does not affect the parent is passed on to the child, and in these families, more than one child may be affected.  A PWS-like disorder can also be acquired after birth if the hypothalamus portion of the brain becomes damaged through injury or surgery.

PWS FACT 4:

What is the cause of Prader-Willi syndrome?

Basically, the occurrence of PWS is due to lack of several genes on one of an individual’s two chromosome 15’s – the one normally contributed by the father.  In the majority of cases, there is a deletion – the critical genes are somehow lost from the chromosome.  In some of the remaining cases, the entire chromosome from the father is missing and there are instead two chromosome 15’s from the mother (uniparental disomy).  The critical paternal genes lacking in people with PWS have a role in the regulation of appetite.  This is an area of active research in a number of laboratories around the world, since understanding this defect may be very helpful not only to those with PWS, but to understanding obesity in otherwise normal people.

People with PWS have a flaw in the hypothalamus part of their brain, which normally registers feelings of hunger and satiety.  While the problem is not yet fully understood, it is apparent that people with this flaw never feel full; they have a continuous urge to eat that they cannot learn to control.  To compound this problem, people with PWS need less food than their peers without the syndrome because their bodies have less muscle and tend to burn fewer calories.

*Information gathered from the PWSAUA Website.