Individuals with Smith-Magenis syndrome display profound neurodevelopmental behavioral deficiencies and exhibit food-related behaviors equivalent to Prader-Willi syndrome.
Treat food issues in Smith-Magenis syndrome as aggressively as you do in Prader-Willi syndrome—locks, schedules, and body-size lessons.
01Research in Context
What this study did
The team looked at adults with Smith-Magenis syndrome, a rare genetic condition. They wanted to know if these adults show more behavior and feeding problems than other people with intellectual disability.
They watched each person and counted self-injury, stereotypy, aggression, and food-seeking. Then they compared the counts to typical ID levels and to Prader-Willi syndrome.
What they found
SMS adults had far more self-injury, rocking, hand-flapping, and hits or kicks than the usual ID group. Their food sneaking, stealing, and weight gain looked just as severe as in Prader-Willi syndrome.
How this fits with other research
Goodwin et al. (2012) warns that you must define self-injury the same way every time you measure it. T et al. used clear, repeated counts, so their high SMS numbers are believable.
Martin et al. (1997) showed that bad self-injury can shorten life, mostly through chest infections. The new SMS data say the risk may be even higher in this small group.
Ramos-Jiménez et al. (2014) found that simple waist and arm measures predict metabolic syndrome in ID teens. Pair those quick checks with the SMS feeding results to catch obesity early.
Eden et al. (2017) discovered that young adults with ID often do not know they are overweight. If SMS adults share that body-blindness, standard PWS lock-away-food plans will need extra teaching steps.
Why it matters
You now know SMS can bring Prader-Willi-level food chaos plus worse behavior. Start by guarding food access like you would for PWS: locked cupboards, scheduled meals, calorie counts. Add visual body-size training so clients can link "full" to their own shape. Watch lungs too—track coughs and give prompt chest care, because severe SIB raises respiratory risk.
Want CEUs on This Topic?
The ABA Clubhouse has 60+ free CEUs — live every Wednesday. Ethics, supervision & clinical topics.
Join Free →Put a fridge lock in place and post a visual meal schedule for any client diagnosed with SMS.
02At a glance
03Original abstract
Smith-Magenis syndrome (SMS) is a neurodevelopmental disorder associated with intellectual disability, sleep disturbances, early onset obesity and vast behavioral deficits. We used the Behavior Problems Inventory-01 to categorize the frequency and severity of behavioral abnormalities in a SMS cohort relative to individuals with intellectual disability of heterogeneous etiology. Self-injurious, stereotyped, and aggressive/destructive behavioral scores indicated that both frequency and severity were significantly higher among individuals with SMS relative to those with intellectual disability. Next, we categorized food behaviors in our SMS cohort across age using the Food Related Problems Questionnaire (FRPQ) and found that problems began to occur in SMS children as early as 5-11 years old, but children 12-18 years old and adults manifested the most severe problems. Furthermore, we evaluated the similarities of SMS adult food-related behaviors to those with intellectual disability and found that SMS adults had more severe behavioral problems. Many neurodevelopmental disorders exhibit syndromic obesity including SMS. Prader-Willi syndrome (PWS) is the most frequent neurodevelopmental disorder with syndromic obesity and has a well-established management and treatment plan. Using the FRPQ we found that SMS adults had similar scores relative to PWS adults. Both syndromes manifest weight gain early in development, and the FRPQ scores highlight specific areas in which behavioral similarities exist, including preoccupation with food, impaired satiety, and negative behavioral responses. SMS food-related behavior treatment paradigms are not as refined as PWS, suggesting that current PWS treatments for prevention of obesity may be beneficial for individuals with SMS.
Research in developmental disabilities, 2015 · doi:10.1016/j.ridd.2015.08.011