Individuals with Smith-Magenis syndrome display profound neurodevelopmental behavioral deficiencies and exhibit food-related behaviors equivalent to Prader-Willi syndrome

Research in Developmental Disabilities
Joseph T AlaimoSarah H Elsea

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-...Continue Reading

References

Jun 5, 1998·American Journal of Medical Genetics·A C SmithF Greenberg
Sep 4, 1998·European Journal of Pediatrics·H A WollmannM B Ranke
Feb 25, 1999·Journal of Intellectual Disability Research : JIDR·E M Dykens, A C Smith
Mar 15, 2001·American Journal of Mental Retardation : AJMR·B FinucaneE W Simon
Nov 25, 2003·The British Journal of Clinical Psychology·Helen Russell, Chris Oliver
May 2, 2006·Pediatric Neurology·Andrea L GropmanAnn C M Smith
Mar 14, 2007·Current Opinion in Neurology·Andrea L GropmanAnn C M Smith
Jul 20, 2007·Obesity·Elisabeth M DykensElizabeth Roof
Jan 31, 2008·European Journal of Human Genetics : EJHG·Sarah H Elsea, Santhosh Girirajan
Sep 11, 2008·Journal of Pediatric Endocrinology & Metabolism : JPEM·H SchmidtK Dokoupil
Sep 15, 2009·Pediatric Neurology·Pamela L WoltersAnn C M Smith
May 7, 2011·Expert Reviews in Molecular Medicine·Sarah H Elsea, Stephen R Williams
Oct 20, 2012·Journal of Human Nutrition and Dietetics : the Official Journal of the British Dietetic Association·J L MillerD J Driscoll

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Citations

Sep 13, 2017·Children·Laura Beth Mann DosierZheng Fan
Sep 28, 2018·Obesity Reviews : an Official Journal of the International Association for the Study of Obesity·H ReddonD Meyre
Dec 18, 2018·Current Opinion in Psychiatry·Brian J Shayota, Sarah H Elsea
Jan 28, 2020·Journal of Intellectual Disabilities : JOID·Heidi Elisabeth Nag, Terje Nærland
Dec 29, 2020·American Journal on Intellectual and Developmental Disabilities·Bruno FaconYannick Courbois
Dec 3, 2020·International Journal of Molecular Sciences·Martina ChiurazziAntonio Colantuoni

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