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Adaptive and Maladaptive Behavior in Children with Smith-Magenis Syndrome

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Children with Smith-Magenis Syndrome (SMS) exhibit deficits in adaptive behavior but systematic studies using objective measures are lacking. This descriptive study assessed adaptive functioning in 19 children with SMS using the Vineland Adaptive Behavior Scales (VABS). Maladaptive behavior was examined through parent questionnaires and the Childhood Autism Rating Scale. Cognitive functioning was evaluated with an age-appropriate test. Children scored below average on VABS Communication, Daily Living Skills, and Socialization scales. Learning problems and hyperactivity scales on the Conner’s Parent Rating Scale were elevated, and girls were more impulsive than boys. Stereotypic and self-injurious behaviors were present in all children. Cognitive functioning was delayed and consistent with communication and daily living skills, while socialization scores were higher than IQ.

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Acknowledgments

This research was supported by the Intramural Research Program of the National Institutes of Health, the HIV and AIDS Malignancy Branch of the National Cancer Institute through federal contracts 71004-09 and N01-SC-07006 with the Medical Illness Counseling Center, and a Bench-to-Bedside award from the Clinical Center at the NIH to the Medical Genetics Branch of the National Human Genetics Research Institute. The authors would like to express their sincere appreciation to the families who agreed to participate in this research. We also extend our gratitude to members of the SMS Research Team, especially Mary Anne Tamula, M.A., Donna Krasnewich, M.D., Suzan Parada, R.N., and Rebecca Morse, B.A.

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Authors and Affiliations

  1. HIV and AIDS Malignancy Branch, National Cancer Institute, Bethesda, MD, USA

    Staci C. Martin & Pamela L. Wolters

  2. Medical Illness Counseling Center, Chevy Chase, MD, USA

    Staci C. Martin & Pamela L. Wolters

  3. Medical Genetics Branch, National Human Genetics Research Institute, Bethesda, MD, USA

    Ann C. M. Smith

  4. Georgetown University Medical Center, Washington, DC, USA

    Ann C. M. Smith

  5. National Cancer Institute, 9030 Old Georgetown Road, 107, Bethesda, MD, 20892-8200, USA

    Staci C. Martin

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  1. Staci C. Martin
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  2. Pamela L. Wolters
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  3. Ann C. M. Smith
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Correspondence to Staci C. Martin.

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Martin, S., Wolters, P. & Smith, A. Adaptive and Maladaptive Behavior in Children with Smith-Magenis Syndrome. J Autism Dev Disord 36, 541–552 (2006). https://doi.org/10.1007/s10803-006-0093-2

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