Adaptive and Maladaptive Behavior in Children with Smith-Magenis Syndrome

Abstract

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.

Sensory Motor and Functional Skills of Dizygotic Twins: One with Smith–Magenis Syndrome and a Twin Control

“…An earlier study concluded that children with SMS have difficulty regulating activity level, affect, and bodily functions and that sleep disturbance represented the most significant factor predicting these challenging behaviors (Dykens & Smith, 1998). Additional scientific publications have reported the presence of peculiar movements, stereotypical behaviors, hyperactivity, and sensory-processing difficulties in children with SMS (Dykens, Finucane, & Gayley, 1997;Dykens & Smith, 1998;Finucane, Konar, Haas-Givler, Kurtz, & Scott, 1994;Gropman et al, 2006;Hicks, Ferguson, Bernier, & Lemay, 2008;Martin et al, 2006). Sensory integration disorders have also been included as neurobehavioral problems common to SMS (Gropman et al, 2007;Smith & Gropman, 2005).…”

“…Moderate to severe sleep disturbances characterized by decreased total nighttime sleep, increased nighttime arousals, and daytime sleepiness occurs and has been associated with an unusual inverted circadian melatonin rhythm Gropman, Elsea, Duncan, & Smith, 2007). Early expressive speech delays with and without hearing loss and mild to severe cognitive deficits are observed (Greenberg et al, 1996;Madduri et al, 2006;Martin, Wolters, & Smith, 2006;Udwin & Webber, 2001).…”

“…A variety of psychometric tests to assess adaptive behavior, intelligence quotient (IQ), and speech and language development have documented variable levels of cognitive impairment ranging from severe to borderline/mild intellectual deficits (IQ levels in the range 20-84), depending on the age group studied (Greenberg et al, 1996;Madduri et al, 2006;Martin et al, 2006;Smith & Duncan, 2005;Udwin & Webber, 2001). While sensory processing, gross motor, and fine motor abilities are recognized in this syndrome, systematic study of these domains remains cursory at best.…”

Intellectual and Behavioral Disabilities in Smith — Magenis Syndrome

“…Besides hyperactivity and attention seeking, patients with SMS may present agressive and self-injurious behavior, including hand biting, head banging, face slapping, self-hanging, onychotilomania and polyembolokotonia [16,19,[29][30][31][32][33][34][35]. Other signs reported in up to 50% of patients include obesity, cardiacs defects, seizures, cleft lip/palate and male hypogonadism [22].…”

An Indirect Examination of the Function of Problem Behavior Associated with Fragile X Syndrome and Smith-Magenis Syndrome

“…In comparison to other groups, SMS is associated with relatively high levels of aggression, as well as a range of stereotypical behaviors (Dykens, Finucane, & Gayley, 1997;. Estimates of the prevalence of SIB in SMS vary between 67-96% Finucane, Dirrigl, & Simon, 2001;Greenberg et al, 1996;Martin, Wolters, & Smith, 2006). Some topographies of SIB, such as onychotillomania (pulling out finger-and toenails) and polyembolokomania (insertion of objects into body orifices) appear to be relatively unique to the syndrome (e.g., Finucane, Dirrigl, & Simon, 2001).…”