The aim of this article is to provide an in-depth description of early development in a young child With Smith-Magenis syndrome (SMS). SMS is a multisystem, neurodevelopmental genetic disorder associated With mental retardation that predisposes individuals to a distinct pattern of maladaptive behaviors and other neuropsychological impairments. While there is some research describing outcomes for individuals With SMS in middle childhood, adolescence, and young adulthood, very feW descriptions of early development are available in the literature.
Get full access to this article
View all access options for this article.
References
1.
American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text rev.). Washington, DC: Author.
2.
Chen, K., Potocki, L., & Lupski, J. (1996). The Smith-Magenis syndrome [del(17)p11.2]: Clinical review and molecular advances. Mental Retardation and Developmental Disabilities Research Reviews, 2, 122—129.
3.
Clarke, D.J., & Boer, H. (1998). Problem behaviors associated with deletion Prader-Willi syndrome, Smith-Magenis syndrome, and cri du chat syndromes. American Journal of Mental Retardation, 103, 264—271.
4.
Crumley, F. (1998). Smith-Magenis syndrome. Journal of the American Academy of Child and Adolescent Psychiatry, 37, 1131—1132.
5.
Dunst, C.J. (1988). Strange transitioning in the sensori-motor development of Down's Syndrome infants. Journal of Mental Deficiency Research , 32, 405—410.
6.
Dykens, E.M., Finucane, B., & Gayley, C. (1997). Cognitive and behavioral profiles in persons with Smith-Magenis syndrome. Journal of Autism and Developmental Disorders, 27, 203—211.
7.
Dykens, E.M., & Hodapp, R.M. (2001). Research in mental retardation: Toward an etiologic approach. Journal of Child Psychology and Psychiatry, and Allied Disciplines, 42, 49—71.
8.
Dykens, E.M., & Smith, A.C.M. (1998). Distinctiveness and correlates of maladaptive behaviour in children and adolescents with Smith-Magenis syndrome. Journal of Intellectual Disability Research, 42, 481—489.
9.
Finucane, B., Dirrigl, K.J., & Simon, E.W. (2001). Characterization of self-injurious behaviors in children and adults with Smith-Magenis syndrome. American Journal of Mental Retardation, 106, 52—58.
10.
Finucane, B.M., Jager, E.R., Kurtz, M.B., Weinstein, M., & Scott, C.I. (1993). Eye abnormalities in the Smith-Magenis contiguous gene deletion syndrome. American Journal of Medical Genetics , 45, 443—446.
11.
Finucane, B.M., Konar, D., Haas-Givler, B., & Kurtz, M.B. (1994). The spasmodic upper-body squeeze: A characteristic behavior in Smith-Magenis syndrome. Developmental Medicine and Child Neurology, 36, 78—83.
12.
Finucane, B.M., & Simon, E.W. (1999). Genetics and the dual diagnosis: Smith-Magenis syndrome. The NADDBulletin, 2, 8—10.
13.
Fullard, W., McDevitt, S.C., & Carey, W.B. (1984). Assessing temperament in 1- to 3-year-old children . Journal of Pediatric Psychology, 9, 205—217.
14.
Greenberg, F., Guzzetta, V., & de Oca-Luna, R. (1991). Molecular analysis of the Smith-Magenis syndrome: A possible contiguous-gene syndrome associated with del (17)(p11.2). American Journal of Human Genetics, 49, 1207—1218.
15.
Greenberg, R., Lewis, R.A., Potocki, L., Glaze, D., Parke, J., Killian, J., et al. (1996). Multi-disciplinary clinical study of Smith-Magenis syndrome (deletion 17p11.2). American Journal of Medical Genetics , 62, 247—254.
16.
Hagerman, R.J. (1999). Psychopharmacological interventions in Fragile-X Syndrome, Fetal Alcohol Syndrome, Prader-Willi Syndrome, Angelman Syndrome, Smith-Magenis Syndrome, and Velocardiofacial Syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 5, 305—313.
17.
Hagerman, R.J., Schreiner, R.A., Kemper, M.B., Wittenberger, M.D., Zahn, B., & Habicht, K. (1998). Longitudinal IQ changes in fragile X males. American Journal of Medical Genetics, 33, 513— 518. Hodapp, R.M., Fidler, D. J., & Smith , A.C.M. (1998). Stress and coping in families of children with Smith-Magenis syndrome. Journal of Intellectual Disability Research, 42, 331—340.
18.
Lord, C., Rutter, M., DiLavore, P., & Risi, S. (1999). Autism Diagnostic Observation Schedule—WPS edition. Los Angeles: Western Psychological Services.
19.
Mullen, E. (1995). Mullen Scales of Early Learning. Circle Pines, MN: American Guidance Service.
20.
Repacholi, B.M., & Gopnik, A. (1997). Early reasoning about desires: Evidence from 14- and 18-month-olds. Developmental Psychology, 33, 12—21.
21.
Seibert, J.M., Hogan, A.E., & Mundy, P.C. (1982). Assessing interactional competencies: The Early Social-Communication Scales. Infant Mental Health Journal , 3, 244—258.
22.
Sigman, M.D., Kasari, C., Kwon, J., & Yirmiya, N. (1992). Responses to the negative emotions of others by autistic, mentally retarded, and normal children. Child Development , 63, 796—807.
23.
Smith, A.C.M., Dykens, E., & Greenberg, F. (1998a). Behavioral phenotype of Smith-Magenis syndrome (del 17p11.2). American Journal of Medical Genetics B: Neuropsychiatric Genetics, 81, 179—185.
24.
Smith, A.C.M., Dykens, E., & Greenberg, F. (1998b). Sleep disturbance in Smith-Magenis syndrome. American Journal of Medical Genetics B: Neuropsychiatric Genetics , 81, 186—191.
25.
Smith, A.C.M., McGavran, L., Robinson, J., Waldstein, G., Macfarlane, J., Zonona, A.J., et al. (1986). Interstitial deletion of (17) (p11.2p11.2) in nine patients. American Journal of Human Genetics, 24, 393—414.
26.
Smith, A., McGavran, L., & Waldstein, G. (1982). Deletion of the 17 short arm in two patients with facial clefts. American Journal of Human Genetics, 24, 393—414.
Udwin, O. (2002). Williams and Smith-Magenis syndromes. In P. Howlin & O. Udwin (Eds.), Outcomes in neurodevelopmental and genetic disorders (pp. 299—325). Cambridge: Cambridge University Press.
29.
Udwin, O., & Dennis, J. (1995). Psychological and behavioral phenotypes in genetically determined syndromes: A review of research findings. In G. O'Brien & W. Yule (Eds.), Behavioral phenotypes (pp. 90—208). London: MacKeith Press.
30.
Udwin, O., Webber, C., & Horn, I. (2001). Abilities and attainment in Smith-Magenis syndrome . Developmental Medicine and Child Neurology, 43, 823—828.
31.
Vostanis, P., Harrington, R., Prendergast, M., & Farndon, P. (1994). Case reports of autism with interstitial deletion of chromosome 17 (p11.2.p11.2) and monosomy of chromosome 5 (5pter…5p15.3). PsychiatricGenetics, 4, 109—111.
32.
Willekens, D., De Cock, P., & Fryns, J.P. (2000). Three young children with Smith-Magenis syndrome: Their distinct, recognisable behavioral phenotype as the most important clinical symptoms. Genetic Counseling (Geneva, Switzerland), 11, 103—110.
33.
Zimmerman, I.L., Steiner, V.G., & Pond, R.E. (1992). Preschool Language Scale (4th ed.). San Antonio, TX: Psychological Corp.
34.
Zori, R.T., Lupski, J.R., Heju, Z., Greenberg, F., Killian, J.M., Gray, B.A., et al. (1993). Clinical, cytogenic, and molecular evidence for an infant with Smith-Magenis syndrome born from a mother having a mosaic 17p11.2p12 deletion. American Journal of Medical Genetics , 47, 504—511.