Children with Williams syndrome frequently present with symptoms of attention deficit hyperactivity disorder (ADHD), but there is little information that stimulant medication is useful in this population. A series of double-blind, placebo-controlled case studies was used to evaluate the cognitive and behavioral effects of methylphenidate on four children with Williams syndrome. Teachers and mothers completed behavioral rating scales and cognitive tests of attention, learning and memory, and academic productivity and accuracy in mathematics in each medication condition. Two of the children responded favorably in terms of decreased impulsivity, decreased irritability, and lower activity level as well as improved ability to pay attention. Methylphenidate is a useful adjunct in the treatment of some children with Williams syndrome. (J Child Neurol 1997;12:248—252).
Jones KL, Smith DW: The Williams elfin facies syndrome: A new perspective. J Pediatr1975;86:718-723.
3.
Curran ME, Atkinson DL, Ewart AK, et al: The elastin gene is disrupted by a translocation associated with supravalvular aortic stenosis. Cell1993 ;73:159-168.
4.
Bennett FC, LaVeck B., Sells CJ: The Williams elfin facies syndrome: The psychological profile as an aid in syndrome identification. Pediatrics1978;61:303-306.
5.
Udwin O., Yule W., Martin N.: Cognitive abilities and behavioural characteristics of children with idiopathic infantile hypercalcaemia. J Child Psychol Psychiatry1987;28:297-309.
6.
MacDonald GW , Roy DL: Williams syndrome: A neuropsychological profile. J Clin Exp Neuropsychol1988;10:125—131.
7.
Udwin O., Yule W.: A cognitive and behavioural phenotype in Williams syndrome. J Clin Exp Neuropsychol1991;13:232—244.
8.
Crisco JJ, Dobbs JM, Mulhern RK: Cognitive processing of children with Williams syndrome. Dev Med Child Neurol1988;30: 650-656.
9.
Wang PL, Bellugi V.: Williams syndrome, Down syndrome, and cognitive neuroscience. Am J Dis Child1993;147:1246—1251.
10.
Morris CA, Demsey SA, Lenard CO, et al: Natural history of Williams syndrome: Physical characteristics . J Pediatr1988;113: 318-326.
11.
Handen BL, Breaux AM, Gosling A., et al: Efficacy of methylphenidate among mentally retarded children with attention deficit hyperactivity disorder. Pediatrics1990;86:922-930.
12.
Aman MG, Marks RE, Turbott SH, et al: Clinical effects of methylphenidate and thioridazine in intellectually subaverage children. J Am Acad Child Adolesc Psychiatry1991;30:246—256.
13.
Goyette CH, Conners CK, Ulrich RF: Normative data on revised Conners Parent and Teacher Rating Scales . J Abnorm Child Psychol1978;6:221-236.
14.
Achenbach TM : The Child Behavior Profile: 1. Boys aged 6-11 . J Consult Clin Psychol1978;46:478—488.
15.
American Psychiatric Association, Committee on Nomenclature and Statistics: Diagnostic and Statistical Manual of Mental Disorders, 3rd ed, revised. Washington, DC, American Psychiatric Association , 1987.
16.
Swanson JM: Side Effects Questionnaire. Toronto, Author, 1978.
17.
Rosvold HE, Mirsky AF, Sarason I., et al: A continuous-performance test of brain damage. J Consult Psychol1956;20:343—352.
Swanson JM, Kinsbourne M.: The cognitive effects of stimulant drugs on hyperactive children, in Hale GA, Lewis M (eds): Attention and Cognitive Development. New York, Plenum, 1979, pp 249-274.
20.
Wechsler D.: Manual for the Wechsler Intelligence Scale for Children-Revised. New York, Psychological Corporation, 1974.
21.
Firestone P. : Factors associated with children's adherence to stimulant medication. Am J Orthopsychiatry1982;52:447-457.
22.
Johnston C. , Fine S.: Methods of evaluating methylphenidate in children with attention deficit hyperactivity disorder: Acceptability, satisfaction, and compliance. J Pediatr Psychol1993;18:717—730.
23.
Handen BL, Feldman H., Gosling A., et al: Adverse side effects of methylphenidate among mentally retarded children with ADHD. J Am Acad Child Adolesc Psychiatry1991;30:241-245.
24.
Rapport MD, Stoner G., DuPaul GJ, et al: Methylphenidate in hyperactive children: Differential effects of dose on academic, learning and social behavior. J Abnorm Child Psychol1985;13: 227-244.
25.
Anderson PE, Rourke BP: Williams syndrome, in Rourke BP (ed): Syndrome of Nonverbal Learning Disabilities: Neurodevelopmental Manifestations. New York, Guilford Press, 1995.
26.
Rourke BP: Treatment program for the child with NLD, in Rourke BP (ed): Syndrome of Nonverbal Learning Disabilities: Neurodevelopmental Manifestations. New York, Guilford Press, 1995.
27.
Udwin O., Yule W.: Infantile Hypercalcaemia and Williams Syndrome: Guidelines for Parents . London, Bell Press, 1989.
28.
Levine K.: Williams Syndrome: Information For Teachers. Claw-son, MO, Williams Syndrome Association, 1994.
