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

Volumn 5, Issue 4, 1999, Pages 305-313

  Hagerman, Randi J ^a,b  

^a CHILDREN S HOSPITAL   (United States)

^b Child Development Unit B140   (United States)

Author keywords

ADHD; Angelman syndrome; Fetal alcohol syndrome; Fragile X syndrome; Mental retardation; Prader Willi syndrome; Psychopharmacology; Smith Magenis syndrome; Stimulants; Velocardiofacial syndrome; Williams syndrome

Indexed keywords

AMANTADINE; AMPHETAMINE; BECLOMETASONE DIPROPIONATE; CARBAMAZEPINE; CATECHOL METHYLTRANSFERASE; CENTRAL STIMULANT AGENT; CLONIDINE; DESIPRAMINE; DEXAMPHETAMINE; DEXFENFLURAMINE; FLUOXETINE; FOLIC ACID; GABAPENTIN; GUANFACINE; IMIPRAMINE; LITHIUM; METHYLPHENIDATE; PEMOLINE MAGNESIUM; PSYCHOTROPIC AGENT; RISPERIDONE; SERTRALINE; TIAGABINE; VALPROIC ACID;

AFFECTIVE NEUROSIS; AGGRESSION; ATTENTION DEFICIT DISORDER; FETAL ALCOHOL SYNDROME; FRAGILE X SYNDROME; HAPPY PUPPET SYNDROME; HUMAN; NONHUMAN; PHENOTYPE; PRADER WILLI SYNDROME; PRIORITY JOURNAL; PSYCHOPHARMACOLOGY; REVIEW; SKIN IRRITATION; SMITH MAGENIS SYNDROME; SOMNOLENCE; VELOCARDIOFACIAL SYNDROME; VERTIGO; WELLBEING;

EID: 0033373434     PISSN: 10804013     EISSN: None     Source Type: Journal    
DOI: 10.1002/(SICI)1098-2779(1999)5:4<305::AID-MRDD8>3.0.CO;2-L     Document Type: Review

References (116)

1. Aman CJ, Roberts RJ Jr, Pennington BF. 1998. A neuropsychological examination of the underlying deficit in attention deficit hyperactivity disorder: frontal lobe versus right parietal lobe theories. Dev Psychol 34:956-969.
2. Aman MG. 1996. Stimulant drugs in the developmental disabilities revisited. J Dev Phys Disabil 8:347-365.
3. Aman MG, Kern RA, McGhee DE, Arnold LE. 1993. Fenfluramine and methylphenidate in children with mental retardation and ADHD: clinical and side effects. J Am Acad Child Adolesc Psychiatry 32:851-859.
4. American Psychiatric Association (APA). 1994. Diagnostic and statistical manual of mental disorders, 4th edition (DSM-IV). Washington, DC: American Psychiatric Association.
5. Angulo MA, Castro-Magana M, Canas JA, et al. 1997. Penile length and sexual maturation in Prader-Willi syndrome (PWS) children on growth hormone (GH) therapy [Abstract]. Am J Med Genet 73:396.
6. Arnold LE, Abikoff HB, Cantwell DP, et al. 1997. National Institute of Mental Health Collaborative Multimodal Treatment Study of Children with ADHD (the MTA). Design challenges and choices. Arch Gen Psychiatry 54:865-870.
7. Barkley RA. 1997. Attention-deficit/hyperactivity disorder, self-regulation, and time: toward a more comprehensive theory. J Dev Behav Pediatr 18:271-279.
8. Barkley RA, DuPaul GJ, McMurray MB. 1991. Attention deficit disorder with and without hyperactivity: clinical response to three dose levels of methylphenidate. Pediatrics 87:519-531.
9. Beardsmore A, Dorman T, Cooper SA, et al. 1998. Affective psychosis and Prader-Willi syndrome. J Intellect Disabil Res 42:463-471.
10. Benjamin E, Buot-Smith T. 1993. Naltrexone and fluoxetine in Prader-Willi syndrome. J Am Acad Child Adolesc Psychiatry 32:870-873.
11. Brodie MJ, Dichter MA. 1996. Antiepileptic drugs. N Engl J Med 334:168-175.
12. Burger J, Buiting KBD, Grob S, et al. 1997. Different mechanisms and recurrence risks of imprinting defects in Angelman syndrome. Am J Hum Genet 61:88-93.
13. Cantwell DP, Swanson J, Connor DF. 1997. Case study: adverse response to clonidine. J Am Acad Child Adolesc Psychiatry 36:539-544.
14. Carlson C, Papolos D, Pandita RK, et al. 1997. Molecular analysis of velo-cardio-facial syndrome patients with psychiatric disorders. Am J Hum Genet 60:851-859.
15. Carlson CL, Pelham WE Jr, Milich R, et al. 1992. Single and combined effects of methylphenidate and behavior therapy on the classroom performance of children with attention-deficit hyperactivity disorder. J Abnorm Child Psychol 20:213-232.
16. Cassidy SB. 1995. Genetics of Prader-Willi syndrome. In: Greenswag LR, Alexander RC, editors. Management of Prader-Willi syndrome. New York: Springer-Verlag. p 18-31.
17. Clarren SK, Astley SJ, Bowden DM, et al. 1990. Neuroanatomic and neurochemical abnormalities in nonhuman primate infants exposed to weekly doses of ethanol during gestation. Alcohol Clin Exp Res 14:674-683.
18. Coe A, Sidders J, Riley K, et al. 1999. A survey of medication responses in children and adolescents with fetal alcohol syndrome. Submitted.
19. Comery TA, Harris JB, Willems PJ, et al. 1997. Abnormal dendritic spines in fragile X knockout mouse: maturation and pruning deficits. Proc Natl Acad Sci USA 94:5401-5404.
20. Connolly HM, Crary JL, McGoon MD, et al. 1997. Valvular heart disease associated with fenfluramine-phentermine. N Engl J Med 337: 581-588.
21. Crumley FE. 1998. Smith-Magenis syndrome. J Am Acad Child Adolesc Psychiatry 37:1131-1132.
22. Dech B, Budow L. 1991. The use of fluoxetine in an adolescent with Prader-Willi syndrome. J Am Acad Child Adolesc Psychiatry 30:298-302.
23. DeLong GR, Teague LA, McSwain Kamran M. 1998. Effects of fluoxetine treatment in young children with idiopathic autism. Dev Med Child Neurol 40:551-62.
24. Dykens EM, Cassidy SB. 1996. Prader-Willi syndrome: genetic, behavioral, and treatment issues. Ment Retard 5:1056-4993.
25. Efron D, Jarman F, Barker M. 1997. Side effects of methylphenidate and dexamphetamine in children with attention deficit hyperactivity disorder: a double-blind, crossover trial. Pediatrics 100:662-666.
26. Elia J, Ambrosini PJ, Rapoport JL. 1999. Treatment of attention-deficit-hyperactivity disorder. N Engl J Med 340:780-788.
27. Famy C, Streissguth AP, Unis AS. 1998. Mental illness in adults with fetal alcohol syndrome or fetal alcohol effects. Am J Psychiatry 155:552-554.
28. Franke P, Leboyer M, Gansicke M, et al. 1998. Genotype-phenotype relationship in female carriers of the premutation and full mutation of FMR-1. Psychiatry Res 80:113-127.
29. Freeman MP, Stoll AL. 1998. Mood stabilizer combinations: a review of safety and efficacy. Am J Psychiatry 155:12-21.
30. Gadow KD, Nolan E, Sprafkin J, et al. 1995. School observations of children with attention-deficit hyperactivity disorder and comorbid tic disorder: effects of methylphenidate treatment. J Dev Behav Pediatr 16:167-176.
31. Gillberg C, Melander H, von Knorring AL, et al. 1997. Long-term stimulant treatment of children with attention-deficit hyperactivity disorder symptoms. A randomized, double-blind, placebo-controlled trial. Arch Gen Psychiatry 54:857-864.
32. Gillberg C, Aman MG, Reiss A. 1998. Fenfluramine. In: Reiss S, Aman MG, editors. Psychtropic medications and developmental disabilities: the international consensus handbook. Columbus, OH: Nisonger Center, Ohio State University. p 303-309.
33. Glenn CC, Saitoh S, Jong MT, et al. 1996. Gene structure, DNA methylation, and imprinted expression of the human SNRPN gene. Am J Hum Genet 58:335-346.
34. Goldman LS, Genel M, Bezman RJ, et al. 1998. Diagnosis and treatment of attention-deficit/ hyperactivity disorder in children and adolescents. JAMA 279:1100-1107.
35. Greenberg F, Guzzetta V, Montes de Oca-Luna R, et al. 1991. Molecular analysis of the Smith-Magenis syndrome: a possible contiguous-gene syndrome associated with del(17)(p11.2). Am J Hum Genet 49:1207-1218.
36. Greenberg F, Lewis RA, Lorraine P, et al. 1996. Multi-disciplinary clinical study of Smith-Magenis syndrome (deletion 17p11.2). Am J Med Genet 62: 247-254.
37. Gualtieri CT. 1992. Psychopharmacology and the fragile X syndrome. In: Hagerman RJ, McKenzie P, editors. 1992 International Fragile X Conference Proceedings. Dillon, CO: National Fragile X Foundation and Spectra Publishing. p 167-178.
38. Guerrini R, De Lorey TM, Bonanni P, et al. 1996. Cortical myoclonus in Angelman syndrome. Ann Neurol 40:39-48.
39. Hagerman RJ. 1996a. Medical follow-up and pharmacotherapy. In: Hagerman RJ, Cronister A, editors. Fragile X syndrome: diagnosis, treatment, and research. Baltimore: The Johns Hopkins University Press. p 283-331.
40. Hagerman RJ. 1996b. Physical and behavioral phenotype. In: Hagerman RJ, Cronister A, editors. Fragile X syndrome: diagnosis, treatment and research, 2nd edition. Baltimore: The Johns Hopkins University Press. p 3-87.
41. Hagerman RJ. 1999a. 22q Deletion syndromes. In: Neurodevelopmental disorders: diagnosis and treatment. New York: Oxford University Press. p 291-312.
42. Hagerman RJ. 1999b. Angelman syndrome and Prader Willi syndrome. In: Neurodevelopmental disorders: diagnosis and treatment. New York: Oxford University Press. p 243-290.
43. Hagerman RJ. 1999c. Fetal alcohol syndrome. In: Neurodevelopmental disorders: diagnosis and treatment. New York: Oxford University Press. p 3-59.
44. Hagerman RJ. 1999d. Fragile X syndrome. In: Neurodevelopmental disorders: diagnosis and treatment. New York: Oxford University Press. p 61-132.
45. Hagerman RJ. 1999e. Sex chromosome aneuploidy in males. In: Neurodevelopmental disorders: diagnosis and treatment. New York: Oxford University Press. p 173-206.
46. Hagerman RJ. 1999f. Smith-Magenis syndrome. In: Neurodevelopmental disorders: diagnosis and treatment. New York: Oxford University Press. p 341-363.
47. Hagerman RJ, Murphy MA, Wittenberger MD. 1988. A controlled trial of stimulant medication in children with the fragile X syndrome. Am J Med Genet 30:377-392.
48. Hagerman RJ, Fulton MJ, Leaman A, et al. 1994. A survey of fluoxetine therapy in fragile X syndrome. Dev Brain Dysfunct 7:155-164.
49. Hagerman RJ, Bregman JD, Tirosh E. 1998. Clonidine. In: Reiss S, Aman MG, editors. Psychotropic medication and developmental disabilities: the international consensus handbook. Columbus, OH: The Ohio State University Nisonger Center. p 259-269.
50. Hagerman RJ, Hills J, Scharfenaker S, et al. 1999. Fragile X syndrome and selective mutism. Am J Med Genet 83:313-317.
51. Handen BL, Feldman H, Gosling A, et al. 1991. Adverse side effects of methylphenidate among mentally retarded children with ADHD. J Am Acad Child Adolesc Psychiatry 30: 241-245.
52. Handen BL, Janosky J, McAuliffe S, et al. 1994. Prediction of response to methylphenidate among children with ADHD and mental retardation. J Am Acad Child Adolesc Psychiatry 33:1185-1193.
53. Hannigan JH, Randall S. 1996. Behavioral pharmacology in animals exposed prenatally to alcohol. In: Abel EL, editor. Fetal alcohol syndrome: from mechanism to prevention. New York: CRC Press. p 191-213.
54. Harris JC. 1998. Advances in genetics, behavioral phenotypes, pharmacotherapy, health and aging for mentally retarded persons. Curr Opin Psychiatry 11:503-505.
55. Hellings JA, Warnock JK. 1994. Self-injurious behavior and serotonin in Prader-Willi syndrome. Psychopharmacol Bull 30:245-250.
56. Hellings JC, Gaffney GR. 1992. A trigger for Tourette's syndrome [letter]. J Am Acad Child Adolesc Psychiatry 31:371-372.
57. Hilton DK, Martin CA, Heffron WM, et al. 1991. Imipramine treatment of ADHD in a fragile X child. J Am Acad Child Adolesc Psychiatry 30:831-834.
58. Hutchinson T. 1997. Seizures - diagnosis and treatment. Angelman Syndrome Foundation International Conference, Seattle, WA.
59. Inui A, Uemoto M, Takamiya S, et al. 1997. A case of Prader-Willi syndrome with long term mazindol treatment. Arch Intern Med 157: 464.
60. Itoh M, Koeda T, Ohno K, et al. 1995. Effects of mazindol in two patients with Prader-Willi syndrome. Pediatr Neurol 13:349-351.
61. Jan JE, O'Donnell ME. 1996. Use of melatonin in the treatment of paediatric sleep disorders. J Pineal Res 21:193-199.
62. Jay P, Rougeulle C, Massacrier A, et al. 1997. The human necdin gene, NDN, is maternally imprinted and located in the Prader-Willi syndrome chromosomal region. Nat Genet 17:357-361.
63. Kapur S, Remington G. 1996. Serotonin-dopamine interaction and its relevance to schizophrenia. Am J Psychiatry 153:466-476.
64. Keck PE, McElroy SL, Strakowski SM. 1998. Anticonvulsants and antipsychotics in the treatment of bipolar disorder. J Clin Psychiatry 59(suppl 6):74-81.
65. Kishino T, Lalande M, Wagstaff J. 1997. UBE3A/ E6-AP mutations cause Angelman syndrome. Nat Genet 15: 70-73.
66. Kuslich CD, Kobori JA, Mohapatra G, et al. 1999. Prader-Willi syndrome is caused by disruption of the SNRPN gene. Am J Hum Genet 64:70-76.
67. Lachiewicz AM, Dawson DV. 1994. Behavior problems of young girls with fragile X syndrome: factor scores on the Conners' Parent's Questionnaire. Am J Med Genet 51:364-369.
68. Lachman HM, Morrow B, Shprintzen R, et al. 1996. Association of codon 108/158 catechol-O-methyltransferase gene polymorphism with the psychiatric manifestations of velo-cardio-facial syndrome. Am J Med Genet 67:468-472.
69. Lachman HM, Kelsoe JR, Remick RA, et al. 1997. Linkage studies suggest a possible locus for bipolar disorder near the velo-cardio-facial syndrome region on chromosome 22. Am J Med Genet (Neuropsychiatr Genet) 74:121-128.
70. Lindgren AC, Hagenas L, Muller J, et al. 1998. Growth hormone treatment of children with Prader-Willi syndrome affects linear growth and body composition favourably. Acta Paediatr 87:28-31.
71. Maier SE, Miller JA, Blackwell JM, et al. 1999. Fetal alcohol exposure and temporal vulnerability: regional differences in cell loss as a function of the timing of binge-like alcohol exposure during brain development. Alcohol Clin Exp Res 23:726-734.
72. Manteuffel MD. 1996. Neurotransmitter function: changes associated with in utero alcohol exposure. In: Abel EL, editor. Fetal alcohol syndrome: from mechanism to prevention. New York: CRC Press. p 171-189.
73. Mattson SN, Riley EP. 1995. Prenatal exposure to alcohol - what the images reveal. Alcohol Health Res World Imaging Alcohol Res 19:273-278.
74. Mattson SN, Riley EP, Sowell ER, et al. 1996. A decrease in the size of the basal ganglia in children with fetal alcohol syndrome. Alcohol Clin Exp Res 20:1088-1093.
75. Mattson SN, Riley EP, Gramling L, et al. 1997. Heavy prenatal alcohol exposure with or without physical features of the fetal alcohol syndrome leads to IQ deficits. J Pediatr 131:718-721.
76. Mayes SD, Crites DL, Bixler EO, et al. 1994. Methylphenidate and ADHD: influence of age, IQ and neurodevelopmental status. Dev Med Child Neurol 36:1099-1107.
77. McCandless SE, Scott JA, Robin NA. 1998. Deletion 22q11: a newly recognized cause of behavioral and psychiatric disorders. Arch Pediatr Adolesc Med 152:481-484.
78. Miller LJ, McIntosh DN, McGrath J, et al. 1999. Electrodermal responses to sensory stimuli in individuals with fragile X syndrome: a preliminary report. Am J Med Genet 83:268-279.
79. Oesterheld JR, Kofoed L, Tervo R, et al. 1998. Effectiveness of methylphenidate in Native American children with fetal alcohol syndrome and attention deficit/hyperactivity disorder: a controlled pilot study. J Child Adolesc Psychopharmacol 8:39-48.
80. O'Malley K, Hagerman RJ. 1998. Developing clinical practice guidelines for pharmacological interventions with alcohol-affected children. Intervening with children affected by prenatal alcohol exposure, special focus session: September 10-11, 1998; Proceedings available from National Institute on Alcohol Abuse and Alcoholism, Bethesda, MD.
81. Pelham WE Jr, Wheeler T, Chronis A. 1998. Empirically supported psychosocial treatments for attention deficit hyperactivity disorder. J Clin Child Psychol 27:190-205.
82. Pelham WE, Aronoff HR, Midlam JK, et al. 1999. A comparison of Ritalin and Adderall: efficacy and time-course in children with attention-deficit/hyperactivity disorder. Pediatrics 103: e43.
83. Potacki L, Reiter RJ, Glaze D, et al. 1997. Twenty-four hour urinary excretion of 6-sulphatoxymelatonin in Smith-Magenis syndrome. American College of Medical Genetics Conference A31.
84. Pulver AE, Nestadt G, Goldberg R, et al. 1994. Psychotic illness in patients diagnosed with velo-cardio-facial syndrome and their relatives. J Nerv Ment Dis 182:476-478.
85. Rosenbaum M, Leibel RL, Hirsch J. 1997. Obesity. N Engl J Med 337:396-407.
86. Rosh JR, Dellert SF, Narkewicz M, et al. 1998. Four cases of severe hepatotoxicity associated with pemoline: possible autoimmune pathogenesis. Pediatrics 101:921-923.
87. Rougeulle C, Glatt H, Lalande M. 1997. The Angelman syndrome candidate gene, UBE3A/ E6-AP, is imprinted in brain. Nat Genet 17:14-15.
88. Sampson PD, Streissguth AP, Bookstein FL, et al. 1997. Incidence of fetal alcohol syndrome and prevalence of alcohol-related neurodevelopmental disorder. Teratology 56:317-326.
89. Shprintzen RJ, Goldberg RB, Lewin ML, et al. 1978. A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: velo-cardio-facial syndrome. Cleft Palate J 15:56-62.
90. Shprintzen RJ, Goldberg R, Golding-Kushner KJ, et al. 1992. Late-onset psychosis in the velo-cardio-facial syndrome. Am J Med Genet 42:141-142.
91. Smith ACM, Gropman A. 1999. Smith-Magenis syndrome. In: Cassidy S, Allanson J, editors. Clinical management of common genetic syndromes. New York: John Wiley.
92. Smith ACM, McGavran L, Waldstein G. 1982. Deletion of the 17 short arm in 2 patients with facial clefts. Am J Hum Genet 34:410A.
93. Smith ACM, McGavran L, Robinson J, et al. 1986. Interstitial deletion of (17) (p11.2p11.2) in nine patients. Am J Med Genet 24:393-414.
94. Smith ACM, Dykens E, Greenberg F. 1998a. The behavioral phenotype of Smith-Magenis syndrome (del 17p11.2). Am J Med Genet 81:179-185.
95. Smith ACM, Dykens E, Greenberg F. 1998b. Sleep disturbance in Smith-Magenis syndrome (del 17p11.2). Am J Med Genet 81:186-191.
96. Snyder J, Nanson J, Snyder RE, et al. 1997. Stimulant efficacy in children with FAS. In: Stein DJ, Keating J, Zar HJ, et al. 1994. A survey of the phenomenology and pharmacotherapy of compulsive and impulsive-aggressive symptoms in Prader-Willi syndrome. J Neuropsychiatry Clin Neurosci 6:23-29.
97. Stratton KR, Howe CJ, Battaglia FC. 1996. Fetal alcohol syndrome: diagnosis, epidemiology, prevention and treatment. Washington, DC: National Academy Press.
98. Streissguth A, Kanter J, editors. The challenge of fetal alcohol syndrome: overcoming secondary disabilities. Seattle: University of Washington Press.
99. Streissguth AP. 1997. Fetal alcohol syndrome: a guide for families and communities. Baltimore: Brooks Publishing.
100. Streissguth AP, Barr HM, Kogan J, 1996. Understanding the occurrence of secondary disabilities in clients with fetal alcohol syndrome (FAS) and fetal alcohol effects (FAE). Seattle: University of Washington School of Medicine.
101. Summers JA, Lynch PS, Harris JC, et al. 1992. A combined behavioral/pharmacological treatment of sleep-wake schedule disorder in Angelman syndrome. J Dev Behav Pediatr 13:284-287.
102. Swanson JM, Flockhart D, Udrea, et al. 1995. Clonidine and the treatment of ADHD: questions about safety and efficacy. J Child Adolesc Psychopharmacol 5:301-304.
103. Swanson J, Wigal S, Greenhill L, et al. 1998a. Objective and subjective measures of the pharmacodynamic effects of Adderall in the treatment of children with ADHD in a controlled laboratory classroom setting. Psychopharmacol Bull 34:55-60.
104. Swanson JM, Wigal S, Greenhill LL, et al. 1998b. Analog classroom assessment of Adderall in children with ADHD. J Am Acad Child Adolesc Psychiatry 37:19-526.
105. Szymanski LS, Munir KM, Wiedhoff S. 1998. Psychiatric and behavior disorders in mental retardation syndromes. Curr Opin Psychiatry 11:515-518.
106. Thomas JA, Graham JM Jr. 1997. Chromosomes 22q11 deletion syndrome: an update and review for the primary pediatrician. Clin Pediatr 36:253-266.
107. Tu J-B, Hartridge C, Izawa J. 1992. Pharmacogenetic aspects of Prader-Willi syndrome. J Am Acad Child Adolesc Psychiatry 31:1137-1140.
108. Turk J. 1992. Fragile X syndrome and folic acid. In: International Fragile X Conference Proceedings. Dillon, CO: The National Fragile X Foundation and Spectra Publishing.
109. Verhoeven WMA, Curfs LMG, Tuinier S. 1998. Prader-Willi syndrome and cycloid psychoses. J Intellect Disabil Res 42:455-462.
110. Warnock JK, Kestenbaum T. 1992. Pharmacologic treatment of severe skin-picking behaviors in Prader-Willi syndrome. Arch Dermatol 128: 1623-1625.
111. Waslick BD, Walsh BT, Greenhill LL, et al. 1999. Cardiovascular effects of desipramine in children and adults during exercise testing. J Am Acad Child Adolesc Psychiatry 38:179-186.
112. Wilens TE, Spencer TJ, Swanson JM, et al. 1999. Combining methylphenidate and clonidine: a clinically sound medication option. J Am Acad Child Psychiatry 38:614-619; discussion 619-622.
113. Williams CA, Zori RT, Hendrickson J, et al. 1995. Angelman syndrome. Curr Probl Pediatr 25:216-231.
114. Zametkin AJ, Ernst M. 1999. Problems in the management of attention-deficit-hyperactivity disorder. N Engl J Med 340:40-46.
115. Zhdanova IV, Lynch HJ, Wurtman RJ. 1997. Melatonin: a sleep-promoting hormone. Sleep 20:899-907.
116. Zori RT, Boyar FZ, Williams WN, et al. 1998. Prevalence of 22q11 region deletions in patients with velopharyngeal insufficiency. Am J Med Genet 77:8-11.