Recent research on the Lesch-Nyhan syndrome is reviewed. An overview of the etiology and characteristics of L-N children is provided and discussion also focuses on biomedical treatment options, behavior modification, daily management concerns, and educational intervention. Specific attention is given to alternatives for dealing with self-injurious behavior in those individuals affected by the disorder.
Get full access to this article
View all access options for this article.
References
1.
AndersonL., DancisJ., & AlpertM.Behavioral contingencies and self-mutilitation in Lesch-Nyhan disease. Journal of Consulting and Clinical Psychology, 1978, 46, 529–536.
2.
BondyP. K., & RosenbergL. E., (Eds.). Metabolic control and disease. Philadelphia, PA: W. B. Saunders, 1980.
3.
BullM., & LaVecchioF.Behavior therapy for a child with Lesch-Nyhan syndrome. Developmental Medicine and Child Neurology, 1978, 20, 368–375.
4.
CarterC. H.Handbook of mental retardation syndromes (3rd Ed.). Springfield, IL: Charles C. Thomas, 1975.
5.
CastellsS., ChakrabartiC., WinsbergB. G., HurwicM., PerelJ. M., & NyhanW. L.Effects of L-5 hydroxytryptophan on monoamine and amino acids turnover in the Lesch-Nyhan syndrome. Journal of Autism and Developmental Disorders, 1979, 9, 95–103.
6.
DizmangL. H., & CheathamC. F.The Lesch-Nyhan syndrome. American Journal of Psychiatry, 1970, 127, 671–677.
7.
DukerP.Behavior control of self-biting in a Lesch-Nyhan patient. Journal of Mental Deficiency Research, 1975, 19, 11–19.
8.
FernaldC. D.The Lesch-Nyhan syndrome: Cerebral palsy, mental retardation, and self-mutilation. Journal of Pediatric Psychology, 1976, 1(3), 51–55.
9.
GilbertS., SpellacyE., & WattsR. W. E.Problems in the behavioral treatment of self-injury in the Lesch-Nyhan syndrome. Developmental Medicine and Child Neurology, 1979, 21, 795–800.
10.
HoefnagelD.The syndrome of athetoid cerebral palsy, mental deficiency, self-mutilation and hyperuricemia. Journal of Mental Deficiency Research, 1965, 9, 69–74.
11.
HoefnagelD., AndrewE. D., MireaultN. G., & BerndtW. O.Hereditary choreathetosis, self-mutilation, and hyperuricemia in young males. New England Journal of Medicine, 1965, 273, 130–135.
12.
LeschM., & NyhanW. L.A familial disorder of uric acid metabolism and central nervous system function. American Journal of Medicine, 1964, 36, 561–570.
13.
LettsR. M., & HobsonD.A. Special devices as aids in the management of child self-mutilation in the Lesch-Nyhan syndrome. Pediatrics, 1975, 55, 852–855.
14.
LloydK. G., HornykiewiczO., DavidsonL., ShannakK., FarleyI., GoldsteinM., ShibuyaM., KelleyW. N., & FoxI. N.Biochemical evidence of dysfunction of brain neurotransmitters in the Lesch-Nyhan syndrome. New England Journal of Medicine, 1981, 305, 1106–1111.
15.
MenolascinoF. J., & EggerM. L.Medical dimensions of mental retardation. Lincoln, NB: University of Nebraska Press, 1978.
16.
MizunoT. I., & YugariY.Self-mutilation in the Lesch-Nyhan syndrome. Lancet, 1974, 1, 761.
17.
NyhanW. L.Behavior in the Lesch-Nyhan syndrome. Journal of Autism and Childhood Schizophrenia, 1976, 6, 235–252.
18.
NyhanW. L., JohnsonH. G., KaufmanI. A., & JonesK. L.Serotonergic approaches to the modification of behavior in the Lesch-Nyhan syndrome. Applied Research in Mental Retardation, 1980, 1, 25–40.