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Neurotransmitter changes in the pathophysiology of Lesch-Nyhan syndrome
1Department of Mental Retardation and Birth Defect Research, National Center of Neurology and Psychiatry (NCNP), Kodaira, Tokyo, Japan.
Brain & Development
|September 14, 2000
Abstract:
The neurological symptoms of Lesch-Nyhan syndrome (LNS) are assumed to result from the neurotransmitter changes in this disorder. Among them, the dopaminergic system is believed to play a role in the self-injurious behavior through receptor supersensitivity. However, the precise mechanism underlying the dopamine supersensitivity remains unclear. An increased serotonergic action in the striatum may be crucial for the appearance of self-injurious behavior, and pharmacological evidence suggests the efficacy of serotonin agonists/antagonists for the treatment of the self-mutilation in LNS.