title: Serotonin-GABA treatment is hypothesized for self-injury in Lesch-Nyhan syndrome.
abstract: The self-injurious movements of Lesch-Nyhan patients are considered extremely severe and currently intractable. Lesch-Nyhan syndrome is a genetic disorder of purine metabolism resulting in several abnormalities, one of which is elevated levels of xanthine. The author reasons that elevated plasma xanthine sets off a chain of events that produce excessive endogenous convulsants and diminished endogenous anticonvulsants. Treatment is proposed for self-injury in Lesch-Nyhan that entails reducing production of two endogenous convulsants (kynurenine and quinolinic acid) and enhancing two endogenous anticonvulsants (serotonin and gamma aminobutyric acid).
authors: A
authors: A
authors: Gedye
mesh_terms: D001418:Baclofen / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D006801:Humans; D007737:Kynurenine / Q000037:antagonists & inhibitors / Q000096:biosynthesis; D007926:Lesch-Nyhan Syndrome / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D008297:Male; D016728:Self-Injurious Behavior / Q000188:drug therapy* / Q000209:etiology; D012701:Serotonin / Q000008:administration & dosage* / Q000378:metabolism; D005680:gamma-Aminobutyric Acid / Q000378:metabolism
chemical_list: D012701:Serotonin; D007737:Kynurenine; D005680:gamma-Aminobutyric Acid; D001418:Baclofen