title: Brain amino compounds in a Huntington's disease patient on isoniazid therapy.
abstract: We describe biochemical abnormalities in autopsied brain of a patient with early Huntington's disease (HD) who died of pentobarbital overdosage while under treatment with isoniazid (INH). The brain contained hydrazine, a terminal metabolite of INH, which inhibits gamma-aminobutyric acid (GABA) aminotransferase. GABA content in the basal ganglia was higher than expected for HD, and GABA content was supranormal in some brain regions. Homocarnosine (GABA-histidine) content was greatly elevated in all brain regions, suggesting chronic GABA elevation in life. Therefore, the increase in brain GABA content observed in experimental animals given INH or hydrazine also occurs in human patients.
authors.forename: T L
authors.initials: TL
authors.lastname: Perry
authors.forename: R A
authors.initials: RA
authors.lastname: Wall
authors.forename: S
authors.initials: S
authors.lastname: Hansen
mesh_terms: D000328:Adult; D001923:Brain Chemistry / Q000187:drug effects*; D002336:Carnosine / Q000031:analogs & derivatives / Q000032:analysis; D002421:Caudate Nucleus / Q000032:analysis; D002529:Cerebellar Nuclei / Q000032:analysis; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy* / Q000378:metabolism; D007538:Isoniazid / Q000378:metabolism / Q000494:pharmacology* / Q000627:therapeutic use; D008297:Male; D011699:Putamen / Q000032:analysis; D013378:Substantia Nigra / Q000032:analysis; D005680:gamma-Aminobutyric Acid / Q000032:analysis* / Q000378:metabolism
chemical_list: C005038:homocarnosine; D005680:gamma-Aminobutyric Acid; D002336:Carnosine; D007538:Isoniazid