Long term treatment of Huntington disease with L-glutamate and pyridoxine.
Decreased levels of gamma aminobutyric acid (GABA) and its synthetic enzyme, glutamic acid decarboxylase, have been found in the brains of patients with Huntington disease. In an attempt to augment GABA-mediated neurotransmission, daily doses of 25 gm of L-glutamate (the substrate for glutamic acid decarboxylase) and 500 mg of pyridoxine, its cofactor, were given to five patients with Huntington disease. This regimen was continued for 2 years. Assessment of motor and behavioral function indicated no improvement on this regimen.
D000328:Adult; D001921:Brain / Q000378:metabolism; D005260:Female; D005971:Glutamates / Q000627:therapeutic use*; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy* / Q000378:metabolism; D008134:Long-Term Care; D008297:Male; D008875:Middle Aged; D011736:Pyridoxine / Q000627:therapeutic use*; D005680:gamma-Aminobutyric Acid / Q000378:metabolism
D005971:Glutamates; D005680:gamma-Aminobutyric Acid; D011736:Pyridoxine