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Long term treatment of Huntington disease with L-glutamate and pyridoxine

Department of Neurology, University of Illinois at the Medical Center (Dr. Barr), the Department of Psychiatry, University of Illinois (Dr. Heinze), the Department of Neurology, University of Illinois (Drs. Mendoza and Perlik), Chicago, Illinois.

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.

Dr. Barr's address is Department of Neurology, University of Illinois, 912 S. Wood Street, Chicago, IL 60612.

Accepted for publication January 10, 1978.