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Treatment of Huntington disease with -acetylenic GABA, an irreversible inhibitor of GABA- transaminase

Increased CSF GABA and homocarnosine without clinical amelioration

From the Centre de Recherche Merrell International (Drs. Tell, Bohlen, Schechter, and Koch-Weser), Strasbourg, Hôpital de le Salpètrière (Drs. Agid and Bonnet), Clinique de Neurologie et de Neuropsychologie (Prof. Lhermitte), Paris, Hôpital Civil (Dr. Coquillat), Clinique Neurologique (Prof. Rohmer), Strasbourg, and Hôpltal Neurologique (Drs. Chazot and Fischer), Service de Neurologie (Professor Schott), Lyon, France.

-Acetylenic GABA (GAG, RMI 71.645), a potent irreversible inhibitor of -aminobutyric acid transaminase, was given orally in various dosage schedules to 14 patients with Huntington disease. The biochemical effects of the drug on cerebrospinal fluid (CSF) concentrations of -aminobutyric acid (GABA) and the GABA-containing dipeptide, homocarnosine, were measured in 10 of 14 patients. Treatment with GAG increased CSF concentrations of GABA and homocarnosine as compared to pretreatment values, suggesting that the drug increased brain GABA concentration. Despite this neurochemical effect, the clinical state was not improved. Except for single seizure episodes in five patients, GAG therapy was well tolerated. These results do not exclude the possibility that agents that augment CNS GABAergic function may prove useful in therapy of Huntington disease.

Address correspondence and reprint requests to Dr. Schechter, Director of Clinical Research, Centre de Recherche Merrell International, 16 rue d'Ankara, 67084 Strasbourg Cedex, France.

Accepted for publication April 21, 1980.