Neurology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Perry, T. L.
Right arrow Articles by MacLeod, P. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Perry, T. L.
Right arrow Articles by MacLeod, P. M.
NEUROLOGY 1980;30:772
© 1980 American Academy of Neurology

Failure of aminooxyacetic acid therapy in Huntington disease

Thomas L. Perry, M.D., James M. Wright, M.D., F. R. C. P.(C), Shirley Hansen, B.A., Barbara M. Allan, M. D., F. R. C. P. (C), Patricia A. Baird, M. D., F. R. C. P. (C) and Patrick M. MacLeod, M. D., F. R. C. P. (C)

Departments of Pharmacology (Drs. Perry, Wright, and Hansen), Medicine (Dr. Allan), and Medical Genetics (Drs. Baird and MacLeod), University of British Columbia, Vancouver, Canada.

Seven patients with Huntington disease were treated with aminooxyacetic acid (AOAA), an inhibitor of µgM-aminobutyric acid aminotransferase (GABA-T), in an effort to alleviate symptoms by increasing brain GABA content. AOAA was given orally in a placebo-controlled crossover trial in which patients, relatives, and three of the evaluating physicians remained blind. Toxic symptoms occurred in all seven patients when AOAA dosage was increased beyond 2 mg per kilogram per day, and included drowsiness, ataxia, seizures, and psychotic behavior. In five patients who took AOAA for 4 months, no clinical improvement was observed. Biochemical monitoring showed that less inhibition of hepatic GABA-T enzyme activity was achieved than in patients treated with large doses of isoniazid. Results of this trial neither support nor exclude the possible therapeutic usefulness of increasing brain GABA content in Huntington disease.

Address correspondence and reprint requests to Dr. Perry, Department of Pharmacology, University of British Columbia, Vancouver, BC, Canada V6T 1W5.

Accepted for publication October 11, 1979.






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 1980 by AAN Enterprises, Inc.