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Americo Negrette (1924 to 2003)

Diagnosing Huntington disease in Venezuela

From the Departments of Neurology, Neurosurgery, Psychiatry, and History, University of Florida, McKnight Brain Institute, Movement Disorders Center, Gainesville, FL.

Address correspondence and reprint requests to Dr. Michael S. Okun, Movement Disorders Center, University of Florida, Departments of Neurology, Neurosurgery, and Psychiatry, P.O. Box 100236, Gainesville, FL 32610; e-mail: okun{at}neurology.ufl.edu

Objective: To elucidate the role of Dr. Americo Negrette in diagnosing and reclassifying the dancing mania in Maracaibo, Venezuela as Huntington disease (HD).

Methods: All of the medical and nonmedical books and articles by Negrette were collected and reviewed. Personal interviews with Negrette were performed to confirm the details of his life and original work. His childhood, medical education, and contribution to HD, as well as contributions to medicine, art, and poetry, were covered in interview sessions. Excerpts from his autobiography, Ciudad de Fuego, were translated into English for publication. A complete bibliography of 70 books and research articles authored by Negrette was assembled. Negrette himself reviewed the compilation of this manuscript just days before his sudden death on September 14, 2003.

Results: Americo Negrette, a Venezuelan physician, biochemist, artist, and poet, observed a dancing epidemic in 1952. He acquired, through interviews with locals, the knowledge that there were two other small towns along Lake Maracaibo devastated by a syndrome called el mal (the bad). Negrette changed the diagnosis of these patients from a dancing mania to what he believed was Huntington chorea, later termed HD. He presented his findings at the Venezuelan Sixth Congress of Medical Science in 1955. He was met with reluctance in the local scientific community and a passive ear from government authorities. His description, written in Spanish, was not widely distributed beyond Venezuela, and its importance would have to wait until one of his students shared his observations (more than a decade later) with the HD research community and the rest of the world.

Conclusion: The "dancing mania" of Maracaibo, because of the work of Americo Negrette, has been reclassified as Huntington disease.

Received October 28, 2003. Accepted in final form March 17, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the July 27 issue to find the title link for this article.