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Observations About Amyotrophic Lateral Sclerosis and the Parkinsonism-Dementia Complex of Guam with Regard to Epidemiology and Etiology

Published online by Cambridge University Press:  05 January 2016

John C. Steele  and
Tomasa Guzman
John C. Steele*
Affiliation:
Veterans Administration Office, U.S. Naval Hospital, Guam
Tomasa Guzman
Affiliation:
Veterans Administration Office, U.S. Naval Hospital, Guam
*
Veterans Administration Office, U.S. Naval Hospital, P.O. Box 7613, F.P.O., San Francisco, CA., U.S.A. 96630
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Abstract:

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For more than 150 years, Chamorro natives of the Mariana Islands in the Western Pacific Ocean, have developed fatal paralysis in middle and later life, which we term amyotrophic lateral sclerosis/parkinsonism-dementia (ALS/PD). The cause of the disease might be exposure to seeds of the indigenous cycad. Motor system disease is induced in cynomolgus monkeys by feeding them beta-N-methylamino-L-alanine (BMAA), an amino acid present in cycad seeds. We believe that the cycad seeds which usually cause no immediate adverse symptoms when prepared and eaten as flour, or applied topically as medicine, can give rise to widespread and severe nerve cell degeneration after a latency of many decades. Furthermore, it may be that only a single exposure to this potent but silent toxin(s) can result in fatal neurological disease years later.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 14 , Issue S3 , August 1987 , pp. 358 - 362
Copyright
Copyright © Canadian Neurological Sciences Federation 1987

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