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May 20, 2008; 70 (21) Views and Reviews

The ALS/PDC syndrome of Guam and the cycad hypothesis

John C. Steele, Patrick L. McGeer
First published May 19, 2008, DOI: https://doi.org/10.1212/01.wnl.0000312571.81091.26
John C. Steele
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Patrick L. McGeer
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The ALS/PDC syndrome of Guam and the cycad hypothesis
John C. Steele, Patrick L. McGeer
Neurology May 2008, 70 (21) 1984-1990; DOI: 10.1212/01.wnl.0000312571.81091.26

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Abstract

There is a high incidence on Guam of a severe tauopathy known as the Parkinson- dementia complex (PDC). It is linked with an even more malignant amyotrophic lateral sclerosis (ALS) syndrome. There is great interest in determining the cause, or causes, of the Guam ALS/PDC syndrome because insight might be gained regarding ALS and the more common tauopathies found throughout the world. Research into the disorder is stimulated by hypotheses as to cause. Such hypotheses should be compatible with the known epidemiology and pathology of the syndrome. These include a high, if not exclusive, restriction to the Chamorro population, familial occurrence, a regional variation on Guam itself, a definite persistence but with declining incidence, and a possible duplication in isolated villages on the Kii peninsula of Japan. Proposed causation factors should also be able to reproduce the syndrome in experimental systems. This includes induction of neurofibrillary tangles with a tau isoform distribution similar to that of Alzheimer disease and association of the lesions with TDP-43 and Lrrk2. A recurring hypothesis as to causation is exposure to Cycas micronesica, the false Sago palm known locally as fadang. We review the reasons why this hypothesis falls short of the minimal criteria needed for further serious consideration and discuss some other possibilities that should not be excluded.

GLOSSARY: AD = Alzheimer disease; ALS = amyotrophic lateral sclerosis; FTLD-U = frontotemporal lobar degeneration with ubiquitinated inclusions; Lrrk2 = leucine-rich-repeat-kinase 2; MAPKKK = microtubule associated protein kinase kinase kinase; NFT = neurofibrillary tangle; PDC = Parkinson-dementia complex; PSP = progressive supranuclear palsy; SNPs = single nucleotide polymorphisms; TAR = transactive response; TDP-43 = TAR-DNA-binding protein 43.

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Disputes & Debates: Rapid online correspondence

  • The ALS/PDC syndrome of Guam and the cycad hypothesis
    • Amy R. Borenstein, University of South Florida, COPH, Epi and Biostat, 13201 Bruce B Downs Blvd., Tampa, FL 33612aborenst@health.usf.edu
    • James A. Mortimer, Gerard D. Schellenberg ( Philadelphia, PA) , Douglas Galasko ( San Diego, CA.)
    Submitted July 25, 2008
  • The ALS/PDC syndrome of Guam and the cycad hypothesis
    • Walter G. Bradley, Department of Neurology, Miller School of Medicine, University of Miami, CRB 1315, 1120 NW 14th Street, Miami, FL 33136wbradley@med.miami.edu
    • Sandra Anne Banack, Paul Alan Cox
    Submitted July 25, 2008
  • The ALS/PDC syndrome of Guam and the cycad hypothesis
    • Reyniel Cruz-Aguado, University of British Columbia, 386-828 W.10th Ave., Vancouver, BC, V5Z1L8, Canadareynielc@yahoo.com
    • Christopher A. Shaw
    Submitted July 25, 2008
  • The ALS/PDC syndrome of Guam and the cycad hypothesis
    • Peter S. Spencer, CROET/Oregon Health & Science University (OHSU), 3181 SW Sam Jackson Park Rd,, Portland, OR 97239spencer@ohsu.edu
    • Valerie Palmer and Glen Kisby
    Submitted July 25, 2008
  • Reply from the authors
    • Patrick L. McGeer, University of British Columbia, Kinsmen Laboratory of Neurological Research, 2255 Wesbrook Mall, Vancouver, BC Canada V6T1Z3mcgeerpl@interchange.ubc.ca
    • John C. Steele
    Submitted July 25, 2008
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  • Article
    • Abstract
    • EPIDEMIOLOGIC ASPECTS
    • PATHOLOGIC ASPECTS
    • GENETIC ASPECTS
    • ENVIRONMENTAL FACTORS
    • THE CYCAD HYPOTHESIS
    • Footnotes
    • REFERENCES
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  • Alzheimer's disease
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