Parkinsonism-dementia of Guam

Perl DP, Gajdusek DC, Garruto RM, et al. 1982. Intraneuronal aluminum accumulation in amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam. Science 217 1053-1055. 

Other Pulmonary fibrosis, bronchopneumonia, anemia, osteodystrophy, amyotrophic lateral sclerosis and parkinsonism-dementia of Guam, macrophagic myofasciitis... 

Duncan, M.W., Steele, J.C., Kopin, I.J. and Markey, S.P. (1990) 2-Amino-3-(methylamino)-propanoic acid (BMAA) in cycad flour an unlikely cause of amyotrophic lateral sclerosis and parkinsonism-dementia of Guam. Neurology 40 767-772. 

Garruto RM, Fukatsu R, Yanagihara R, Gajdusek DC, Hook G Fiori CE. (1983). Imaging of calcium and aluminum in neurofibrillary tangle-bearing neurons in parkinsonism-dementia of Guam. Proc Natl Acad Sci USA 81, 1875-1879. 

AD is a primary degenerative dementia affecting humans as young as in their forties. The German physician Alois Alzheimer first described the disease in 1906. It is characterized by senile plaques and paired helical filaments (PHFs), and the severity of the condition directly parallels their number.63 The involvement of aluminum in this and related dementias (dialysis encephalopathy and amyotrophic lateral sclerosis — Parkinson dementia in Guam) is currently a highly contested issue in neurological research. 

Garruto, R. M., Yanagihara, R. and Gajdusek D. C. (1985) Disappearance of high-incidence amyotrophic lateral sclerosis and parkinsonism-dementia on Guam. Neurology. 35 193-198. 

Ince R G. and Codd G. A. (2005). Return of the cycad hypothesis-does the amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC) of Guam have new implications for global health Neuropathol. Appl. Neurobiol. 31 345-353. 

Masliah E., Alford M., Galasko D., Salmon D., Hansen L. A., Good R F., Perl D. P., and Thai L. (2001). Cholinergic deficits in the brains of patients with parkinsonism-dementia complex of Guam. NeuroReport 12 3901-3903. 

Winton M. J., Joyce S., Zhukareva V., Practico D., Perl D. P., Galasko D., Craig U., Trojanowski J. Q., and Lee V. M. (2006). Characterization of tau pathologies in gray and white matter of Guam parkinsonism-dementia complex. Acta Neuropathol. (Berl) 111 401 112. 

Elizan, T. S., Hirano, A., Abrams, B. M., Need, R. L., Van Nuis, C., et al. (1966) Amyotrophic lateral sclerosis and parkinsonism-dementia complex of Guam. Neurological reevaluation. Arch Neurol 14, 356-368. 

Crapper MacLachlan D. R., MacLachlan C. D., Krishnan B., Krishnan S. S., Dalton A. J., and Steele J. C. (1989) Aluminum and calcium in soil and food from Guam, Palau, and Jamaica implications for amyotrophic lateral sclerosis and parkinsonism-dementia syndromes of Guam. Environ. Geochem. Health 11, 45-53. 

Perl D. P. (1997) Amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam. In Neuropathology of Dementia... 

The Chamorro people of Guam have a high incidence of a syndrome that resembles amyotrophic lateral sclerosis (ALS) with elements of Parkinson s disease and dementia. This syndrome developed during World War II when, as a result of food shortages, the tribe ate large quantities... 

Kato, S., Hirano, A., Liena, J.F. and Yen, S.-H. (1992) Ultra-struetural identifieation of neurofibrillary tangles in the spinal cord in guamanian amyotrophic lateral selerosis and parkinsonism-dementia eomplex on Guam. Acta Neuropa-thol. 83 277-282. 

Zang, Z.X., Anderson, D.W. and Mantel, N. (1990) Geographic, patterns of parkinsonism-dementia complex on Guam. Arch. Neurol. 47 1069-1074. 

Amyotrophic lateral sclerosis-Parkinsonism-dementia complex of guam... 

Although BMAA has been an instructive agent, it is unclear whether it is the principal toxin in cy-cad plants (Kisby et al., 1992). Studies using cycad extracts found no correlation between BMAA content and toxicity in cultured neurons. However, the cycad samples had a high content of Zn +, and Zn + produced a dose-dependent damage of neurons that parallelled the Zn + content of the cycad extracts (Duncan et al., 1992). It is possible that a complex mechanism involving Zn +, BMAA and perhaps other cycad toxins contributes to the ALS-parkinsonism-dementia complex of Guam. 

Among the Guam population, the disease known locally as lytico-bodighSiS been common for hundreds of years (Kurland, 1972). The disease is one of paralysis, tremors, and dementia and is an amyotrophic lateral sclerosis-parkinsonism... 

Certain individuals on Guam and other South Sea Islands exhibit an increased incidence of amyotrophic lateral sclerosis (ALS), parkinsonism and dementia. The absence of inheritable or transmissible factors in this syndrome led to a search for environmental causes and the identification of the false sago palm Cycas circinalis) as a likely source of the causative agent. This plant contains a number of potential neurotoxins including -N-methylamino-L-alanine (BMAA), an agent with weak excitotoxic properties (Spencer et al., 1990). 

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