Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam: quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders

  title={Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam: quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders},
  author={Patrick R. Hof and Esther A. Nimchinsky and Valérie Buée-Scherrer and Luc Bu{\'e}e and Jade Nasrallah and Andreas F. Hottinger and Dushyant P. Purohit and Arthur J. Loerzel and John C.H. Steele and André Delacourte and Constantin Bouras and John H. Morrison and Daniel P. Perl},
  journal={Acta Neuropathologica},
Amyotrophic lateral sclerosis/parkinsonism-dementia complex (lytico-bodig) is a chronic neurodegenerative disorder with high prevalence among the native Chamorro population of Guam. Neuropathological, biochemical, and immunohistochemical analyses were performed on a relatively large series of Guamanian cases and compared to Alzheimer's disease cases. Thioflavin S and antibodies to amyloid βA4 and tau proteins were used for analysis of pathological changes, and antibodies to the calcium-binding… 
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The hypothesis that cycad exposure plays an important role in the etiology of the Guam disease is supported, as existing epidemiological and animal data support the hypothesis.
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Neuropathological evidence for a relationship between ALS and Alzheimer's disease is provided and tissues associated with both diseases regardless of the principal clinical are examined.
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The objective of this report is to analyze in detail the neurological features of Guam ALS and P-D and compare them with classical ALS, Parkinson's disease, and other relevant disorders, and to show the unique relationship between the ALS andP-D syndromes, clinically and histologically.
Parkinsonism-dementia complex, an endemic disease on the island of Guam. I. Clinical features.
The main purpose of this paper is to present the pertinent neuropathological features of the 17 necropsied cases of Parkinsonism with presenile dementia reported here were autopsied.
Relationship of aluminum to Alzheimer's disease.
  • D. Perl
  • Biology
    Environmental health perspectives
  • 1985
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The unique pattern of neuronal degeneration found in ALS motor cortex is consistent with non-N-methyl-Dxxx-aspartate glutamate receptor-mediated cytotoxicity and suggests that cortical degeneration in ALS is distinctive from other neurodegenerative diseases affecting cerebral cortex.
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