Acta Neuropathologica

, Volume 88, Issue 5, pp 397–404 | Cite as

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

  • P. R. Hof
  • E. A. Nimchinsky
  • V. Buée-Scherrer
  • L. Buée
  • J. Nasrallah
  • A. F. Hottinger
  • D. P. Purohit
  • A. J. Loerzel
  • J. C. Steele
  • A. Delacourte
  • C. Bouras
  • J. H. Morrison
  • D. P. Perl
Regular Paper


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 proteins parvalbumin and calretinin, and to a nonphosphorylated epitope on neurofilament protein to study select neuronal populations. A differential distribution of neurofibrillary tangles was observed in the neocortex of Guamanian cases compared to Alzheimer's disease cases, with much higher lesion counts in supragranular than in infragranular layers. Also, Guamanian cases with predominant parkinsonism had generally higher neurofibrillary tangle densities than cases with predominant amyotrophic lateral sclerosis. In addition, there was a certain degree of heterogeneity, qualitatively and quantitatively, in the biochemical distribution of tau proteins among Guamanian and Alzheimer's disease cases as revealed by Western blot analysis. Previous studies have suggested that the clinical symptomatology observed in patients suffering from Alzheimer's disease is related to the dramatic loss of specific corticocortically projecting neurons in the neocortex. Interestingly, a subset of neurofilament-rich pyramidal neurons known to be dramatically affected in Alzheimer's disease appears to be resistant in lytico-bodig. Finally, as in Alzheimer's disease, calcium-binding protein-containing interneurons are not affected. These data suggest that the set of projection neurons affected in Guamanian cases may not correspond to those involved in Alzheimer's disease, and that both disorders are characterized by specific patterns of neuronal vulnerability.

Key words

Alzheimer's disease Amyotrophic lateral sclerosis Dementia Guam Parkinson's disease 


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Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • P. R. Hof
    • 1
    • 2
  • E. A. Nimchinsky
    • 1
  • V. Buée-Scherrer
    • 3
  • L. Buée
    • 3
  • J. Nasrallah
    • 4
  • A. F. Hottinger
    • 1
    • 4
  • D. P. Purohit
    • 4
  • A. J. Loerzel
    • 5
  • J. C. Steele
    • 5
    • 6
  • A. Delacourte
    • 2
    • 3
  • C. Bouras
    • 1
    • 7
  • J. H. Morrison
    • 1
    • 2
  • D. P. Perl
    • 1
    • 4
    • 8
  1. 1.Department of NeurobiologyMount Sinai School of MedicineNew YorkUSA
  2. 2.Department of Geriatrics and Adult DevelopmentMount Sinai School of MedicineNew YorkUSA
  3. 3.INSERM Unité 156LilleFrance
  4. 4.Department of PathologyMount Sinai School of MedicineNew YorkUSA
  5. 5.Guam Memorial HospitalAgañaUSA
  6. 6.Micronesian Health SurveyMangilaoUSA
  7. 7.Department of Psychiatry, IUPG Belle-IdéeUniversity of Geneva School of MedicineGenevaSwitzerland
  8. 8.Department of PsychiatryMount Sinai School of MedicineNew YorkUSA

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