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, John Morrison, D. P. Perl

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)397-404
Number of pages8
JournalActa Neuropathologica
Volume88
Issue number5
DOIs
StatePublished - Nov 1 1994
Externally publishedYes

Fingerprint

Amyotrophic Lateral Sclerosis
Neurodegenerative Diseases
Alzheimer Disease
tau Proteins
Neurofibrillary Tangles
Calcium-Binding Proteins
Neocortex
Parkinsonian Disorders
Guam
Calbindin 2
Neurofilament Proteins
Amyloidogenic Proteins
Neurons
Parvalbumins
Intermediate Filaments
Antibodies
Pyramidal Cells
Interneurons
Neuropathology
Population

Keywords

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam : quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders. / Hof, P. R.; Nimchinsky, E. A.; Buée-Scherrer, V.; Buée, L.; Nasrallah, J.; Hottinger, A. F.; Purohit, D. P.; Loerzel, A. J.; Steele, J. C.; Delacourte, A.; Bouras, C.; Morrison, John; Perl, D. P.

In: Acta Neuropathologica, Vol. 88, No. 5, 01.11.1994, p. 397-404.

Research output: Contribution to journalArticle

Hof, PR, Nimchinsky, EA, Buée-Scherrer, V, Buée, L, Nasrallah, J, Hottinger, AF, Purohit, DP, Loerzel, AJ, Steele, JC, Delacourte, A, Bouras, C, Morrison, J & Perl, DP 1994, 'Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam: quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders', Acta Neuropathologica, vol. 88, no. 5, pp. 397-404. https://doi.org/10.1007/BF00389490
Hof, P. R. ; Nimchinsky, E. A. ; Buée-Scherrer, V. ; Buée, L. ; Nasrallah, J. ; Hottinger, A. F. ; Purohit, D. P. ; Loerzel, A. J. ; Steele, J. C. ; Delacourte, A. ; Bouras, C. ; Morrison, John ; Perl, D. P. / Amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam : quantitative neuropathology, immunohistochemical analysis of neuronal vulnerability, and comparison with related neurodegenerative disorders. In: Acta Neuropathologica. 1994 ; Vol. 88, No. 5. pp. 397-404.
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