[Neuropathological findings in early-onset Alzheimer's disease (E280a-PS1 mutation)]

Rev Neurol. 1999 Jul;29(1):1-6.
[Article in Spanish]

Abstract

Introduction and material: Nine brains belonging to early onset Alzheimer disease (E280A-PS1 mutation) affected individuals from Antioquia, Colombia, were analyzed by neuropathological standard techniques. All individuals were ascertained from genealogies descendents from a common ancestor that shows a dominant autosomical pattern of inheritance.

Results: All cases analyzed were carriers to the E280A-PS1 mutation. This type of mutation produce beta-amyloid deposits of 42 aminoacids in the CNS. The mean of onset age was 48.4 years with an average of evolution time of 7.55 years and a mean of death age of 56.55. Although, all the cases showed symmetrical atrophy and them was more severe in the hippocampal region, a definitive anterior pattern (temporo-frontal) was showed. The higher the time of evolution of disease the lower the brain weight.

Conclusions: All types of senile plaques and abundant neurofibrillary tanggles were found. In the stem, similar lesions were found but they were in lower number. Only the mesencephalic region showed a significative positive correlation between the number of senile plaques and the number of neurofibrillary tanggels (p < 0.05, r = 0.76). Only the parietal region showed a significant positive correlation between the number of senile plaques and the disease evolution time (p < 0.02, r = 0.74). Particularly, the cerebellum only showed senile plaques but neurofibrillary degeneration was not observed. With the exception of the Hirano bodies, all findings traditionally described were observed.

Publication types

  • English Abstract
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age of Onset
  • Alzheimer Disease / epidemiology
  • Alzheimer Disease / genetics
  • Alzheimer Disease / pathology*
  • Atrophy
  • Brain / pathology*
  • Cerebral Cortex / pathology
  • Colombia / epidemiology
  • Female
  • Genes, Dominant
  • Genetic Predisposition to Disease
  • Hippocampus / pathology
  • Humans
  • Male
  • Membrane Proteins / genetics*
  • Middle Aged
  • Nerve Tissue Proteins / genetics*
  • Neurofibrillary Tangles / ultrastructure
  • Organ Size
  • Plaque, Amyloid / ultrastructure
  • Point Mutation*
  • Presenilin-1
  • Severity of Illness Index

Substances

  • Membrane Proteins
  • Nerve Tissue Proteins
  • PSEN1 protein, human
  • Presenilin-1