Journal of Molecular Neuroscience

, Volume 17, Issue 2, pp 225–232

Lewy body pathology in Alzheimer’s disease

  • Paul T. Kotzbauer
  • John Q. Trojanowski
  • Virginia M.-Y. Lee
Article

DOI: 10.1385/JMN:17:2:225

Cite this article as:
Kotzbauer, P.T., Trojanowski, J.Q. & Lee, V.M. J Mol Neurosci (2001) 17: 225. doi:10.1385/JMN:17:2:225

Abstract

Lewy bodies, the characteristic pathological lesion of substantia nigra neurons in Parkinson’s disease (PD), are frequently observed to accompany the amyloid plaque and neurofibrillary tangle pathology of Alzheimer’s disease (AD). However the typical anatomic distribution of Lewy bodies in AD is distinct from PD. The most common site of occurrence is the amygdala, where Lewy bodies are observed in approximately 60% of both sporadic and familial AD. Other common sites of occurrence include the periamygdaloid and entorhinal cortex, while neocortical and brainstem areas develop Lewy bodies in a lower percentage of cases. In contrast, dementia with Lewy bodies (DLB), defined by widespread neocortical and brainstem Lewy bodies but frequently accompanied by variable levels of AD-type pathology, represents the other end of a spectrum of pathology associated with dementia. The observation of Lewy bodies in familial AD cases suggests that like neurofibrillary tangles, the formation of Lewy bodies can be induced by the pathological state caused by Aβ-amyloid over-production. The role of Lewy body formation in the dysfunction and degeneration of neurons remains unclear. The protein α-synuclein appears to be an important structural component of Lewy bodies, an observation spurred by the discovery of point mutations in the α-synuclein gene linked to rare cases of autosomal dominant PD. Further investigation of α-synuclein and its relationship to pathological conditions promoting Lewy body formation in AD, PD, and DLB may yield further insight into pathogenesis of these diseases.

Index Entries

Lewy bodiesAlzheimer’s diseaseFamilial Alzheimer’s DiseaseParkinson’s diseaseDementia with Lewy bodiesα-synucleinAβ-amyloidAmyloid plaquesNeurofibrillary tanglesTau proteinoxidative stress

Copyright information

© Humana Press Inc 2001

Authors and Affiliations

  • Paul T. Kotzbauer
    • 1
  • John Q. Trojanowski
    • 1
  • Virginia M.-Y. Lee
    • 1
  1. 1.Center for Neurodegenerative Disease ResearchUniversity of Pennsylvania School of MedicinePhiladelphia