Ultrastructural study of florid plaques in variant Creutzfeldt-Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease.

Abstract

BACKGROUND:

Although the histological features of the amyloid plaques in variant Creutzfeldt-Jakob disease (vCJD) are distinct from those in other forms of prion disease [kuru, sporadic Creutzfeldt-Jakob disease (sCJD) and Gerstmann-Sträussler-Scheinker disease (GSS)], their ultrastructural features have only been described in a single case report.

AIMS:

To study vCJD plaques systematically and compare them with plaques in kuru, sCJD, GSS and Alzheimer disease (AD).

METHODS:

Amyloid plaques were studied by transmission electron microscopy and image analysis in five cases of vCJD, three cases of GSS, two cases of sCJD, one case of kuru and five cases of AD. Immunohistochemistry was performed on paraffin sections from one case of vCJD, two cases of GSS, one case of kuru and two cases of sCJD.

RESULTS:

The florid plaques in vCJD were either compact or more diffuse; in both forms, the radiating fibrils were organized into thick 'tongues', in contrast to kuru plaques. Dystrophic neurites (DNs) containing lysosomal electron-dense bodies or vesicles surrounded florid plaques. Microglial cells were found within florid plaques; occasional amyloid fibrils were identified in membrane-bound pockets of microglial cells. In vCJD, there was significant tau immunoreactivity in DNs around florid plaques while, in sCJD, GSS and kuru, minimal tau immunoreactivity was observed around plaques.

CONCLUSIONS:

The ultrastructure of the florid plaques and DNs in vCJD is more reminiscent of neuritic plaques in AD than kuru or multicentric plaques. These findings may reflect differences both in the strains of the transmissible agents responsible for these disorders and in host factors.

• Authors: B Sikorska, P P Liberski, T Sobów, H Budka, J W Ironside
• Journal: Neuropathology and applied neurobiology (Neuropathol Appl Neurobiol) Vol. 35 Issue 1 Pg. 46-59 (Feb 2009) ISSN: 1365-2990 [Electronic] England
• PMID: 18513219 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Amyloid
• Glial Fibrillary Acidic Protein
• Ubiquitin
• tau Proteins

Topics

• Adolescent
• Adult
• Alzheimer Disease (metabolism, pathology)
• Amyloid (analysis)
• Brain (ultrastructure)
• Brain Chemistry
• Creutzfeldt-Jakob Syndrome (metabolism, pathology)
• Endosomes (ultrastructure)
• Female
• Gerstmann-Straussler-Scheinker Disease (metabolism, pathology)
• Glial Fibrillary Acidic Protein (analysis)
• Gliosis (pathology)
• Humans
• Kuru (pathology)
• Male
• Microglia (chemistry, ultrastructure)
• Neurons (chemistry, ultrastructure)
• Plaque, Amyloid (ultrastructure)
• Ubiquitin (analysis)
• tau Proteins (analysis, metabolism)