Acta Neuropathologica

, Volume 99, Issue 4, pp 358–364 | Cite as

Retrospective study of Creutzfeldt-Jakob disease in Belgium: neuropathological findings

  • B. Van Everbroeck
  • P. Pals
  • T. Dziedzic
  • R. Dom
  • C. Godfraind
  • R. Sciot
  • J. M. Brucher
  • J. J. Martin
  • P. Cras
Regular paper

Abstract

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that affects about 1 in 106 inhabitants in most countries. Recently, a new variant of CJD has been linked to the epidemic of bovine spongiform encephalopathy. Therefore, vigilance concerning the disease’s incidence has been increased. We conducted a comprehensive, nation-wide and retrospective study. In 79 Belgian autopsies, we found the characteristic triad of spongiosis, neuronal loss and reactive gliosis. The occipital cortex was most affected, while the cerebellum was mostly spared. Immunohistochemistry was performed using hydrated autoclave pretreatment and several monoclonal antibodies directed against the prion protein. We identified prion-immunoreactive patterns and locations reflecting the important heterogeneity, independently of the antibody that was used. Granular prion immunoreactivity was observed in astrocytes. We studied the regional intensity of the prion immunostaining and determined that the frontal cortex with 95% positive immunoreactivity was best suited for a biopsy. We studied the disease duration in sporadic CJD patients who showed neuropathological lesions of other neurodegenerative disorders (such as Alzheimer’s disease). The study shapes the framework in which a prospective neuropathological registry will be able to function.

Key words Creutzfeldt-Jakob disease Dementia Spongiosis Neuronal loss Reactive gliosis 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • B. Van Everbroeck
    • 1
  • P. Pals
    • 1
  • T. Dziedzic
    • 1
  • R. Dom
    • 2
  • C. Godfraind
    • 3
  • R. Sciot
    • 2
  • J. M. Brucher
    • 4
  • J. J. Martin
    • 1
  • P. Cras
    • 1
  1. 1.Born Bunge Foundation, Laboratory of Neurobiology, University of Antwerp, Universiteitsplein 1, B-2610 Wilrijk, Belgium e-mail: cras@uia.ua.ac.be, Tel.: +32-3-8213423, Fax: +32-3-8255467BE
  2. 2.Department of Neurology, Catholic University of Leuven, B-3000 Leuven, BelgiumBE
  3. 3.Laboratory of Neuropathology, Université Catholique de Louvain, B-1050 Brussels, BelgiumBE
  4. 4.4 Rue de Beaumont 88, B-1390 Grez-Doiceau/Graven, BelgiumBE

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