Severe hypoxia and multiple infarctions resembling Creutzfeldt-Jakob disease

Michel Mittelbronn*, David Capper, Benedikt Bader, Jens Schittenhelm, Johannes Haybaeck, Petra Weber, Richard Meyermann, Hans A. Kretzschmar, Horst Wiethölter

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

3 Citations (Scopus)


Although neuropathological examination is still required for the definite diagnosis of Creutzfeldt-Jakob disease (CJD), specialised clinical assessment predicts probable CJD. Here we present a 73-year-old female patient presenting with rapid cognitive decline, visual, acoustic and cerebellar disturbances, ataxia and EEG changes compatible with early CJD stages. MRI revealed hyperintensities within the thalami, hypothalami, corpora mammillaria, the tectum and the cortex. Initial neuropathological examination showed severe cortical and subcortical spongiosis. However, both immunohistochemistry and Western blotting showed no pathological prion protein. Finally small infarctions affecting the tectum, tegmentum, corpora mammillaria and global hypoxic-ischaemic changes could be identified as the probable reason for the changes interpreted as CJD-related pathology. Hypoxic-ischaemic CNS alterations mainly affecting the supply area of the basilar artery should be ruled out in case of probable CJD. In addition, severe spongiosis can be misleading in the histological examination, suggesting the diagnosis of a prion-induced spongiform encephalopathy.

Original languageEnglish
Pages (from-to)149-153
Number of pages5
JournalFolia Neuropathologica
Issue number2
Publication statusPublished - 2008
Externally publishedYes


  • Creutzfeldt-Jakob disease
  • Hypoxia
  • Infarction
  • Mesencephalon
  • Prion


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