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

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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2/2008
vol. 46

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abstract:

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

Folia Neuropathol 2008; 46 (2): 149-153

Online publish date: 2008/06/24

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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.

keywords:

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

Creutzfeldt-Jakob disease, hypoxia, infarction, mesencephalon, prion

Michel Mittelbronn

,

David Capper

,

Benedikt Bader

,

Jens Schittenhelm

,

Johannes Haybaeck

,

Petra Weber

,

Richard Meyermann

,

Hans A. Kretzschmar

,

Horst Wiethölter