title: Severe hypoxia and multiple infarctions resembling Creutzfeldt-Jakob disease.
abstract: 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.
authors.affiliation: Institute of Brain Research, University of Tuebingen, Germany. hirnforschung@uni-tuebingen.de
authors.forename: Michel
authors.initials: M
authors.lastname: Mittelbronn
authors.forename: David
authors.initials: D
authors.lastname: Capper
authors.forename: Benedikt
authors.initials: B
authors.lastname: Bader
authors.forename: Jens
authors.initials: J
authors.lastname: Schittenhelm
authors.forename: Johannes
authors.initials: J
authors.lastname: Haybaeck
authors.forename: Petra
authors.initials: P
authors.lastname: Weber
authors.forename: Richard
authors.initials: R
authors.lastname: Meyermann
authors.forename: Hans A
authors.initials: HA
authors.lastname: Kretzschmar
authors.forename: Horst
authors.initials: H
authors.lastname: Wietholter
mesh_terms: D000368:Aged; D001921:Brain / Q000098:blood supply*; D002540:Cerebral Cortex / Q000473:pathology; D002560:Cerebrovascular Circulation / Q000502:physiology; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000473:pathology; D020925:Hypoxia-Ischemia, Brain / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008636:Mesencephalon / Q000473:pathology; D013788:Thalamus / Q000473:pathology