TY - JOUR
T1 - Severe hypoxia and multiple infarctions resembling Creutzfeldt-Jakob disease
AU - Mittelbronn, Michel
AU - Capper, David
AU - Bader, Benedikt
AU - Schittenhelm, Jens
AU - Haybaeck, Johannes
AU - Weber, Petra
AU - Meyermann, Richard
AU - Kretzschmar, Hans A.
AU - Wiethölter, Horst
PY - 2008
Y1 - 2008
N2 - 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.
AB - 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.
KW - Creutzfeldt-Jakob disease
KW - Hypoxia
KW - Infarction
KW - Mesencephalon
KW - Prion
UR - http://www.scopus.com/inward/record.url?scp=48049110066&partnerID=8YFLogxK
M3 - Article
AN - SCOPUS:48049110066
SN - 1641-4640
VL - 46
SP - 149
EP - 153
JO - Folia Neuropathologica
JF - Folia Neuropathologica
IS - 2
ER -