Creutzfeldt-Jakob disease (CJD)
Rapidly progressive dementia. No focal neurological deficits.
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Asymmetric restricted cortical diffusion involving predominantly the left hemisphere.
The FLAIR shows multiple non-specific hyperintense white-matter lesions and a hyperintensity of the right thalamus.
Symmetrical cortical atrophy.
No pathological contrast enhancement.
The cortical restricted diffusion on DWI gives the best clue to the diagnosis of CJD.
The diagnosis was confirmed by the German Prion Research Center in Goettingen.
- 14-3-3 protein positive
- PrPSc positive
- S100b elevated
The patient died shortly after the diagnosis.