Presentation
Rapidly progressive dementia. No focal neurological deficits.
Patient Data
Age: 85 years
Gender: Male
From the case:
Creutzfeldt-Jakob disease (CJD)










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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.
Case Discussion
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.
Diagnostic markers:
- 14-3-3 protein positive
- PrPSc positive
- S100b elevated
The patient died shortly after the diagnosis.