Creutzfeldt-Jakob disease (CJD)

Case contributed by Dr Christof Igler

Presentation

Rapidly progressive dementia. No focal neurological deficits.

Patient Data

Age: 85 years
Gender: Male

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.

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