Creutzfeldt-Jakob disease (CJD)

Case contributed by Dr C.Igler

Presentation

Rapidly progressive dementia. No focal neurological deficits.

Patient Data

Age: 83
Gender: Male
MRI

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.

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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Case information

rID: 41280
Case created: 24th Nov 2015
Last edited: 12th Oct 2016
Inclusion in quiz mode: Included

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