Sporadic Creutzfeldt-Jakob disease (sCJD)

Case contributed by Jason Martin

Presentation

Rapidly progressing dementia

Patient Data

Age: 73
Gender: Female
Modality: MRI

Basal ganglia T1 and DWI hyperintensity. High cortical DWI signal. 

Case Discussion

This is certainly a tough case. There is considerable overlap in presentation between much of the differential, but a characteristic feature in this patient provides a clue to diagnosis. But first, let’s narrow down the differential of high basal ganglia T1 signal:

  • Leigh syndrome is primarily seen in children
  • osmotic demyelination syndrome is T2 hyperintense, and usually in putamen and external capsule
  • Wilson disease can be distinguished based on the current disease trajectory
    • if copper predominating, T1 hyperintense, T2 hypointense
    • if gliosis predominates, T1 hypointense, T2 hyperintense
  • hypoxic ischemic injury is more common in globus pallidus, but can affect all deep nuclei

The likely diagnosis is Creutzfeldt-Jakob disease (CJD).

PlayAdd to Share

Case Information

rID: 20920
Case created: 21st Dec 2012
Last edited: 10th Sep 2015
System: Head & Neck
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.