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Sporadic Creutzfeldt-Jakob disease (sCJD)

Case contributed by Jason Martin
Diagnosis probable

Presentation

Rapidly progressing dementia

Patient Data

Age: 75 years
Gender: Female
  • Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

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).

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