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.
From the case:
Sporadic Creutzfeldt-Jakob disease (sCJD)
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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).