Creutzfeldt Jakob disease

Case contributed by Dr Brad Hayhow

Presentation

Rapid decline in occupational function associated with progressive cognitive impairment, abnormal involuntary movements, falls and urinary incontinence.

Patient Data

Age: 50 years
Gender: Female
CT

No acute intracranial pathology identified

MRI

Bilateral symmetric hyperintensities involving the caudate head, globus pallidus, putamen and posterior medial thalami which have associated diffusion restriction and possible further involvement of the mesial cortex of the superior frontal gyri.  Subtle hyperintensity involving the insular subcortical white matter.  No micro-hemorrhage.

In an appropriate clinical context, this pattern is very suggestive of sporadic CJD. Toxic or metabolic etiologies are considered less likely. 
 

Case Discussion

In addition to the MRI findings, this patient returned a positive 14,3,3 result on CSF analysis.

Her EEG showed generalized slowing without periodic sharp wave complexes (PSWC).

The clinical outcome was relatively rapid progression to death.

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