Creutzfeldt Jakob disease

Case contributed by Dr Brad Hayhow


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

Patient Data

Age: 49
Gender: Female

No acute intracranial pathology identified


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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Case information

rID: 43970
Published: 26th Apr 2016
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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