Creutzfeldt-Jakob disease (sporadic)

Case contributed by A.Prof Frank Gaillard

Presentation

3 weeks progressive confusion and dizziness. Known multiple myeloma.

Patient Data

Age: 70 years
Gender: Female

There is diffusion restriction involving the left frontal cortex, predominantly anteriorly and in a parafalcine position. There is some mild parafalcine left parietal cortical diffusion restriction. Further diffusion restriction of the left caudate head is present, and there is faint diffusion restriction involving the left putamen and the head of right caudate and putamen. No convincing white matter involvement. There is mild corresponding high FLAIR and T2 signal intensity in these regions. 

No other intra or extraaxial abnormality. No mass-effect. Ventricles and sulci are within normal limits for age. No posterior fossa abnormality.  No suspicious enhancing lesions, in particular, no parenchymal or leptomeningeal enhancement. 

Diffuse myelomatous deposits involving the calvarium again noted. Right maxillary paranasal sinus mucosal disease noted. 

Conclusion:

Diffusion restriction involving the left frontoparietal cortex (frontal > parietal) and left caudate head. The pattern of diffusion restriction and abnormality is  suspicious for Creutzfeldt-Jakob disease. Vasculitis and ischaemia/infarction in the left anterior circulation territory are less likely differentials. 

The patient went on to have a lumbar puncture. 

14-3-3 was strongly positive. Everything else was negative (viral PCR etc...)

EEG was supportive of the diagnosis also. 

Case Discussion

This case illustrates fairly typical appearance of sporadic CJD with DWI giving the best clue to the diagnosis. 

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Case information

rID: 22077
Case created: 9th Mar 2013
Last edited: 29th Jun 2017
Inclusion in quiz mode: Included

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