Creutzfeldt-Jacob disease (CJD)

Case contributed by A.Prof Frank Gaillard

Presentation

Four weeks rapid decline in cognition and fluctuating conscious state and labile mood.

Patient Data

Age: 80 years
Gender: Female
MRI

There is cortical restricted diffusion within the left frontal, parietal and occipital lobes as well as within the right parietal lobe. There is possible minor FLAIR hyperintensity within the left frontal cortex, however no cortical swelling is identified.

No abnormal contrast enhancement. No MRA abnormality.

Extensive bilateral T2/FLAIR white matter hyperintensities are most likely consistent with extensive chronic small vessel ischemia. Cerebral volume is commensurate with age.

Conclusion:

Findings are suggestive of sporadic CJD.

Diagram

EEG

EEG demonstrates characteristic periodic triphasic activity. A normal EEG from another patient is also included.  

Case Discussion

Typical presentation, imaging and EEG findings of Creutzfeldt-Jacob disease (CJD).

Cerebrospinal fluid (CSF)

  • CJD Protein (14-3-3): POSITIVE
  • Protein 0.47H g/L (normal <0.45)
  • Glucose 4.1H mmol/L (normal 2.2-3.9)

COMMENT: The 14-3-3 protein is a non-specific marker of central nervous system neuronal injury or death. A positive result has approximately 90% sensitivity and specificity for sporadic Creutzfeldt-Jacob disease. "False" positives are frequently recognized in encephalitis (especially Herpes simplex) & recent cerebral infarcts. 

 

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Case information

rID: 29461
Published: 13th Jun 2014
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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