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Creutzfeldt-Jakob Disease

Confirmed case

• Positive testing of brain tissue obtained upon autopsy using neuropathological examination, immune-cytochemical methods, or Western blot detection of disease‐specific PrP.

Probable case

• Progressive dementia;

AND

• At least two of the following: 

    • Myoclonus;
      OR
    • Visual or cerebellar disturbance;
    • OR
    • Pyramidal or extra-pyramidal dysfunction;
      OR

    • Akinetic mutism;
AND

• At least one of the following: 

    • periodic sharp wave complexes on EEG;
      OR
    • positive 14‐3‐3 assay on CSF;
      OR
    • MRI imaging showing high abnormal signal in the caudate nucleus and putamen on fluid attenuated inversion recovery (FLAIR) or Diffusion weighted imaging (DWI).

Suspect Case

• Progressive dementia;

AND

• At least two of the following:

    • Myoclonus;
      OR
    • Visual or cerebellar disturbance;
    OR
    • Pyramidal or extra-pyramidal dysfunction;
      OR
    • Akinetic mutism;
AND

• None of the following:

    • periodic sharp wave complexes on EEG;
      OR
    • positive 14‐3‐3 assay on CSF;
      OR
    • MRI imaging showing high abnormal signal in the caudate nucleus and putamen on fluid attenuated inversion recovery (FLAIR) or Diffusion weighted imaging (DWI).


SOURCE: Creutzfeldt-Jakob Disease ( )
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