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).
