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. Author manuscript; available in PMC: 2013 Mar 6.
Published in final edited form as: Can J Neurol Sci. 2012 May;39(3):304–310. doi: 10.1017/s0317167100013421

Table 1.

Summary of Criteria for Probable sCJD

UCSF Require dementia w/2 of 6 following:

  1. Myoclonus

  2. Pyramidal/Extrapyramidal signsa

  3. Visual symptomsb

  4. Cerebellar signs

  5. Akinetic Mutism

  6. Other higher focal cortical signsc

 WHO Require dementia w/2 of 4 following:

  1. Myoclonus

  2. Pyramidal/Extrapyramidala signs

  3. Visualb/Cerebellar signs

  4. Akinetic Mutism
   AND    AND
Typical EEG or MRI8, 9 Typical EEG OR positive CSF 14-3-3 (if total disease duration less than 2 years)
   AND    AND
no other condition exists to explain the disorder no other condition to exists to explain the disorder
a

such as limb rigidity, parkinsonism, and dystonic, choreatic, athetoid or ballistic movements.

b

including but not limited to: deteriorated or blurred vision, visual field restriction, and disturbed perception of structures and colors. Visual hallucinations were rated separately.

c

Other such as neglect, aphasia, apraxia, acalculia, etc….