Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease (CJD)

Zerr et al. modifying the current clinical diagnostic criteria for sporadic CJD to include the detection of either hyperintensity in the basal ganglia (both caudate nucleus and putamen) or in at least two cortical regions (from either the temporal, parietal or occipital cerebral cortices). This implies that the detection of the specified high signal abnormalities in FLAIR or DWI MRI will be considered at the same level of diagnostic importance as periodic sharp wave complexes on the EEG or 14-3-3 protein detection in the CSF

MRI-CJD Consortium criteria for sporadic Creutzfeldt–Jakob disease.

I- Clinical signs

  1. Dementia
  2. Cerebellar or visual
  3. Pyramidal or extrapyramidal
  4. Akinetic mutism

II- Tests

  1. Periodic sharp wave complexes on the EEG
  2. 14-3-3 protein detection in the CSF (in patients with a duration of less than 2 year)
  3. High signal abnormalities in caudate nucleus and putamen or at least two cortical regions (temporal, parietal or occipital) either in diffusion-weight imaging (DWI) or fluid attenuated inversion recovery (FLAIR) MRI

Probable CJD

  • Two out of I and at least one out of II

Possible CJD

  • Two out of I and duration less than 2 years

 

 

References:

  1. Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, Breithaupt M, Varges D, Meissner B, Ladogana A, Schuur M, Haik S, Collins SJ, Jansen GH, Stokin GB, Pimentel J, Hewer E, Collie D, Smith P, Roberts H, Brandel JP, van Duijn C, Pocchiari M, Begue C, Cras P, Will RG, Sanchez-Juan P. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009 Oct;132(Pt 10):2659-68. [Medline]
  2. Meissner B, Kallenberg K, Sanchez-Juan P, Collie D, Summers DM, Almonti S, Collins SJ, Smith P, Cras P, Jansen GH, Brandel JP, Coulthart MB, Roberts H, Van Everbroeck B, Galanaud D, Mellina V, Will RG, Zerr I. MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology. 2009 Jun 9;72(23):1994-2001. [Medline]

Created: Jan 08, 2010