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Table 1 Diagnostic criteria CJD based on the Rotterdam criteria and the UK criteria

From: Overview and evaluation of 15 years of Creutzfeldt-Jakob disease surveillance in Belgium, 1998-2012

SPORADIC CDJ

Criteria

 I

 

Rapidly progressive dementia

 II

A

Myoclonus

B

Visual or cerebellar problems

C

Pyramidal or extrapyramidal features

D

Akinetic mutism

 III

 

CJD-typical periodic sharpwave complexes in electroencephalography

 IVa

 

High signal in caudate/putamen on MRI brain scan

Classification

 Confirmed sporadic CJD

Neuropathological/immunocytochemical confirmation

 Probable sporadic CJD

I and 2 of II and III OR I and 2 of II and IVa OR possible sporadic CJD and positive 14-3-3

 Possible sporadic CJD

I and 2 of II and duration < 2 years

aAs of 2010

IATROGENIC CJD

Progressive cerebellar syndrome in a pituitary hormone recipient OR sporadic CJD with a recognised exposure risk, e.g., dura mater transplant

FAMILIAL CJD

Confirmed or probable CJD plus confirmed or probable CJD in a first-degree relative OR neuropsychiatric disorder plus disease-specific PRNP mutation

VARIANT CDJ

Criteria

 I

A

Progressive neuropsychiatric disorder

B

Duration of illness > 6 months

C

Routine investigations do not suggest an alternative diagnosis

D

No history of potential iatrogenic exposure

E

No evidence of a familial form of TSE

 II

A

Early psychiatric featuresa

B

Persistent painful sensory symptomsb

C

Ataxia

D

Myoclonus or chorea or dystonia

E

Dementia

 III

A

EEG does not show the typical appearance of sporadic CJD in the early stages of illnessc

B

Bilateral pulvinar high signal on MRI scan

 IV

A

Positive tonsil biopsy

Classification

 Confirmed variant CJD

IA and neuropathological confirmation of vCJDd

 Probable variant CJD

I and 4/5 of II and IIIA and IIIB OR 1 and IVA

 Possible variant CJD

I and 4/5 of II and IIIA

  1. aDepression, anxiety, apathy, withdrawal, delusions
  2. bIncluding both frank pain and/or dysaesthesia
  3. cGeneralised triphasic periodic complexes at approximately one per second
  4. dSpongiform change and extensive PrP deposition with florid plaques, throughout the cerebrum and cerebellum