The Department of Health for the United Kingdom developed criteria for the case definition of variant Creutzfeldt-Jakob Disease.
|
Clinical Finding |
Criterion |
|
progressive neuropsychiatric disorder |
IA |
|
duration of illness > 6 months |
IB |
|
no alternative diagnosis |
IC |
|
no history of potential iatrogenic exposure |
ID |
|
early psychiatric symptoms (depression, anxiety, apathy, withdrawal, and/or delusions) |
IIA |
|
persistent, painful sensory symptoms (may include pain and/or unpleasant dysaesthesias) |
IIB |
|
ataxia |
IIC |
|
myoclonus, chorea or dystonia |
IID |
|
dementia |
IIE |
|
no EEG performed, or EEG does not show typical findings seen in sporadic CJD (generalized triphasic periodic complexes with a rate of about 1 per second) |
IIIA |
|
bilateral pulvinar high signal on MRI scan |
IIIB |
|
positive tonsillar biopsy for abnormal prion proteins on immunohistochemistry |
IV |
|
neuropathologic changes of variant CJD (spongiform change and extensive prion protein deposition with florid plaques throughout the cerebrum and cerebellum |
NP |
Definite diagnosis:
(1) (IA) AND (neuropathologic changes NP)
Probable diagnosis:
(1) (IA + IB + IC + ID) AND (4 or 5 of II criteria) AND (IIIA + IIIB)
(2) (IA + IB + IC + ID) AND (IV)
Specialty: Infectious Diseases, Neurology
