Table 1

Clinical features in six older vCJD cases

Case
Number
Onset yearBlood transmissionAge of onsetDuration of illnessEarly psychiatric/behavioral symptomsInvoluntary
movements (myoclonus/chorea/dystonia)
SensoryAtaxiaClassification in life*MRIOther investigationsWorking diagnosis at death
11999No747Yes
Agitated and paranoid
YesYesYesPossible vCJDNot performed14-3-3 and EEG not performedMulti-infarct dementia
22002No628Yes Depression, WithdrawalYesYesYesProbable vCJDPositive. Bilateral pulvinar signNegative 14-3-3. EEG non-specific slowingvCJD
32002Yes6813Yes
Depression, withdrawal, irritability
YesYesNo (dyspraxic gait)Possible vCJDNegative14-3-3 not performed. EEG non-specific slowingFTD with parkinsonism/PSP (?CJD in late stages)
42006Yes7411NoNoNoYesProbable vCJDNegativePositive tonsil biopsy. 14-3-3 not performed. EEG non-specific slowing with occasional sharp transientsvCJD—but known to be at risk due to blood transfusion from affected case
52006No5640No.
Withdrawal, Behavioural disturbance at 9 months
YesNoYesNegativeFDG PET showed severe right parietal hypometabolism.
FPCIT normal.
14-3-3 and EEG not performed
FTD (?prion disease in late stages)
62010No5933No
Behavioral disturbance, Delusions at 2 years
NoYesYesSuspicious, but not diagnostic. Subtle bilateral caudate and dorsomedial thalamic high signal14-3-3 not performed. EEG non-specific slowingWernickes encephalopathy (?CJD at onset and in later stages)
  • *Retrospective classification.

  • ?, possible; FDG PET, fluorodeoxyglucose-positron emission tomography; FPCIT, iodine-123 fluoropropylbeta-4-iodophenyltropane; FTD, frontotemporal dementia; PSP, progressive supranuclear palsy; vCJD, Variant Creutzfeldt-Jakob disease.