Variant Creutzfeldt-Jakob disease

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Record number: 
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Not specified as very rare
Time to detection: 
6.5 years
Alerting signals, symptoms, evidence of occurrence: 
Withdrawn, irritability, depression, shuffling gait, falls, blurred vision, pain in face and abdomen, difficulty with motor tasks, cognitive impairment, dyspraxia, unsteady gait, fatality.
Demonstration of imputability or root cause: 
Patient homozygous for PRNP at codon 129, received blood from a donor who developed signs of vCJD and died of vCJD 40 months after donation of the implicated unit. Postmortem exam showed changes suggestive of vCJD.
Imputability grade: 
2 Probable
Suggest new keywords: 
variant CJD
red blood cells
Reference attachment: 
Suggest references: 
Llewelyn, C. A., Hewitt, P. E., Knight, R. S., Amar, K., Cousens, S., Mackenzie, J. and Will, R.G (2004). Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 363(9407): 417-21.
Add vCJD in the adverse occurrence taxonomy (Evi)
Expert comments for publication: 
This case was the first published case to associate a donor who died of vCJD with a recipient who developed vCJD 6.5 years after transfusion and subsequently died of the disease. Three cases followed judged as probable (see Notify record number 1382).