Variant Creutzfeldt-Jakob disease

Status:

Ready to upload

Record number:

1379

Adverse Occurrence type:

vCJD

MPHO Type:

Red blood cells

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Withdrawn, irritability, depression, shuffling gait, falls, blurred vision, pain in face and abdomen, difficulty with motor tasks, cognitive impairment, dyspraxia,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

Groups audience:

Infections -- Prion

Infections

Keywords:

vCJD (variant Creutzfeldt-Jakob disease)

CJD (Creutzfeldt-Jacob disease)

References:

Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion.

Suggest new keywords:

variant CJD

red blood cells

Reference attachment:

_ vCJD, Llewelyn, 2004.pdf

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.

Note:

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).