@article {4523,
	title = {Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report.},
	journal = {Lancet (London, England)// // Lancet},
	volume = {368},
	year = {2006},
	note = {Comment in: Lancet. 2006 Dec 9;368(9552):2037-9; PMID: 17161714},
	month = {2006//},
	pages = {2061 - 7},
	address = {England},
	abstract = {BACKGROUND: Concerns have been raised that variant Creutzfeldt-Jakob disease (vCJD) might be transmissible by blood transfusion. Two cases of prion infection in a group of known recipients of transfusion from donors who subsequently developed vCJD were identified post-mortem and reported in 2004. Another patient from this at-risk group developed neurological signs and was referred to the National Prion Clinic., METHODS: The patient was admitted for investigation and details of blood transfusion history were obtained from the National Blood Service and Health Protection Agency; after diagnosis of vCJD, the patient was enrolled into the MRC PRION-1 trial. When the patient died, brain and tonsil tissue were obtained at autopsy and assessed for the presence of disease-related PrP by immunoblotting and immunohistochemistry., FINDINGS: A clinical diagnosis of probable vCJD was made; tonsil biopsy was not done. The patient received experimental therapy with quinacrine, but deteriorated and died after a clinical course typical of vCJD. Autopsy confirmed the diagnosis and showed prion infection of the tonsils., INTERPRETATION: This case of transfusion-associated vCJD infection, identified ante-mortem, is the third instance from a group of 23 known recipients who survived at least 5 years after receiving a transfusion from donors who subsequently developed vCJD. The risk to the remaining recipients of such transfusions is probably high, and these patients should be offered specialist follow-up and investigation. Tonsil biopsy will allow early and pre-symptomatic diagnosis in other iatrogenically exposed individuals at high risk, as in those with primary infection with bovine spongiform encephalopathy prions.},
	keywords = {*Blood Transfusion/ae [Adverse Effects], *Brain/pa [Pathology], *Creutzfeldt-Jakob Syndrome/pp [Physiopathology], *Palatine Tonsil/pa [Pathology], Adult, Creutzfeldt-Jakob Syndrome/di [Diagnosis], Creutzfeldt-Jakob Syndrome/tm [Transmission], Fatal Outcome, Humans, Male, Middle Aged, Prions/ge [Genetics]},
	isbn = {1474-547X},
	author = {Wroe, Stephen J and Pal, Suvankar and Siddique, Durrenajaf and Hyare, Harpreet and Macfarlane, Rebecca and Joiner, Susan and Linehan, Jacqueline M and Brandner, Sebastian and Wadsworth, Jonathan D F and Hewitt, Patricia and Collinge, John}
}
