New Variant Creutzfeldt-Jakob disease

As in several other countries, the Health Council of the Netherlands has prepared recommendations on the need for routine leukodepletion by filtration of blood. The presence of leukocytes in blood products has no beneficial effect for the recipient, except in special cases, such as patients undergoing organ transplantation (41). Apart from preventing the adverse effects associated with leukocyte transfusion, it has been postulated that the risk of transmission of new variant Creutzfeldt-Jakob disease can be prevented by using leukodepleted blood products. 

Two advisory committees of the US FDA, the Transmissible Spongiform Encephalopathies Advisory Committee and the Vaccines and Related Biologicals Product Advisory Committee, said at a joint meeting on 3 August 2000 that vaccines made from bovine-derived materials from countries with a known or uncertain risk of BSE carry only an infinitesimal risk of new variant Creutzfeldt-Jakob disease, and that no change in US immunization practice is indicated (Evans G, personal communication, 3 August 2000). 

The human form of the disease is called Creutzfeldt-Jakob disease (CJD), which is rare and apparently arises spontaneously. The average age of onset of CJD is 64. In 1994, howeva-, several cases of the disease appeared in young adults in the U.K. To date, 200 cases have been reported. This new variant Creutzfeldt-Jakob disease (vCJD) is caused by ingesting meat products of an animal infected with the disease. 

In human populations, exposure to the BSE agent (probably in contaminated bovine-based food products) has been strongly linked to the 1996 appearance of a new transmissible spongiform encephalopathy of humans called variant Creutzfeldt-Jakob Disease (vCJD). 

However, the emergence of variant Creutzfeldt-Jakob disease (vCJD) in the UK and France has raised concern about a new theoretical risk of infection in patients treated with blood and blood products (198). Animal experiments in which blood from sheep infected with bovine spongiform encephalopathy and natural scrapie-infected sheep into scrapie-free recipient animals have suggested disease transmission by the blood transfusion route in 2 of 24 sheep with bovine spongiform encephalopathy and in 4 of 21 sheep with scrapie (199). Many European countries have incorporated leukodepletion of all blood products, as leukocytes are believed to play a key role in the pathogenesis of variant Creutzfeldt-Jakob disease (198). In some countries, people who have lived in the UK for a period longer than 6 months between 1980 and 1996 are excluded from blood donation (13). Furthermore, it has been shown that various steps used in the manufacture of plasma-derived products also contribute to reduced infectivity by bovine spongiform encephalopathy (198). 

Creutzfeldt-Jakob disease (CJD) New variant CJD Gerstmann-Straussler-Scheinker disease Fatal familial insomnia Kuru Prion protein Extracellular deposits... 

Human prion disease models have also been developed in mice [154,155]. Crossing the species barrier into an experimentally accessible animal system, the prions responsible for Creutzfeldt Jakob disease, new variant CJD, Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia produce a reproducible time-dependent neuronal degeneration leading to death. 

Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alp-erovitch A, Poser S, Pocchiari M, Hofman A, Smith PG (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347 921-925. 

Steroid-responsive encephalopathies can be considered vasculitic or non-vasculitic. Clinical features are suggestive of Creutzfeldt-Jakob disease (CJD), dementia with Lewy bodies (DLB), and parkinsonism, but pathological examination revealed only AD-related findings without evidence of Lewy bodies or prion disease in most cases. AD is not diagnosed in life due to the atypical clinical features, lack of hippocampal atrophy on brain imaging, and a dramatic symptomatic response to steroids [97], Some cases of new-variant CJD may also be misdiagnosed as AD. 

Collinge J, Beck J, Campbell T et al (1996) Prion protein gene analysis in new variant cases of Creutzfeldt-Jakob disease. Lancet 348 56... 

Ironside JW, Sutherland K, Bell JE, et al. A new variant of Creutzfeldt-Jakob disease Neuropathological and clinical features. Cold Spring Harb Symp Quant Biol. 1996 50 523-527. 

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