CJD Update - Wife And
Sister-In-Law Both Die

From Patricia Doyle, PhD
Hello Jeff - The last case in the below update is really interesting. A wife and sister-in-law die within 2 years of each other from sporadic CJD. The update also includes more on risk of transmission of CJD via blood.
Patricia Doyle
In this update:
[1] UK DOH vCJD Monthly statistics - October 2004
[2], [3], [4] & [5] Potentially Contaminated Blood Products
[5] Sporadic CJD Cases
Date: Fri 8 2004
From: ProMED-mail
Source: UK Department of Health, monthly Creutzfeldt-Jakob disease
statistics, 2004/0354, Wed 6 Oct 2004 [edited]
[Now that the incidence in the UK of variant Creutzfeldt-Jakob Disease -- abbreviated as CJD (new var.) or vCJD in ProMED-mail -- shows evidence of having peaked, or, at least of having reached a plateau, the Department of Health's full table of definite and probable CJD deaths and referrals will no longer be reproduced in ProMED-mail. Only the monthly summary of variant CJD statistics will be posted, unless there is a substantial change in the situation. The full table, updated monthly, can be accessed at the Department of Health website:
The precise definition of the terms deaths, definite cases, probable vCJD
cases, and, the case definitions can be found by accessing the Department
of Health web-site, or by referring to a previous ProMED-mail post in this
thread (for example, CJD (new var.) - UK: update Mar 2002 20020305.3693) -
Monthly variant Creutzfeldt-Jakob disease statistics as of Mon 4 Oct 2004
The Department of Health is today [Wed 6 Oct 2004] issuing the latest information about the numbers of known cases of Creutzfeldt-Jakob disease. This includes cases of variant Creutzfeldt-Jakob disease (vCJD), the form of the disease thought to be linked to BSE. The position is as follows:
Definite and probable CJD cases in the UK:
Summary of vCJD cases - deaths
Deaths from definite vCJD (confirmed): 104
Deaths from probable vCJD (without neuropathological confirmation): 38
Deaths from probable vCJD (neuropathological confirmation pending): 2
Total number of deaths from definite or probable vCJD (as above): 144
Summary of vCJD cases - alive
Number of probable vCJD cases still alive: 5
Number of definite or probable vCJD (dead and alive): 149
(The next update will be published on Mon 1 Nov 2004)
[Since the previous monthly statistics were released on Mon 6 Sep 2004, the number of deaths from probable vCJD (neuropathological confirmation pending) has increased by 2, raising the total number of deaths from definite or probable vCJD to 144. The number of probable vCJD cases still alive remains at 5. Therefore the overall total number of definite or probable vCJD cases now becomes 149. - Mod.CP]
Date: Wed 22 Sep 2004
From: Pablo Nart
Source: BBC News online, Tue 21 Sep 2004 [edited]
UK - Thousands Warned Over vCJD Rrisk
Thousands of people are to receive letters warning them they may have been exposed to vCJD through contaminated blood products. The CJD Incidents Panel says about 4000 in the UK, mostly haemophiliacs, are thought to be at risk. The patients may have received blood products, such as clotting agents, from donors who subsequently developed vCJD. But the government is stressing the risk is very low, and relates to people exposed to blood products before 1999, when precautions were brought in.
9 donors have been identified as having developed vCJD after giving blood. Their donations would have gone into plasma pools that would have been diluted by thousands of other donations. The CJD Incidents Panel said this diluting would reduce the risk of contamination. In total, 6000 letters are to be sent out informing patients of the risk assessment -- although only 2/3 are being told they may be at direct risk. There is no test for the brain-wasting disease, so those at risk have no way of knowing whether they have vCJD. In addition to haemophiliacs, a small number of people with immune system disorders will receive the letters.
Government scientists say more work is needed to determine the exact risk each individual has been exposed to. But those at risk will be told not to donate blood, and to inform doctors and dentists if they undergo treatment in the future in a bid to stop further onward transmission. Professor Don Jeffries, acting chair of the CJD Incidents Panel, said: "In the interests of public safety, we have been advised to take the most precautionary measure."
Sir Liam Donaldson, the chief medical officer, said the risk needed to be looked at in context of the exposure the public has had to BSE in the 1980s and 1990s through meat. "The entire population of the UK was exposed to a massive risk of vCJD through eating [BSE-contaminated] products." And Professor Noel Gill, of the Health Protection Agency, which is coordinating the informing of patients, said: "The bottom line is that we are taking this precaution to minimise patient-to-patient transmission. The price of that is that a number of patients get a letter saying they could be at risk. This is clearly quite an overwhelming piece of information to come to them."
Health secretary John Reid launched the review of vCJD infection risk in December 2003 after announcing that a person had died from the human form of BSE after receiving blood from a donor who later developed the disease. A 2nd person who had received a blood transfusion has subsequently died from other causes but had signs of vCJD in the spleen. The government has already identified 17 people who received blood transfusions from people who went on to develop vCJD. Since 1997 all cases of vCJD that are reported to the National CJD Surveillance Unit and diagnosed as having "probable" vCJD are passed on to the National Blood Service, which searches its blood donor records. If the patient has given blood, any stocks of that blood are immediately destroyed. White blood cells, which are thought to carry the greatest risk of transmitting the disease, have been removed from all blood used for transfusion since 1999.
And blood products have been prepared from plasma imported from the US since 1998. Graham Whitehead, the chief executive of the Haemophilia Society, said the news would cause anxiety, especially in the wake of HIV and hepatitis C infection from blood products. But he said it must be put in context. "It is very reassuring that no-one in the world with haemophilia has been diagnosed with vCJD."
Any person with concerns can ring a dedicated NHS Direct helpline on 0845 850 9850 for England and Wales, and NHS 24 on 0845 4242424 in Scotland and Northern Ireland.
Pablo Nart
Date: Thu 23 Sep 2004
From: ProMED-mail
Source: Eurosurveillance Weekly, Vol. 8, Issue 39, 23 Sep 2004 [edited]
vCJD And Plasma Products -
Implementation Of Public Health Preecautions In The UK
By Anna Molesworth (, Helen Janecek, Noel Gill, Nicky Connor, Health Protection Agency Communicable Disease Surveillance Centre, London, United Kingdom
The CJD Incidents Panel (CJDIP), a United Kingdom expert committee set up to advise on the management of "incidents" of potential transmission of Creutzfeldt-Jakob disease) between patients, has issued recommendations on the management of variant CJD (vCJD) risk from implicated plasma products. To date, 9 UK plasma donors are known to have developed vCJD. Collectively, they made 23 plasma donations. The donated plasma was used to manufacture factor VIII, factor IX, antithrombin, intravenous immunoglobulin G, albumin, intramuscular human normal immunoglobulin, and anti-D.
The potential risk of vCJD infection following treatment with any implicated plasma products, on top of the risk from dietary exposure to the bovine spongiform encephalopathy (BSE) agent, is very uncertain. So far, there are no recorded instances of vCJD being spread through surgery, nor have there been any cases among recipients of plasma products sourced from individuals who later developed vCJD. In December 2003, the death from vCJD of a person some years after receiving a blood transfusion from a donor who had died of vCJD was announced (1). In July 2004 a 2nd probable case of transfusion-associated vCJD infection was identified (2). These 2 events have increased concern about the potential infectivity of blood and plasma products.
The CJDIP now recommends that certain special public health precautions need to be taken for some recipients of UK-sourced plasma products that were manufactured using donations from individuals who subsequently developed vCJD. This is in order to reduce any possible risk of iatrogenic transmission of vCJD.
The CJDIP has used a vCJD blood risk assessment,
together with information on how the particular batches of plasma products were manufactured, to assess the potential levels of infection that patients were exposed to.
The CJDIP advises certain special public health precautions need to be taken for recipients of UK sourced plasma products who have been exposed to a 1 per cent or greater potential additional risk of vCJD infection, as these patients could pose a risk to others in defined circumstances. These at-risk patients are asked: (a) not to donate blood, organs or tissues, (b) to inform their clinician if they need medical, surgical or dental treatment, so that infection control precautions can be taken to reduce any possible risk of spreading vCJD, and to consider informing their family, in case they (the patients) need emergency surgery in the future
The CJDIP has categorised each batch of implicated plasma products according to the likelihood that special public health precautions need to be taken as follows: (a) High: the amount of potential infectivity in product batches was high enough to warrant special public health precautions following the administration of a very small dose. These batches should be traced, and the recipients advised of their exposure and asked to take special public health precautions. (b) Medium: substantial quantities of the material in question would need to have been administered to warrant special public health precautions. Efforts should be made to trace these batches and assess the additional risk to individual recipients to determine whether pecial precautions should be taken. (c) Low: the potential additional risk to recipients is considered negligible. These batches do not need to be traced and the individual recipients do not need to be informed.
This categorisation is based on very cautious assumptions, and the uncertainties underlying the assessment of 'risk' are great. The CJDIP guidance is to limit any possible iatrogenic human-to-human transmission of vCJD. It should NOT be interpreted as an estimate of an individual patient's additional risk of developing vCJD, which is uncertain, and likely to be very low.
The patients who may be affected include some with bleeding disorders, some patients with primary immunodeficiency (PID), and some patients with other conditions, who may include, for example, patients with secondary immunodeficiencies, certain neurological and autoimmune conditions, plasma exchange recipients and patients with severe burns, and with some other conditions requiring critical care. Patients in the UK who are 'at-risk' of vCJD for public health purposes are being contacted by their doctors and informed of the precautions they will need to take.
The product manufacturers are providing details to individual countries to which parts of batches with a 'High' or 'Medium' likelihood that public health precautions might be required were exported. The UK Department of Health and the Health Protection Agency are providing further details to authoritative bodies in these countries as well as to the European Commission and WHO. The Health Protection Agency's (HPA) CJD section at the Communicable Disease Surveillance Centre is coordinating the patient notification in England, Wales, and Northern Ireland. The Scottish Centre for Infection and Environmental Health (SCIEH) is coordinating this notification in Scotland. Background information about vCJD with useful links is available from their websites: HPA:
/topics_az/cjd/menu.htm; SCIEH:
(1) Llewelyn CA, Hewitt PE, Knight RS, Amar K, Cousens S, Mackenzie J, et al. Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 2004; 363: 417-21.
(2) Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004; 364: 527-9.
(byline: Anna Molesworth (, Helen Janecek, Noel Gill, Nicky Connor, Health Protection Agency Communicable Disease Surveillance Centre, London, United Kingdom)
Date: Mon 27 Sep 2004
From: ProMED-mail
Source: The Times of India, Mon 27 Sep 2004 [edited]
India: Tainted Blood Products Received From The UK
Britain has exported blood products that could be contaminated with the human form of mad cow disease to at least 11 countries, including India, raising fears of further transmission of the deadly condition, a leading daily reported on Mon 27 Sep 2004.
Officials last week contacted 5 of the countries identified as most at risk from the imported blood products, which were donated by 9 people who died from variant Creutzfeldt-Jacob disease (vCJD), The Times reported. However, the government has been accused of "lethal secrecy" after refusing to identify publicly the 5 nations which have been the subject of risk assessments by the Health Protection Agency. The exports of the suspect products were effected in the late 1990s. The warnings have been issued after 2 British cases discovered in the past year in which people are thought to have contracted the brain-wasting disease from blood transfusions.
Besides India, where 953 vials of albumin were sent, the other 10 recipient countries are Ireland (polio vaccine, 83 500 doses), Brazil (44 864 vials albumin, 80 vials immunoglobulin), Dubai (UAE)(2400 vials albumin), Turkey (840 vials immunoglobulin), Brunei (400 vials albumin), Egypt (144 vials albumin), Morocco (100 vials albumin), Oman (100 vials immunoglobulin), Russia (23 vials factor VIII) and Singapore (3 vials immunoglobulin).
Date: Mon 27 Sep 2004
Source: Yahoo News, UK, Mon 27 Sep 2004 [edited]
Britain Exported Potentially Dangerous Blood Products
The United Kingdom has exported to "at least 11 countries" blood products at risk of contamination with the prion responsible for variant Creutzfeldt-Jakob Disease (vCJD), the human form of mad cow disease, The Times reports. The blood plasma products resulted from donations by 9 people who later died of the incurable disease.
The countries, in order of the number of samples sent, are Singapore (3 samples), Russia (23), Oman (100), Morocco (100), Egypt (144), the sultanate of Brunei (400), Turkey (840), India (953), Dubai (2400), Brazil (44 864) and Ireland (83 500). Five of these countries were warned by Britain's sanitary services, The Times said, but the daily did not know which. Last week letters went out to 6000 patients in Britain, most of them haemophiliacs, who may have been exposed to vCJD through blood plasma products.
According to health ministry figures, 141 [144 as of 4 Oct 2004] people are known to have died in Britain from vCJD, a spongy deterioration of the brain causing personality change, loss of body function, and eventually death. Cases of the disease peaked in 2000, when 28 deaths were reported. Since then the trend has been generally downward, with 17 cases in 2002 and 18 last year. In May government-funded scientists estimated 3800 people in Britain could be harbouring the human form of mad cow disease without knowing it.
Date: Sun 3 Oct 2004
From: ProMED-mail <
UK- Wife And Sister Of Man Die Of Sporadic CJD
By Helen Morgan
The Scotsman
A man today described the deaths of his wife and sister from Creutzfeldt-Jakob Disease (CJD) as a "horrific coincidence". The man's wife, from Walham Green, Sandhurst, Gloucestershire, died last month after battling the sporadic form of the brain disease for 9 months.
The man's sister died 2 years ago after contracting the same form of the disease. Both women were 67 when they died. They were both treated in the same ward in the Gloucestershire Royal Hospital. His wife's walking was first affected before her memory started to go. He said latterly she got very angry and violent due to her frustration at the disease.
Her husband, a retired fitter who is 70, said: "I feel terrible. I can't believe it. I feel completely in the dark and have no idea whatsoever why they died." CJD Support Network co-ordinator Gill Turner said there was one in a million cases of this form of the disease. She said every case of the disease is looked at by the CJD incident panel and believed the findings of these deaths would be "very interesting". "The likelihood of such close non-blood relatives getting CJD would be very unusual," she added.
According to the CJD Surveillance Unit in Edinburgh, 993 people have died of all forms of CJD in Britain between 1990 and 2004. Some 740 of those deaths were attributed to sporadic CJD, the most common form of the disease.
Patricia A. Doyle, PhD
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