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BSE is a fatal neurodegenerative brain disease in cattle. Cattle can contract BSE by eating feed made from bovine tissues that are contaminated, a practice that is banned in many world regions. The cause of BSE is unknown, butit is believed to be a prion disease (a prion is a self-replicating protein) or a virus that possesses nucleic acids carrying genetic information. The disease affects the brain and spinal cord of cattle, and lesions in these tissues look “spongy.” BSE is a Transmissible Spongiform Encephalopathies (TSE) and similar diseases can infect animals and humans.
'Novel' CJD
9 Jul 2008
BBC News online [edited] [Promed]
'New CJD type' discovered in US
A new form of Creutzfeldt-Jakob disease (CJD) may have been uncovered in a handful of patients in the US. So far, 10 people have died from a fast-advancing form of fatal dementia called PSPr, according to a report in New Scientist [see (4) below]. Patients develop the trademark brain damage associated with CJD -- the type not linked to BSE -- but scientists believe there may be a genetic cause [familial CJD].
Experts in the UK are now checking records to see if any cases have happened across the Atlantic. There are between 50 and 100 new cases of so-called sporadic CJD diagnosed in the UK every year. Unlike "variant CJD," the human form of BSE in cows contracted by eating contaminated brain tissue in the 1980s and 1990s, the cause of most cases of sporadic CJD is unknown.
The new cases were referred to CJD surveillance units in the US because they were a suspiciously fast-advancing form of dementia with additional symptoms such as the loss of the ability to speak and move, even though traditional tests that normally help diagnose CJD proved negative. Post-mortems on those who died revealed the familiar "spongy" brain tissue, covered with tiny holes. These are thought to be caused by the accumulation of "prions," a misshapen version of a normal brain protein.
Dr. Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center, in Ohio, said that he believed the newly discovered type had probably "been around for years, unnoticed." He suggested one interesting common factor was that the patients came from families with a history of dementia, suggesting a genetic cause, but did not carry the gene traditionally associated with a small number of sporadic CJD cases.
Dr. Mark Head, from the UK's National CJD Surveillance Unit, in Edinburgh, said the finding had prompted scientists to start reviewing cases of sporadic CJD in this country to see if there were any of the newly discovered version. He said: "What is interesting about this is that it may mean there are other genes out there waiting to be found which are associated with prion disease, and looking at these patients in the US could help find them.
Related stories
9 Jul 2008 - New CJD-like disease kills 10 people
New Scientist [edited] pd
A new form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease -- with patients gradually losing their ability to think, speak and move -- but has features that make it distinct from known forms of CJD. No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another 5 have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
United Kingdom - National CJD Surveillance Unit -- Monthly statistics & 2007 totals
7 Jan 2008
UK National CJD Surveillance Unit, monthly statistics, 2007 [edited] Monthly Creutzfeldt-Jakob disease statistics -- as of 7 Jan 2008
These following figures show the number of suspect cases of CJD referred to the CJD surveillance unit in Edinburgh and the number of deaths of definite and probable variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy). Definite and probable vCJD cases in the UK as of 7 Jan 2008 Summary of vCJD cases – deaths Deaths from definite vCJD (confirmed): 114 Deaths from probable vCJD (without neuropathological confirmation): 48 Deaths from probable vCJD (neuropathological confirmation pending): 1 Number of deaths from definite or probable vCJD (as above): 163 Summary of vCJD cases – alive Number of probable vCJD cases still alive: 3 Total Number of definite or probable vCJD (dead and alive): 166 These data indicate that there have been no new cases diagnosed during the past month, but the number of patients alive has decreased by one. These data are still consistent with the view that the vCJD outbreak in the UK is in decline (although the incidence curve may be developing a tail). The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, 5 in 2005, 5 in 2006, and 5 in 2007. Totals for all types of CJD cases in the year 2007 As of 31 Dec 2007 in the UK in the year 2007, there were 111 referrals, 47 deaths from sporadic CJD, 2 deaths from iatrogenic CJD, 4 deaths from familial CJD, one from GSS, and 5 deaths from vCJD.
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'Novel' CJD
9 Jul 2008
BBC News online [edited] [Promed]
'New CJD type' discovered in US
A new form of Creutzfeldt-Jakob disease (CJD) may have been uncovered in a handful of patients in the US. So far, 10 people have died from a fast-advancing form of fatal dementia called PSPr, according to a report in New Scientist [see (4) below]. Patients develop the trademark brain damage associated with CJD -- the type not linked to BSE -- but scientists believe there may be a genetic cause [familial CJD].
Experts in the UK are now checking records to see if any cases have happened across the Atlantic. There are between 50 and 100 new cases of so-called sporadic CJD diagnosed in the UK every year. Unlike "variant CJD," the human form of BSE in cows contracted by eating contaminated brain tissue in the 1980s and 1990s, the cause of most cases of sporadic CJD is unknown.
The new cases were referred to CJD surveillance units in the US because they were a suspiciously fast-advancing form of dementia with additional symptoms such as the loss of the ability to speak and move, even though traditional tests that normally help diagnose CJD proved negative. Post-mortems on those who died revealed the familiar "spongy" brain tissue, covered with tiny holes. These are thought to be caused by the accumulation of "prions," a misshapen version of a normal brain protein.
Dr. Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center, in Ohio, said that he believed the newly discovered type had probably "been around for years, unnoticed." He suggested one interesting common factor was that the patients came from families with a history of dementia, suggesting a genetic cause, but did not carry the gene traditionally associated with a small number of sporadic CJD cases.
Dr. Mark Head, from the UK's National CJD Surveillance Unit, in Edinburgh, said the finding had prompted scientists to start reviewing cases of sporadic CJD in this country to see if there were any of the newly discovered version. He said: "What is interesting about this is that it may mean there are other genes out there waiting to be found which are associated with prion disease, and looking at these patients in the US could help find them.
Related stories
9 Jul 2008 - New CJD-like disease kills 10 people
New Scientist [edited] pd
A new form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease -- with patients gradually losing their ability to think, speak and move -- but has features that make it distinct from known forms of CJD. No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another 5 have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
United Kingdom - National CJD Surveillance Unit -- Monthly statistics & 2007 totals
7 Jan 2008
UK National CJD Surveillance Unit, monthly statistics, 2007 [edited] Monthly Creutzfeldt-Jakob disease statistics -- as of 7 Jan 2008
These following figures show the number of suspect cases of CJD referred to the CJD surveillance unit in Edinburgh and the number of deaths of definite and probable variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy). Definite and probable vCJD cases in the UK as of 7 Jan 2008 Summary of vCJD cases – deaths Deaths from definite vCJD (confirmed): 114 Deaths from probable vCJD (without neuropathological confirmation): 48 Deaths from probable vCJD (neuropathological confirmation pending): 1 Number of deaths from definite or probable vCJD (as above): 163 Summary of vCJD cases – alive Number of probable vCJD cases still alive: 3 Total Number of definite or probable vCJD (dead and alive): 166 These data indicate that there have been no new cases diagnosed during the past month, but the number of patients alive has decreased by one. These data are still consistent with the view that the vCJD outbreak in the UK is in decline (although the incidence curve may be developing a tail). The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, 5 in 2005, 5 in 2006, and 5 in 2007. Totals for all types of CJD cases in the year 2007 As of 31 Dec 2007 in the UK in the year 2007, there were 111 referrals, 47 deaths from sporadic CJD, 2 deaths from iatrogenic CJD, 4 deaths from familial CJD, one from GSS, and 5 deaths from vCJD.
Sources and Terms of Use
