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Published Date: 2014-06-04 17:29:27
Subject: PRO/AH/EDR> Prion disease update (04): conf. VCJD case in Texas
Archive Number: 20140604.2519285
PRION DISEASE UPDATE (04): CONFIRMED VCJD CASE, IN TEXAS
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A ProMED-mail post
ProMED-mail is a program of the
International Society for Infectious Diseases
Date: Mon 2 Jun 2014
Source: CDC [edited]
Laboratory tests have confirmed a diagnosis of variant CJD (a fatal brain disorder) in a patient who recently died in Texas. The confirmation was made when laboratory results from an autopsy of the patient's brain tested positive for variant CJD.
1st described in 1996 in the United Kingdom, variant CJD is a rare, degenerative, fatal brain disorder in humans. It is believed to be caused by consumption of products from cows with the disease bovine spongiform encephalopathy (BSE, or "mad cow" disease).
Worldwide, more than 220 variant CJD patients have been reported, with a majority of them in the United Kingdom (177 cases) and France (27 cases). This case is the 4th to be reported in the United States. In each of the 3 previous cases, infection likely occurred outside the United States, including the United Kingdom (2 cases) and Saudi Arabia (1 case). The history of this 4th patient, including extensive travel to Europe and the Middle East, supports the likelihood that infection occurred outside the United States. CDC assisted the Texas Department of State Health Services (DSHS)'s investigation of this case and will continue to help confirm further details of the patient's history, including the potential source of infection.
A classic form of CJD, which is not caused by the BSE agent, occurs worldwide, including in the United States. Annually, for every one million people in the United States, 1-2 develop classic CJD. More information about variant CJD, including how it differs from classic CJD, is available in the Variant Creutzfeldt-Jakob Disease Fact Sheet [see below and at http://www.cdc.gov/ncidod/dvrd/vcjd/...e-in-texas.htm.
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Communicated by:
ProMED-mail
<promed@promedmail.org>
[The probability is that the infection was contracted outside of the USA and most likely during residence in the UK. In the absence of a link with any of the countries recording vCJD cases, it might mean that the BSE prion is present in cattle in Texas.
Background from the CDC Fact Sheet: Variant Creutzfeldt-Jakob Disease http://www.cdc.gov/ncidod/dvrd/vcjd/...eet_nvcjd.htm:
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Variant CJD (vCJD) is a rare, degenerative, fatal brain disorder in humans. Although experience with this new disease is limited, evidence to date indicates that there has never been a case of vCJD transmitted through direct contact of one person with another. However, a case of probable transmission of vCJD through transfusion of blood components from an asymptomatic donor who subsequently developed the disease has been reported.
Since variant CJD was 1st reported in 1996, a total of 229 patients with this disease from 12 countries have been identified. As of 2 Jun 2014, variant CJD cases have been reported from the following countries: 177 from the United Kingdom, 27 from France, 5 from Spain, 4 from Ireland, 4 from the United States, 3 in the Netherlands, 2 in Portugal, 2 in Italy, 2 in Canada and one each from Japan, Saudi Arabia, and Taiwan. Two of the 4 U.S. cases, 2 of the 4 cases from Ireland, one of the 2 cases from Canada, and the single case from Japan were likely exposed to the BSE agent while residing in the United Kingdom.
There has never been a case of vCJD that did not have a history of exposure within a country where the cattle disease, BSE, was occurring.
It is believed that the persons who have developed vCJD became infected through their consumption of cattle products contaminated with the agent of BSE or -- in 3 cases, each reported from the United Kingdom -- through receipt of blood from an asymptomatic, infected donor. There is no known treatment for vCJD, and it is invariably fatal.
This variant form of CJD should not be confused with the classic form of CJD that is endemic throughout the world, including the United States. There are several important differences between these 2 forms of the disease. The median age at death of patients with classic CJD in the United States, for example, is 68 years, and very few cases occur in persons under 30 years of age. In contrast, the median age at death of patients with vCJD in the United Kingdom is 28 years. vCJD can be confirmed only through examination of brain tissue obtained by biopsy or at autopsy, but a "probable case" of vCJD can be diagnosed on the basis of clinical criteria developed in the United Kingdom.
The incubation period for vCJD is unknown because it is a new disease. However, it is likely that ultimately this incubation period will be measured in terms of many years or decades. In other words, whenever a person develops vCJD from consuming a BSE-contaminated product, he or she likely would have consumed that product many years or a decade or more earlier.
In contrast to classic CJD, vCJD in the United Kingdom predominantly affects younger people, has atypical clinical features with prominent psychiatric or sensory symptoms at the time of clinical presentation, and delayed onset of neurologic abnormalities, including ataxia [lack of voluntary coordination of muscle movements], within weeks or months, dementia and myoclonus [a brief, involuntary twitching of a muscle or a group of muscles] late in the illness, a duration of illness of at least 6 months, and a diffusely abnormal non-diagnostic electroencephalogram.
The BSE epidemic in the United Kingdom reached its peak incidence in January 1993 at almost 1000 new cases per week. The outbreak may have resulted from the feeding of scrapie-containing sheep meat-and-bone meal to cattle. There is strong evidence and general agreement that the outbreak was amplified by feeding rendered bovine meat-and-bone meal to young calves. - Mod.CP
Subject: PRO/AH/EDR> Prion disease update (04): conf. VCJD case in Texas
Archive Number: 20140604.2519285
PRION DISEASE UPDATE (04): CONFIRMED VCJD CASE, IN TEXAS
************************************************** ******
A ProMED-mail post
ProMED-mail is a program of the
International Society for Infectious Diseases
Date: Mon 2 Jun 2014
Source: CDC [edited]
Laboratory tests have confirmed a diagnosis of variant CJD (a fatal brain disorder) in a patient who recently died in Texas. The confirmation was made when laboratory results from an autopsy of the patient's brain tested positive for variant CJD.
1st described in 1996 in the United Kingdom, variant CJD is a rare, degenerative, fatal brain disorder in humans. It is believed to be caused by consumption of products from cows with the disease bovine spongiform encephalopathy (BSE, or "mad cow" disease).
Worldwide, more than 220 variant CJD patients have been reported, with a majority of them in the United Kingdom (177 cases) and France (27 cases). This case is the 4th to be reported in the United States. In each of the 3 previous cases, infection likely occurred outside the United States, including the United Kingdom (2 cases) and Saudi Arabia (1 case). The history of this 4th patient, including extensive travel to Europe and the Middle East, supports the likelihood that infection occurred outside the United States. CDC assisted the Texas Department of State Health Services (DSHS)'s investigation of this case and will continue to help confirm further details of the patient's history, including the potential source of infection.
A classic form of CJD, which is not caused by the BSE agent, occurs worldwide, including in the United States. Annually, for every one million people in the United States, 1-2 develop classic CJD. More information about variant CJD, including how it differs from classic CJD, is available in the Variant Creutzfeldt-Jakob Disease Fact Sheet [see below and at http://www.cdc.gov/ncidod/dvrd/vcjd/...e-in-texas.htm.
--
Communicated by:
ProMED-mail
<promed@promedmail.org>
[The probability is that the infection was contracted outside of the USA and most likely during residence in the UK. In the absence of a link with any of the countries recording vCJD cases, it might mean that the BSE prion is present in cattle in Texas.
Background from the CDC Fact Sheet: Variant Creutzfeldt-Jakob Disease http://www.cdc.gov/ncidod/dvrd/vcjd/...eet_nvcjd.htm:
---------------------------------------
Variant CJD (vCJD) is a rare, degenerative, fatal brain disorder in humans. Although experience with this new disease is limited, evidence to date indicates that there has never been a case of vCJD transmitted through direct contact of one person with another. However, a case of probable transmission of vCJD through transfusion of blood components from an asymptomatic donor who subsequently developed the disease has been reported.
Since variant CJD was 1st reported in 1996, a total of 229 patients with this disease from 12 countries have been identified. As of 2 Jun 2014, variant CJD cases have been reported from the following countries: 177 from the United Kingdom, 27 from France, 5 from Spain, 4 from Ireland, 4 from the United States, 3 in the Netherlands, 2 in Portugal, 2 in Italy, 2 in Canada and one each from Japan, Saudi Arabia, and Taiwan. Two of the 4 U.S. cases, 2 of the 4 cases from Ireland, one of the 2 cases from Canada, and the single case from Japan were likely exposed to the BSE agent while residing in the United Kingdom.
There has never been a case of vCJD that did not have a history of exposure within a country where the cattle disease, BSE, was occurring.
It is believed that the persons who have developed vCJD became infected through their consumption of cattle products contaminated with the agent of BSE or -- in 3 cases, each reported from the United Kingdom -- through receipt of blood from an asymptomatic, infected donor. There is no known treatment for vCJD, and it is invariably fatal.
This variant form of CJD should not be confused with the classic form of CJD that is endemic throughout the world, including the United States. There are several important differences between these 2 forms of the disease. The median age at death of patients with classic CJD in the United States, for example, is 68 years, and very few cases occur in persons under 30 years of age. In contrast, the median age at death of patients with vCJD in the United Kingdom is 28 years. vCJD can be confirmed only through examination of brain tissue obtained by biopsy or at autopsy, but a "probable case" of vCJD can be diagnosed on the basis of clinical criteria developed in the United Kingdom.
The incubation period for vCJD is unknown because it is a new disease. However, it is likely that ultimately this incubation period will be measured in terms of many years or decades. In other words, whenever a person develops vCJD from consuming a BSE-contaminated product, he or she likely would have consumed that product many years or a decade or more earlier.
In contrast to classic CJD, vCJD in the United Kingdom predominantly affects younger people, has atypical clinical features with prominent psychiatric or sensory symptoms at the time of clinical presentation, and delayed onset of neurologic abnormalities, including ataxia [lack of voluntary coordination of muscle movements], within weeks or months, dementia and myoclonus [a brief, involuntary twitching of a muscle or a group of muscles] late in the illness, a duration of illness of at least 6 months, and a diffusely abnormal non-diagnostic electroencephalogram.
The BSE epidemic in the United Kingdom reached its peak incidence in January 1993 at almost 1000 new cases per week. The outbreak may have resulted from the feeding of scrapie-containing sheep meat-and-bone meal to cattle. There is strong evidence and general agreement that the outbreak was amplified by feeding rendered bovine meat-and-bone meal to young calves. - Mod.CP
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