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Published Date: 2013-05-21 23:48:25
Subject: PRO/AH/EDR> Creutzfeldt-Jakob disease - Colombia: variant CJD suspected
Archive Number: 20130521.1729226

CREUTZFELDT-JAKOB DISEASE - COLOMBIA: VARIANT CJD SUSPECTED
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Date: Mon 20 May 2013
Source: La Vanguardia, Colprensa report [in Spanish, trans. Sr.Tech.Ed.MJ, edited]
http://www.vanguardia.com/actualidad/colombia/208988-posible-primer-caso-de-enfermedad-de-vacas-locas-en-colombia


Suspected human case of 'Mad cow disease'
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A 61-year-old woman who was admitted to a hospital in Cali could be the 1st case of 'mad cow disease' [variant Creutzfeldt-Jakob disease (vCJD)] in Colombia, according to specialized tests performed by the Instituto de Referencia Andino [laboratory in Colombia].

The patient's sister said, "The results of tests done on my sister say she has 'mad cow disease'. The persons who reviewed the tests initially say it is the 1st case in Colombia, and if she is infected it would have been through meat and milk consumed in the country."

However, sources from the Ministry of Health consulted by Colprensa ruled out the presence of this disease in the country and warned that this "should not be a matter for concern." Nonetheless, they said the issue is being "reviewed" and "an official statement" on the subject will be issued this afternoon [20 May 2013]. So far, the clinic in the capital of Valle del Cauca, where the patient remains hospitalized, has not commented on her condition.

How 'mad cow disease', whose scientific name is [variant Creutzfeldt-Jakob disease (vCJD)], is contracted has not been fully ascertained, but recent studies suggest that it is through consumption of meat contaminated with the [the prion causing the disease], or through blood transfusions.

The patient's sister said the woman has never traveled to Europe (where cases have been reported) and if, in fact she became infected, it had to be through something she ate in Colombia. "She can not speak because she is in a comatose state, but she has not traveled to Europe where supposedly the disease only occurs, but about a year ago she went to Guayaquil (Ecuador]," she said.

The woman said initially her sister presented numbness of the muscles of the legs accompanied by intense pain, followed by dimness of vision and loss of balance, in addition to other symptoms such as vomiting, disorientation, and dizziness, for which she was taken to the emergency service.

"In Colombia we were told she was completely healthy. In Bogota and Medellin all tests were negative. We decided to send the case to the USA for further exploration and the results we received said she has 'mad cow disease'."

In recent years cases have been reported in Britain, Portugal, Ireland, and France, and sporadic cases have been reported in other European countries, in which patients have died from damage to their nervous systems.

The UK Health Protection Agency recently indicated that more than 1000 Britons could die in the UK because of the disease. The report also warned that some experts estimate that 30 000 citizens of that country could be carriers of the disease.

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[CJD (Creutzfeldt-Jakob Disease) is a rare illness and is one of a group of neurological diseases called prion diseases, which affect [both] humans and animals. Prion diseases exist in different forms, all of which are progressive, currently untreatable and ultimately fatal. Their name arises because they are associated with an alteration in a naturally occurring protein: the prion protein.

CJD was first described in 1920. The commonest form is called sporadic CJD and occurs worldwide, causing around 1-2 deaths per million population per year. A new form of CJD (called variant Creutzfeldt-Jacob disease, vCJD) linked to bovine spongiform encephalopathy (BSE) in cattle was identified in 1996. There are also inherited forms of human prion disease linked to mutations of the prion protein gene and cases caused by infection via medical or surgical treatments (iatrogenic CJD).

Variant Creutzfeldt-Jakob disease is a rare and fatal human neurodegenerative condition which is classified as a Transmissible Spongiform Encephalopathy (TSE) because of its ability to be transmitted and the characteristic spongy degeneration of the brain that it causes. vCJD was first described in the United Kingdom in March 1996 and has been linked with exposure to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE), also known as Classical BSE1, which was first reported in the United Kingdom in 1986. In contrast to the traditional forms of CJD, vCJD has affected younger patients (median age at death of 28 years, as opposed to 68 years) and has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months). vCJD was associated with the consumption of meat from BSE-infected cattle.

From October 1996 to March 2011, 176 cases of vCJD have been reported in the United Kingdom, 25 in France, 5 in Spain, 4 in Ireland, 3 each in the Netherlands and the United States of America (USA), 2 each in Canada, Italy and Portugal, and one each in Japan, Saudi Arabia and Taiwan. The number of cases of vCJD in the United Kingdom peaked in 2000 with 28 deaths and then gradually declined. No cases have been confirmed in the UK since 2011.

It is likely that the patient in Colombia has contracted the sporadic form of CJD, or more rarely is expressing a genetic form of the disease. A conclusive diagnosis will require precise molecular characterisation of the patient's prion protein, and is a matter of considerable general interest. - Mod.CP.

A HealthMap/ProMED-mail map can be accessed at: http://healthmap.org/r/1l0z.]

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