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Published On: Wed, Jan 29th, 2014

Suspect case of Creutzfeldt-Jakob disease investigated in Brazil

The death of a 70-year-old man from  Belo Horizonte, Brazil is being investigated by health authorities as possibly being caused by the prion disease, Creutzfeldt-Jakob disease (CJD), according to a Wall Street Journal report Tuesday.

Image/CIA

Image/CIA

According to the report, tissue samples are currently be analyzed to determine if the cause of death was CJD. Last year, health officials in the city investigated four other suspect cases; however, all tested negative for CJD.

Creutzfeldt-Jakob disease (CJD) is a rare and fatal disease that affects the nervous system and causes deterioration of the brain. It affects about one in a million people each year worldwide. In the United States, only about 200 people are diagnosed with CJD each year.

In the early stages of CJD, the patient may have rapidly failing memory and other cognitive difficulties. These symptoms become worse much faster than with other more common types of dementia, such as Alzheimer’s disease. Other symptoms can include personality changes including anxiety and depression, lack of coordination, and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements such as sudden jerky movements, blindness, weakness of extremities, and coma may occur. There is no treatment or cure.

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About the Author

- Writer, Co-Founder and Executive Editor of The Global Dispatch. Robert has been covering news in the areas of health, world news and politics for a variety of online news sources. He is also the Editor-in-Chief of the website, Outbreak News Today and hosts the Outbreak News This Week Radio Show on http://1380thebiz.com/ Robert is politically Independent and a born again Christian Follow @bactiman63

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  1. Nathália says:

    Many cases in Brazil.
    1. A forestry engineer that worked for Emater, 45, in Santarém (PA), diagnosed with CJD in November 2011, treated at Hospital Porto Dias, Adventist Hospital in Bethlehem, Guadalupe Hospital in Bethlehem, and the Portuguese Beneficent, in São Paulo.
    http://diaconoluizgonzaga.blogspot.com.br/2011/10/doenca-misteriosa-vitima-engenheiro.html

    2. Adalia Pains´ sister, 63, diagnosed with CJD in 2011, in Ceres, Goiás
    http://weblog.aventar.eu/blogue.weblog.com.pt/arquivo/2011/03/doenca_de_creut.html

    3. The Amazon man diagnosed with CJD at University Hospital Getúlio Vargas (HUGV), in Manaus (AM) in 2012. Supposedly two simultaneous cases in the Amazon. The statement of a local politician in the assembly: “let’s shut up about that story.”
    http://g1.globo.com/am/amazonas/noticia/2012/03/homem-e-diagnosticado-com-suspeita-de-mal-da-vaca-louca-no-am.html
    http://al-am.jusbrasil.com.br/noticias/3068328/tony-medeiros-faz-esclarecimentos-sobre-mal-da-vaca-louca
    4. A woman that worked as a cook in Recife (PE), 54, admitted to the Hospital Tercentenary in Olinda, diagnosed with CJD in 2012
    http://www.diariodepernambuco.com.br/vidaurbana/nota.asp?materia=20120423182923

    http://www1.folhape.com.br/cms/opencms/folhape/pt/cotidiano/noticias/arquivos/2011/outubro/1010.html

    5. The engineer in Belo Horizonte (MG), 63, admitted to the Mother Teresa Hospital, diagnosed with CJD in 2012
    http://www.em.com.br/app/noticia/gerais/2012/04/05/interna_gerais,287392/engenheiro-com-sintomas-de-doenca-da-vaca-louca-esta-internado-em-bh.shtml
    6. The agricultural worker aged 27, of St John’s Paradise (MG), admitted to the Hospital Aroldo Tourinho in Montes Claros (MG), diagnosed with CJD in 2012. Received a corneal transplant, which is contemplated as a source of contagion.
    http://odia.ig.com.br/portal/brasil/minas-acompanha-pacientes-com-sintomas-de-doen%C3%A7a-do-mal-da-vaca-louca-1.429737
    7. A 42 y. o. mand and a 31 y.o. woman, both unrelated to each other and admited to the same hospital (interval of one month between admissions) at the University Hospital Onofre Lopes, both diagnosed with CJD in 2011. Before them, other six individuals were diagnosed with the disease since 2006 and admited to that very same hospital, what can be considered a cluster, such as Meixoeiro´s in Galicia (Spain).
    http://tribunadonorte.com.br/news.php?not_id=193394
    http://www.jeancarlos.com.br/index.php?option=com_content&view=article&id=4446:mal-da-vaca-louca-e-descartado-em-pacientes-de-natal&catid=1:blog&Itemid=1
    8. Claudia Correia, 51, Rio de Janeiro, diagnosed with CJD in 2012. Assisted by her family, at home. Biopsy confirmed CJD.
    http://terratv.terra.com.br/videos/Noticias/Brasil/4194-424616/Mulher-e-contaminada-com-mal-da-vaca-louca-no-Rio-de-Janeiro.htm
    9. Maria do Carmo L. Sena, retired, 74, died in 2013, diagnosed with CJD in 2012 in Brasília, DF. Even with police interfering it was not allowed to have an autopsy, allegedly because the authorities lacked infrastructure and there was the risk of contagion for the professionals involved.
    http://www.fabiocampana.com.br/2012/12/ministerio-deve-confirmar-exame-positivo-para-vaca-louca-no-parana/
    10. The farmer in Cajazeiras (PB), 66, admitted to the University Hospital of João Pessoa, diagnosed with CJD in 2012. According to the report, fed on “cow skin”, which is prohibited.

    http://www.paraiba.com.br/2012/12/19/84599-diretor-do-hu-de-jp-confirma-suspeita-de-mal-da-vaca-louca-demencia-progressiva
    http://saudeparaiba.blogspot.com.br/2012/12/mal-da-vaca-louca-na-paraiba.html
    11. E´s uncle. (Prefers to remain anonymous), in Curitiba (PR), diagnosed with CJD in 2013.
    12. Maria Cristina Lessa´s uncle, who died in Taubaté (SP), diagnosed with CJD in 2012, died in two months. Upon police request, an autopsy was performed.
    http://noticias.terra.com.br/mundo/brazil-no-radar/blog/2012/12/10/brasil-escondeu-casos-de-vaca-louca-diz-revista-americana/
    http://youtu.be/piGkw8ThRFM

    13. Ribamar C. Almeida´s brother,, diagnosed with CJD in Maranhão in 2012, died in March 2013.
    http://noticias.terra.com.br/mundo/brazil-no-radar/blog/2012/12/10/brasil-escondeu-casos-de-vaca-louca-diz-revista-americana/
    14. Adelia Amorim, 69, diagnosed with CJD in 2013 in Rio de Janeiro.
    http://oglobo.globo.com/rio/ancelmo/posts/2013/02/21/suspeita-de-mal-da-vaca-louca-assusta-cidade-do-rio-487100.asp

    • please see ;

      > but the possibility that a small proportion of human cases so far classified as “sporadic” CJD are of zoonotic origin could not be excluded.

      > Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

      >Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents.
      > This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

      >2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt->Jakob disease. They were not sure if it was due to mad sheep disease or a different form.

      >The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

      >Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.

      >These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

      >These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.

      >In summary, we have transmitted one atypical form of BSE (BASE) to a cynomolgus macaque monkey that had a shorter incubation period than monkeys infected with classical BSE, with distinctive clinical, neuropathological, and >biochemical features; and have shown that the molecular biological signature resembled that seen in a comparatively uncommon subtype of sporadic CJD.

      Thursday, August 12, 2010

      Seven main threats for the future linked to prions

      First threat

      The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

      ***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

      Second threat

      snip…

      http://www.neuroprion.org/en/np-neuroprion.html

      Monday, October 10, 2011

      EFSA Journal 2011 The European Response to BSE: A Success Story

      snip…

      EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far

      ***but the possibility that a small proportion of human cases so far classified as “sporadic” CJD are of zoonotic origin could not be excluded.

      Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

      snip…

      http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1

      http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf

      Thursday, August 15, 2013

      The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

      http://bse-atypical.blogspot.com/2013/08/the-emergence-of-novel-bse-prions-by.html

      Monday, September 02, 2013

      Atypical BSE: role of the E211K prion polymorphism

      Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

      Location: Virus and Prion Research Unit

      http://bse-atypical.blogspot.com/2013/09/atypical-bse-role-of-e211k-prion.html

      Sunday, September 1, 2013

      Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy

      We previously described the biochemical similarities between PrPres derived from L-BSE infected macaque and cortical MM2 sporadic CJD: those observations suggest a link between these two uncommon prion phenotypes in a primate model (it is to note that such a link has not been observed in other models less relevant from the human situation as hamsters or transgenic mice overexpressing ovine PrP [28]). We speculate that a group of related animal prion strains (L-BSE, c-BSE and TME) would have a zoonotic potential and lead to prion diseases in humans with a type 2 PrPres molecular signature (and more specifically type 2B for vCJD)

      snip…

      Together with previous experiments performed in ovinized and bovinized transgenic mice and hamsters [8,9] indicating similarities between TME and L-BSE, the data support the hypothesis that L-BSE could be the origin of the TME outbreaks in North America and Europe during the mid-1900s.

      http://transmissiblespongiformencephalopathy.blogspot.com/2013/09/evaluation-of-zoonotic-potential-of.html

      *** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

      Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

      *** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

      *** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

      Wednesday, January 01, 2014

      Molecular Barriers to Zoonotic Transmission of Prions

      *** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

      *** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

      http://wwwnc.cdc.gov/eid/article/20/1/13-0858_article.htm

      http://chronic-wasting-disease.blogspot.com/2014/01/molecular-barriers-to-zoonotic.html

      *** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

      http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf

      Thursday, January 2, 2014

      *** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

      http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/cwd-tse-prion-in-cervids-to-htgmice.html

      Wednesday, January 29, 2014

      Another Suspect case of Creutzfeldt-Jakob disease investigated in Brazil

      http://creutzfeldt-jakob-disease.blogspot.com/2014/01/another-suspect-case-of-creutzfeldt.html

      kind regards,
      terry

  2. Suspect case of Creutzfeldt-Jakob disease investigated in Brazil

    brain disease Brazil Creutzfeldt-Jakob disease Latin America and the Caribbean prion prion disease transmissible spongiform encephalopathies

    The death of a 70-year-old man from Belo Horizonte, Brazil is being investigated by health authorities as possibly being caused by the prion disease, Creutzfeldt-Jakob disease (CJD), according to a Wall Street Journal report Tuesday.

    According to the report, tissue samples are currently be analyzed to determine if the cause of death was CJD. Last year, health officials in the city investigated four other suspect cases; however, all tested negative for CJD.

    Creutzfeldt-Jakob disease (CJD) is a rare and fatal disease that affects the nervous system and causes deterioration of the brain. It affects about one in a million people each year worldwide. In the United States, only about 200 people are diagnosed with CJD each year.

    In the early stages of CJD, the patient may have rapidly failing memory and other cognitive difficulties. These symptoms become worse much faster than with other more common types of dementia, such as Alzheimer’s disease. Other symptoms can include personality changes including anxiety and depression, lack of coordination, and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements such as sudden jerky movements, blindness, weakness of extremities, and coma may occur. There is no treatment or cure.

    http://www.theglobaldispatch.com/suspect-case-of-creutzfeldt-jakob-disease-investigated-in-brazil-81276/

    >>> health officials in the city investigated four other suspect cases; however, all tested negative for CJD. …

    ???

    makes you wonder who’s doing the testing, and what test are being used ???

    tss

    Friday, December 07, 2012

    ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012

    http://bse-atypical.blogspot.com/2012/12/atypical-bse-brazil-2010-finally.html Wednesday, December 19, 2012

    Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil

    http://bse-atypical.blogspot.com/2012/12/scientific-report-of-european-food.html

    Sunday, December 15, 2013

    *** FDA PART 589 — SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE

    http://madcowusda.blogspot.com/2013/12/fda-part-589-substances-prohibited-from.html

    Wednesday, January 01, 2014

    Molecular Barriers to Zoonotic Transmission of Prions

    *** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

    *** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

    http://wwwnc.cdc.gov/eid/article/20/1/13-0858_article.htm

    http://chronic-wasting-disease.blogspot.com/2014/01/molecular-barriers-to-zoonotic.html

    Subtype 1: (sCJDMM1 and sCJDMV1)

    This subtype is observed in patients who are MM homozygous or MV heterozygous at codon 129 of the PrP gene (PRNP) and carry PrPSc Type 1. Clinical duration is short, 3‑4 months.32 The most common presentation in sCJDMM1 patients is cognitive impairment leading to frank dementia, gait or limb ataxia, myoclonic jerks and visual signs leading to cortical blindness (Heidenhain’s syndrome)…

    https://www.landesbioscience.com/pdf/06Ahmad_Liberski.pdf

    Animals injected with iatrogenic Creutzfeldt–Jakob disease MM1 and genetic Creutzfeldt–Jakob disease MM1 linked to the E200K mutation showed the same phenotypic features as those infected with sporadic Creutzfeldt–Jakob disease MM1 prions…

    http://brain.oxfordjournals.org/content/early/2010/09/07/brain.awq234.full.pdf

    *** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***

    http://www.jbc.org/cgi/content/abstract/M704597200v1?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=Cross-sequence+transmission+of+sporadic+Creutzfeldt-Jakob+disease+creates+a+new+&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

    snip…see full text ;

    http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/agent-strain-variation-in-human-prion.html

    Friday, January 17, 2014

    Annual report of the Scientific Network on BSE-TSE EFSA, Question No EFSA-Q-2013-01004, approved on 11 December 2013

    TECHNICAL REPORT

    http://efsaopinionbseanimalprotein.blogspot.com/2014/01/annual-report-of-scientific-network-on.html

    Sunday, January 19, 2014

    National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014

    http://prionunitusaupdate.blogspot.com/2014/01/national-prion-disease-pathology.html

    Thursday, January 23, 2014

    Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]

    http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/medical-devices-containing-materials.html

    Wednesday, January 15, 2014

    INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014

    http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/infection-prevention-and-control-of-cjd.html

    Sunday, August 11, 2013

    Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

    *** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010

    http://creutzfeldt-jakob-disease.blogspot.com/2013/08/creutzfeldt-jakob-disease-cjd-cases.html

    Sunday, October 13, 2013

    *** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

    http://creutzfeldt-jakob-disease.blogspot.com/2013/10/cjd-tse-prion-disease-cases-in-texas-by.html

    Saturday, November 16, 2013

    Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

    Infect Control Hosp Epidemiol.

    http://creutzfeldt-jakob-disease.blogspot.com/2013/11/management-of-neurosurgical-instruments.html

    Monday, December 02, 2013

    *** A parliamentary inquiry has been launched today into the safety of blood, tissue and organ screening following fears that vCJD – the human form of ‘mad cow’ disease – may be being spread by medical procedures

    http://creutzfeldt-jakob-disease.blogspot.com/2013/12/a-parliamentary-inquiry-has-been.html

    Wednesday, December 11, 2013

    Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

    http://creutzfeldt-jakob-disease.blogspot.com/2013/12/detection-of-infectivity-in-blood-of.html

    wasted days and wasted nights…Freddy Fender

    MOM DOD 12/14/97 confirm ‘hvCJD’ just made a promise to mom, NEVER FORGET! and never let them forget. …

    kind regards, terry

    layperson

    Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 [email protected]

    Wednesday, January 29, 2014

    Another Suspect case of Creutzfeldt-Jakob disease investigated in Brazil

    http://creutzfeldt-jakob-disease.blogspot.com/2014/01/another-suspect-case-of-creutzfeldt.html

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