Creutzfeldt-Jakob Disease Surveillance System

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The Creutzfeldt-Jakob Disease Surveillance System (CJDSS) is a unit of the Public Health Agency of Canada. It studies the various variants of Creutzfeldt-Jakob Disease, and at least as of 2017, assisted "with DNA sequencing, autopsy and case confirmation". [1] As of 2014, the CJDSS conducted "prospective national surveillance for all types of human prion disease in Canada. The main purposes of the CJDSS [were then] to better understand the epidemiology of human prion diseases, to improve the options available for their rapid and accurate diagnosis, and ultimately to protect the health of Canadians by reducing risks of prion disease transmission." [2]

Contents

History

CJD deaths in Canada [3]
YearDeathsPopulationMortality
19982430,244,9820.79
19993230,492,1061.05
20003530,783,9691.14
20013031,130,0300.96
20023631,450,4431.14
20032931,734,8510.91
20044432,037,4341.37
20054432,352,2331.36
20064432,678,9861.35
20073933,001,0761.18
20084933,371,8101.47
20095333,756,7141.57
20103834,131,4511.11
20115134,472,3041.48
20126334,880,2481.81
20135135,289,0031.45
20145635,675,8341.57
20155235,702,7071.46
20166436,286,4001.76
20178636,712,6582.34
20188037,589,2622.13
20197837,802,0432.06

The CJDSS was created in 1998 (along with a slew of other OECD national reporting systems [4] [5] [6] ) "in response to the widespread outbreak of vCJD in the United Kingdom", [7] and "is a research project that relies on direct CJD reporting by all neurologists, neurosurgeons, neuropathologists, geriatricians, and infectious disease physicians to the" PHAC. [8]

The CJDSS issued its first annual report in 1998. [7]

In 2002, the CJDSS issued a report entitled "Infection Control Guidelines: Classic CJD in Canada". [9]

In July 2003 with the help of Agriculture and Agri-Food Minister Lyle Vanclief who is also an accredited Bachelor of Agriculture and active farmer, [10] [11] the SRM feed ban was imposed by the Canadian Food Inspection Agency (CFIA). [12] This was the first regulatory change to bovine farm practice in Canada after and as a result of the British BSE disaster. [11] [13] Feeding cattle to cattle had been practiced in Canada at least since 1992 [14] when the British culled at least 100,000 cattle in an attempt to control BSE. [15] Then in January 2004, the CFIA "announced that it would enhance its BSE surveillance testing to at least 8,000 cattle during the first year and to 30,000 per year in subsequent years to calculate the prevalence of BSE in Canadian adult cattle. The level and design of this enhanced program continues to be in full accordance with the guidelines recommended by the OIE." [14] As late as 2010, an audit report produced by external management consultant advisors from Ottawa told the Canadian government that the feed bans "will not show results for another five years." [12]

In 2004, the CJDSS, which had up until then been part of Health Canada's Population and Public Health Branch, was incorporated into the newly formed PHAC.[ citation needed ]

In 2007, the CJDSS issued a report entitled "Classic Creutzfeldt-Jakob Disease in Canada Quick Reference Guide 2007". [16]

In 2010, the CJDSS was evaluated, as part of the evaluation of the Prion Diseases Program of which it forms part. [12]

In 2011, a novel case of CJD that was detected in an immigrant patient from Saudi Arabia and noticed by the CJDSS in the Canada Communicable Disease Report, [17] prompted the Canadian Blood Services and Hema-Quebec to issue deferral orders for blood donors from the middle eastern country. [18]

In 2013, Coulthart et al. described at an academic conference Creutzfeldt-Jakob disease reporting in Canada. [19]

By 2014, "all human prion diseases [were] provincially reportable [at least in Newfoundland and Labrador] and nationally notifiable in Canada." [2]

In 2017, Fan, Deilgat and Speechley wrote that "the role of the CJDSS is to assist in the investigation and diagnosis of suspected cases of CJD in Canada, provide support to patients and health care providers, and ultimately protect the health of Canadians by reducing the risk of CJD transmission." [7]

In budget year 2019–20, the CJDSS advertised that it sought autopsy services in the National Capital Region. [20] [21]

Leadership

Related Research Articles

<span class="mw-page-title-main">Creutzfeldt–Jakob disease</span> Degenerative neurological disorder

Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis. The name "Creutzfeldt–Jakob disease" was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob.

<span class="mw-page-title-main">Fatal insomnia</span> Prion disease of the human brain

Fatal insomnia is an extremely rare neurodegenerative prion disease that results in trouble sleeping as its hallmark symptom. The majority of cases are familial, stemming from a mutation in the PRNP gene, with the remainder of cases occurring sporadically. The problems with sleeping typically start out gradually and worsen over time. Eventually, the patient will succumb to total insomnia, most often leading to other symptoms such as speech problems, coordination problems, and dementia. It results in death within a few months to a few years and has no known cure.

<span class="mw-page-title-main">Scrapie</span> Degenerative disease that affects sheep and goats

Scrapie is a fatal, degenerative disease affecting the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), and as such it is thought to be caused by a prion. Scrapie has been known since at least 1732 and does not appear to be transmissible to humans. However, it has been found to be experimentally transmissible to humanised transgenic mice and non-human primates.

<span class="mw-page-title-main">Transmissible spongiform encephalopathy</span> Group of brain diseases induced by prions

Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive, incurable, and fatal conditions that are associated with prions and affect the brain and nervous system of many animals, including humans, cattle, and sheep. According to the most widespread hypothesis, they are transmitted by prions, though some other data suggest an involvement of a Spiroplasma infection. Mental and physical abilities deteriorate and many tiny holes appear in the cortex causing it to appear like a sponge when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function which may result in memory loss, personality changes, and abnormal or impaired movement which worsen over time.

<span class="mw-page-title-main">Chronic wasting disease</span> Prion disease affecting the deer family

Chronic wasting disease (CWD), sometimes called zombie deer disease, is a transmissible spongiform encephalopathy (TSE) affecting deer. TSEs are a family of diseases thought to be caused by misfolded proteins called prions and include similar diseases such as BSE in cattle, Creutzfeldt–Jakob disease (CJD) in humans and scrapie in sheep. Natural infection causing CWD affects members of the deer family. In the United States, CWD affects mule deer, white-tailed deer, red deer, sika deer, elk, caribou, and moose. The transmission of CWD to other species such as squirrel monkeys and humanized mice has been observed in experimental settings.

The Public Health Agency of Canada is an agency of the Government of Canada that is responsible for public health, emergency preparedness and response, and infectious and chronic disease control and prevention.

John Mark Purdey was an English organic farmer who came to public attention in the 1980s, when he began to circulate his own theories regarding the causes of bovine spongiform encephalopathy.

<span class="mw-page-title-main">Variant Creutzfeldt–Jakob disease</span> Degenerative brain disease caused by prions

Variant Creutzfeldt–Jakob disease (vCJD), commonly referred to as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family. Initial symptoms include psychiatric problems, behavioral changes, and painful sensations. In the later stages of the illness, patients may exhibit poor coordination, dementia and involuntary movements. The length of time between exposure and the development of symptoms is unclear, but is believed to be years to decades. Average life expectancy following the onset of symptoms is 13 months.

<span class="mw-page-title-main">2008 US beef protest in South Korea</span>

The 2008 US beef protest in South Korea was a series of protest demonstrations made between 24 May 2008 and mid August 2008. against president Lee Myung-bak in Seoul, Korea. The protest involved several hundred thousand and at its height up to one million people. The protest began after the South Korean government reversed a ban on US beef imports. The ban had been in place since December 2003, when mad cow disease was detected in US beef cattle.

<span class="mw-page-title-main">Bovine spongiform encephalopathy</span> Fatal neurodegenerative disease of cattle

Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is an incurable and invariably fatal neurodegenerative disease of cattle. Symptoms include abnormal behavior, trouble walking, and weight loss. Later in the course of the disease, the cow becomes unable to function normally. There is conflicting information about the time between infection and onset of symptoms. In 2002, the World Health Organization (WHO) suggested it to be approximately four to five years. Time from onset of symptoms to death is generally weeks to months. Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). As of 2018, a total of 231 cases of vCJD had been reported globally.

The term Feed ban is usually a reference to the regulations that have prohibited the feeding of most mammalian-derived proteins to cattle as a method of preventing the spread of bovine spongiform encephalopathy (BSE). Feeding of infected ruminant material back to ruminants is believed to be the most likely means of transmission of the disease.

The National Prion Clinic (UK) is part of the University College London Hospitals NHS Foundation Trust. Its aim is to diagnose and treat patients with any form of human prion disease (Creutzfeldt-Jakob disease, CJD). In addition, the clinic facilitates research in diagnostics and therapeutics, organises clinical trials, and counsels those with an increased genetic risk of the disease.

<span class="mw-page-title-main">Variably protease-sensitive prionopathy</span> Medical condition

Variably protease-sensitive prionopathy (VPSPr) is a sporadic prion protein disease first described in an abstract for a conference on prions in 2006, and this study was published in a 2008 report on 11 cases. The study was conducted by Gambetti P., Zou W.Q., and coworkers from the United States National Prion Disease Pathology Surveillance Center. It was first identified as a distinct disease in 2010 by Zou W.Q. and coworkers from the United States National Prion Disease Pathology Surveillance Center.

<span class="mw-page-title-main">Sheila Bird</span> British statistician

Sheila Macdonald Bird OBE FRSE FMedSci is a Scottish biostatistician whose assessment of misuse of statistics in the British Medical Journal (BMJ) and BMJ series ‘Statistics in Question’ led to statistical guidelines for contributors to medical journals. Bird's doctoral work on non-proportional hazards in breast cancer found application in organ transplantation where beneficial matching was the basis for UK's allocation of cadaveric kidneys for a decade. Bird led the Medical Research Council (MRC) Biostatistical Initiative in support of AIDS/HIV studies in Scotland, as part of which Dr A. Graham Bird and she pioneered Willing Anonymous HIV Surveillance (WASH) studies in prisons. Her work with Cooper on UK dietary bovine spongiform encephalopathy (BSE) exposure revealed that the 1940–69 birth cohort was the most exposed and implied age-dependency in susceptibility to clinical vCJD progression from dietary BSE exposure since most vCJD cases were younger, born in 1970–89. Bird also designed the European Union's robust surveillance for transmissible spongiform encephalopathies in sheep which revolutionised the understanding of scrapie.

Frank O. Bastian is an American physician and neuropathologist, who previously worked at Louisiana State University, moved to a university in New Orleans in 2019. He specializes in the transmissible spongiform encephalopathies (TSEs), which include, but are not limited to, Bovine spongiform encephalopathy (BSE) "Mad cow disease" in cattle, scrapie in sheep and goats, and Creutzfeldt–Jakob disease (CJD) in humans.

<span class="mw-page-title-main">United Kingdom BSE outbreak</span> Mad cow disease outbreak in the 1980s and 90s

The United Kingdom was afflicted with an outbreak of Bovine spongiform encephalopathy, and its human equivalent variant Creutzfeldt–Jakob disease (vCJD), in the 1980s and 1990s. Over four million head of cattle were slaughtered in an effort to contain the outbreak, and 178 people died after contracting vCJD through eating infected beef. A political and public health crisis resulted, and British beef was banned from export to numerous countries around the world, with some bans remaining in place until as late as 2019.

Real-time quaking-induced conversion (RT-QuIC) is a highly sensitive assay for prion detection.

A neurological syndrome of unknown cause was identified as a potential novel degenerative disease in a cluster of individuals with similar clinical signs and symptoms in the Canadian province of New Brunswick beginning in 2019. Symptoms listed on the New Brunswick Public Health (NBPH) website include memory problems, muscle spasms, balance problems, difficulty walking or falls, blurred vision or visual hallucinations, unexplained or significant weight loss, behaviour changes, and pain in the upper or lower limbs.

Michael Coulthart is a Canadian microbiologist who is employed as the head of the Canadian Creutzfeldt–Jakob Disease Surveillance System (CJDSS) within the Public Health Agency of Canada (PHAC), which terms CJD a zoonotic and infectious disease. In 2006, a working group named "classic CJD" as well as Variant Creutzfeldt–Jakob disease as two notifiable diseases. It is unknown whether PHAC tracks in an official capacity other transmissible spongiform encephalopathies (TSE), but Coulthart is on the Advisory Committee of the Center for Infectious Disease Research and Policy for Chronic Wasting Disease of cervidae.

<span class="mw-page-title-main">Mad cow crisis</span> Health crisis in 1990s

The mad cow crisis is a health and socio-economic crisis characterized by the collapse of beef consumption in the 1990s, as consumers became concerned about the transmission of bovine spongiform encephalopathy (BSE) to humans through the ingestion of this type of meat.

References

  1. ↑ "Creutzfeldt-Jakob Disease: A Resource for Health Professionals" (PDF). British Columbia Provincial Health Services Authority. British Columbia Centre for Disease Control. 23 September 2017.
  2. 1 2 "Diseases Transmitted by Direct Contact and through the Provision of Health Care" (PDF). Newfoundland and Labrador Disease Control Manual. Disease Control Manual - Section 7. August 2014.
  3. ↑ "Creutzfeldt-Jakob disease surveillance system (CJDSS) report". Public Health Agency of Canada. 2021-04-14.
  4. ↑ Litzroth, Amber; Cras, Patrick; De Vil, Bart; Quoilin, Sophie (2015). "Overview and evaluation of 15 years of Creutzfeldt-Jakob disease surveillance in Belgium, 1998-2012". BMC Neurology. 15: 250. doi: 10.1186/s12883-015-0507-x . PMC   4668685 . PMID   26630984.
  5. ↑ Sun, Yu; Liu, Chih-Ching; Fan, Ling-Yun; Huang, Chung-Te; Chen, Ta-Fu; Lu, Chien-Jung; Guo, Wan-Yuo; Chang, Yang-Chyuan; Chiu, Ming-Jang (2020). ". Incidence of and Mortality Due to Human Prion Diseases in Taiwan: A Prospective 20-Year Nationwide Surveillance Study from 1998 to 2017". Clinical Epidemiology. 12: 1073–1081. doi: 10.2147/CLEP.S274093 . PMC   7569055 . PMID   33116901.
  6. ↑ "CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK" (PDF). The National CJD Research & Surveillance Unit Western General Hospital, Edinburgh, EH4 2XU. 2017.
  7. 1 2 3 Fan, E., Deilgat, M., Speechley, M. (2017). Investigating Suspected Outbreaks of Rare Infectious Disease Using Surveillance Data: The CJDSS Perspective. in: John-Baptiste, A. & McKinley, G. [eds] Western Public Health Casebook 2017. London, ON: Public Health Casebook Publishing.
  8. ↑ "Creutzfeldt-Jakob Disease - Classic and Variant" (PDF). Alberta Health. Public Health Disease Management Guidelines. May 2018.
  9. ↑ "Infection Control Guidelines: Classic CJD in Canada". Public Health Agency of Canada. Creutzfeldt-Jakob Disease Surveillance System. 2002. Archived from the original on 12 June 2017. Retrieved 30 April 2021.{{cite news}}: CS1 maint: bot: original URL status unknown (link)
  10. ↑ "Grad News". No. 28. University of Guelph Alumnus Magazine. Fall 1997.
  11. 1 2 Roos, Robert (24 July 2003). "Canada moves to shield food supply from BSE-infected material". CIDRAP.
  12. 1 2 3 "Evaluation of The Prion Diseases Program: 3.0 Relevance". Public Health Agency of Canada. 2010-06-15.
  13. ↑ "Bovine Spongiform Encephalopathy (BSE) in North America". Canadian Food Inspection Agency. 25 March 2011. Archived from the original on 9 May 2011.
  14. 1 2 "BSE Enhanced Surveillance Program". Canadian Food Inspection Agency. 2011-04-14. Archived from the original on 7 May 2011.
  15. ↑ "'Mad cow disease': What is BSE?". BBC . 18 October 2018. Retrieved 12 July 2019.
  16. ↑ "Classic Creutzfeldt-Jakob Disease in Canada Quick Reference Guide 2007". Public Health Agency of Canada. Creutzfeldt-Jakob Disease Surveillance System. 2007.
  17. ↑ Coulthart, Michael B.; Geschwind, Michael D.; Qureshi, Shireen; Phielipp, Nicolas; Demarsh, Alex; Abrams, Joseph Y.; Belay, Ermias; Gambetti, Pierluigi; Jansen, Gerard H.; Lang, Anthony E.; Schonberger, Lawrence B. (2016). "A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia". Brain. 139 (10): 2609–2616. doi:10.1093/brain/aww206. PMC   5082737 . PMID   27671029.
  18. ↑ "New vCJD Case in Canada Prompts CBS to Plan Saudi Arabia Residency Deferral" (PDF). America's Blood Centers. ABC Newsletter. March 18, 2011. Archived from the original (PDF) on April 30, 2021. Retrieved April 30, 2021.
  19. ↑ "Prion and Prion-like Diseases in Humans: Poster Abstracts". Prion. 7 (sup1): 54–80. 2013. doi:10.4161/pri.24865. PMC   4031668 . PMID   29095080.
  20. ↑ "Autopsy services for Creutzfeldt-Jakob Disease (1000210808)". No. 2019–05–27. Public Works and Government Services Canada. 2020-05-29. Retrieved 3 April 2021.
  21. ↑ "AVIS DE PROJET DE MARCHÉ (APM) No de la demande : 1000210808 Services d'autopsie pour la maladie de Creutzfeldt-Jakob" (PDF) (in French). Public Works and Government Services Canada. 31 July 2019.
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