Hong Kong reports probable case of sporadic Creutzfeldt-Jakob disease - Outbreak News Today | Outbreak News Today Outbreak News Today
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The Hong Kong Centre for Health Protection (CHP) recorded a probable case of sporadic Creutzfeldt-Jakob disease (CJD) on February 13, 2015, affecting a 65-year-old man with unremarkable past health.

Hong Kong/CIA

Hong Kong/CIA

He presented with dizziness since August 2014 and developed onset of cognitive decline since September 21, 2014. He was admitted to a public hospital on September 22, 2014. Subsequently, he developed rapidly progressive dementia, cerebellar disturbance and extrapyramidal dysfunction.

Findings from magnetic resonance imaging of the brain and electroencephalography were suggestive of CJD. He was classified as a probable case of sporadic CJD. He had no known family history of CJD. No risk factors for iatrogenic or variant CJD were identified.

His condition continued to deteriorate and he succumbed on January 5, 2015.

According to the National Institute of Neurological Disorders and Stroke, Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder.

CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year.

In the early stages of disease, people may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85 percent of cases.

CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).

5 Comments

  1. Thursday, July 24, 2014

    *** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigations

    http://bse-atypical.blogspot.com/2014/07/protocol-for-further-laboratory.html

    Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. *** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries. ***

    see page 176 of 201 pages…tss

    http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf

    *** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

    http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143

    ruminant feed ban for cervids in the United States ?

    31 Jan 2015 at 20:14 GMT

    http://www.plosone.org/annotation/listThread.action?root=85351

    Saturday, January 24, 2015

    Bovine Spongiform Encephalopathy: Atypical Pros and Cons

    http://bse-atypical.blogspot.com/2015/01/bovine-spongiform-encephalopathy.html

    Saturday, January 31, 2015

    RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings in countries with a “negligible risk status for BSE” and on the risk of modification of the list of specified risk materials (SRM) with regard to BSE

    http://bse-atypical.blogspot.com/2015/01/rapid-advice-17-2014-evaluation-of-risk.html

    2014

    ***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

    ***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.

    *** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

    snip…

    http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4213560/pdf/viruses-06-03766.pdf

    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 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

    Thursday, January 15, 2015

    41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE Prion: Case Report

    http://creutzfeldt-jakob-disease.blogspot.com/2015/01/41-year-old-navy-commander-with.html

    Saturday, January 17, 2015

    *** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease

    http://creutzfeldt-jakob-disease.blogspot.com/2015/01/becky-lockhart-46-utahs-first-female.html

  2. […] Pranab / June 5, 2015 A 65 year old man has reportedly succumbed to what is being stated to be sporadic Creutzfeldt Jakob disease according to a report in Outbreak News Today. […]

  3. Sunday, October 25, 2015

    USAHA Detailed Events Schedule – 119th USAHA Annual Meeting CAPTIVE LIVESTOCK CWD SCRAPIE TSE PRION

    http://chronic-wasting-disease.blogspot.com/2015/10/usaha-detailed-events-schedule-119th.html

    Sunday, October 18, 2015

    World Organisation for Animal Health (OIE) and the Institut Pasteur Cooperating on animal disease and zoonosis research

    http://bovineprp.blogspot.com/2015/10/world-organisation-for-animal-health.html

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