The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD) Case Report

Case Report The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD)

Akiyo Shinde, 1 Takenobu Kunieda, 1 Yoshimi Kinoshita, 1 Reika Wate, 1 Satoshi Nakano, 1 Hidefumi Ito, 1 Masahito Yamada, 2 Tetsuyuki Kitamoto, 3 Yosikazu Nakamura, 4 Sadayuki Matsumoto 5 and Hirofumi Kusaka 1 1 Department of Neurology, Kansai Medical University, Moriguchi, 2 Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, 3 Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, Sendai, 4 Department of Public Health – Inquiry, Jichi Medical University, Shimotsuke, and 5 Department of Neurology, Kitano Hospital, Osaka, Japan Correspondence to Akiyo Shinde, md, Department of Neurology, Kansai Medical University, Fumizono-cho 10-15, Moriguchi 570-8506, Japan. Email: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:shindea@takii.kmu.ac.jp This case was presented in a preliminary form in the annual meeting of Japanese Neuropathological Association in Tokyo, 2008.

Copyright © 2009 Japanese Society of Neuropathology KEYWORDS latent period • periodic synchronous discharge • pulvinar sign • spongiform encephalopathy • variant Creutzfeldt–Jakob disease


ABSTRACT


Eleven years after a brief visit to some European countries, a 48-year-old Japanese man developed writing difficulty, irritability and general fatigue. Then he complained of dysesthetic pains in his legs, for which benzodiazepines were prescribed. However, at the time pulvinar sign was retrospectively confirmed on brain MRI. Eighteen months after the onset, his gait became ataxic with rapid deterioration of mental status over the following several months. Thirty-one months after the onset, he became akinetic and mute with periodic synchronous discharges on EEG, and died at the age of 51. The total clinical course was approximately 43 months. Pathological examination revealed the characteristic alterations of spongiform encephalopathy, severe in the thalamus, moderate but widely spread in the cerebral cortices, and moderate in the cerebellum. Abundant amyloid plaques were easily identified in the cerebral cortex and the cerebellum on HE staining. Immunohistochemistry for abnormal prion protein (PrPsc) confirmed amyloid plaques in several forms, such as florid, uni- and multi-centric plaques as well as perineuronal and periaxonal deposits in the basal ganglia and synaptic patterns in the thalami. A Western blotting study identified type 2B protease-resistant PrP. This is the first Japanese patient who was definitely diagnosed as variant Creutzfeldt-Jakob disease (vCJD). The pathological findings were similar to those of previous reports of vCJD in the UK. However, the changes were much more severe both in degree and distribution, probably due to a longer duration of the illness than those in the UK.

--------------------------------------------------------------------------------

Received 5 November 2008; revised and accepted 7 January 2009.

DIGITAL OBJECT IDENTIFIER (DOI) 10.1111/j.1440-1789.2009.01006.x About DOI




http://www3.interscience.wiley.com/journal/122241574/abstract?CRETRY=1&SRETRY=0



http://www.ncbi.nlm.nih.gov/pubmed/19389077?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum



----- Original Message -----
From: "TERRY SINGELTARY" <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:flounder9@VERIZON.NET>
To: <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:BSE-L@LISTS.AEGEE.ORG>
Sent: Tuesday, March 10, 2009 9:03 PM
Subject: [BSE-L] JAPAN-Local governments to carry on BSE testing despite subsidy cuts


-------------------- mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:BSE-L@LISTS.AEGEE.ORG --------------------

11/03/2009 00:54:58

Japan-BSE Testing.

JAPAN-Local governments to carry on BSE testing despite subsidy cuts

Every local government across the country with meat inspection facilities will continue to test all beef cows for mad cow disease during the next fiscal year, a Mainichi survey has found.

The finding comes despite the central government's abolition of about 200 million yen in annual subsidies to local governments for bovine spongiform encephalopathy (BSE) tests. Japan is the only country where all beef cows are tested for the disease.

In August 2005, the Health, Labor and Welfare Ministry deemed that there is no need for BSE tests on cows 20 months old or younger, on the grounds that no cow born before January 2002 has been found infected with BSE and that there is little chance of finding BSE in such young cows even if they have been infected.

Nevertheless, the ministry had extended subsidies to local governments conducting BSE tests on all beef cows until July last year.

Officials in charge at all 77 prefectural and municipal governments that have beef inspection facilities said they will continue BSE tests on all beef cows in fiscal 2009.

Among the reasons given was the need to "maintain the brand image of their locally produced beef" and "prevent confusion in the marketing process."

However, 30 government bodies said that there was no discussion on whether to continue testing. The survey also suggested that governments tend to abide by the policy of their peers and requests from local residents.

"It would take a lot of nerve to stop it while other prefectures are continuing it," said an official at the Akita Prefectural Government.

"We'd like to stop it but we can't gain support from local residents," a Miyagi Prefectural Government representative said. An official at the Yokohama Municipal Government said that the national government needs to take the initiative in convincing the public of the safety of beef.

The Toyohashi Municipal Government in Aichi Prefecture called on the national government to organize a nationwide BSE testing system. "Since beef is marketed in widespread areas, there is no point in conducting inspections on them unless they are coordinated.."

The government has also applied with the World Organization for Animal Health to raise its evaluation of Japan's BSE countermeasures from the lowest level of "a country whose BSE risk is unknown" to the middle level of "a country having a controlled BSE risk.."

Japan filed the application after it was decided to abolish a practice called "pithing" at all meat treatment centers across the country by the end of this fiscal year. In pithing, a wire is inserted into the cow's head to destroy the brains and spinal marrow and to prevent them from thrashing around. The practice is feared to raise the risk of BSE infections.

The government expects its application to be approved at a general meeting of the organization to be held in May this year.



http://www.farminguk.com/news/Japan-BSE-Testing.12958.asp



I applaud Japans effort to continue to try and eradicate BSE (TSE) i.e. mad cow from their herds. A far cry as to what the USDA has done here in the USA. they did just the opposite. the truth hurts sometimes when reality sets in $$$

WITHOUT a doubt, IF the USA, Canada, and Mexico can have a terribly flawed favorable rating, even though they are BSE GBR risk factor III, and even at that it was on flawed data, with all this, why not Japan being as controlled as the USA and North America ??? it's all about money is it not $$$ that's what Prusiner et al told the hearing committee in California ;

DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN

''they don't wanna know, the dont' care''



http://maddeer.org/video/embedded/prusinerclip.html



----- Original Message -----

From: TERRY SINGELTARY
To: mailto:BSE-L@LISTS.AEGEE.ORG
Cc:
Sent: Friday, March 06, 2009 4:27 PM
Subject: Risk of Introduction of BSE into Japan by the Historical Importation of Live Cattle from the United Kingdom

Journal of Veterinary Medical Science

Vol. 71 (2009) , No. 2 February pp.133-138

Risk of Introduction of BSE into Japan by the Historical Importation of Live Cattle from the United Kingdom

Katsuaki SUGIURA1), Toyoko KUSAMA1), Tomotaro YOSHIDA1), Naoki SHINODA1) and Takashi ONODERA2)

1) Food and Agricultural Materials Inspection Center 2) Department of Molecular Immunology, University of Tokyo

(Received 10-Mar-2008) (Accepted 3-Sep-2008)

ABSTRACT. All cattle imported from the United Kingdom to Japan since 1980 and slaughtered before 2002 were traced (n=33), and the number of cattle that were possibly infected with BSE and entered the animal feed chain was calculated. Because there was no effective system to avoid recycling of the BSE agent via animal feed until the early 1990s, of the 33 cattle imported from the UK into Japan, most probably 7 or 8 were infected and entered the animal feed chain, 2 of which entered the animal feed chain in each of 1992 and 1993. In terms of infectivity, 400-550 cattle oral ID50 of the BSE agent entered the feed chain in each of these years. The amount of infectivity that entered the feed chain in 1989, 1991 and 1995 was smaller but still substantial, suggesting that the BSE agent might have entered the Japanese feed chain in any of these years.

KEY WORDS: bovine spongiform encephalopathy (BSE), import risk analysis, Japan, live cattle, simulation

snip...



http://www.jstage.jst.go.jp/article/jvms/71/2/133/_pdf



REFERENCES



http://www.jstage.jst.go.jp/article/jvms/71/2/71_133/_cit



Greetings BSE-L members !

Because there was no effective system to avoid recycling of the BSE agent via animal feed until the early 1990s, of the 33 cattle imported from the UK into Japan, most probably 7 or 8 were infected and entered the animal feed chain, 2 of which entered the animal feed chain in each of 1992 and 1993. In terms of infectivity, 400-550 cattle oral ID50 of the BSE agent entered the feed chain in each of these years. The amount of infectivity that entered the feed chain in 1989, 1991 and 1995 was smaller but still substantial, suggesting that the BSE agent might have entered the Japanese feed chain in any of these years.<<< usa =" 496" canada =" 198" href="http://www.inspection.gc.ca/english/sci/ahra/bseris/bserise.pdf">http://www.inspection.gc.ca/english/sci/ahra/bseris/bserise.pdf



HERE is another look at all the imports for both the USA and Canada of UK live cattle and greaves exports ;

UK Exports of Live Cattle by Value 1986-96

USA 697 LIVE CATTLE

CANADA 299 LIVE CATTLE



http://www.bseinquiry.gov.uk/files/mb/m11f/tab11.pdf



UK EXPORTS OF MBM TO WORLD



http://www.bseinquiry.gov.uk/files/mb/m11g/tab05.pdf



OTHERS


SNIP...


*** SEE FULL TEXT ;



Risk of Introduction of BSE into Japan by the Historical Importation of Live Cattle from the United Kingdom



http://bseusa.blogspot.com/2009/03/risk-of-introduction-of-bse-into-japan.html



SEE FULL TEXT HERE ;

Tuesday, March 10, 2009

JAPAN-Local governments to carry on BSE testing despite subsidy cuts



http://madcowtesting.blogspot.com/2009/03/japan-local-governments-to-carry-on-bse.html



USA

Monday, April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)



http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathology.html




TSS