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From: TSS ()
Subject: TRACKING A KILLER DISEASE
Date: June 10, 2007 at 7:12 am PST

Posted on Sun, Jun. 10, 2007
Tracking a killer disease

Ghetti

Three months after his bill sank without a hearing, Rep. David Orentlicher’s
quest to require Indiana counties to report suspected cases of
Creutzfeldt-Jakob Disease to the state seems almost prescient.

As Michael Schroeder reported last Sunday, northeast Indiana public health
officials are so concerned about a sudden uptick in suspected cases of the
rare, fatal neurological disorder that they notified state and federal
authorities.

Whether or not the four cases in northeast Indiana are confirmed, the
questions they raise should encourage legislators to revisit the issue
during the next legislative session.

Orentlicher, an Indianapolis Democrat who is a physician and a lawyer, said
he plans to reintroduce the bill next session because he believes questions
and uncertainties that surround CJD merit closer attention.

The U.S. Centers for Disease Control and Prevention, which monitors public
health risks, doesn’t require state health departments to report suspected
CJD cases as it does for several other diseases. But several states,
including Wisconsin, North Dakota and Montana, require local health
officials to report CJD cases to state health officials.

“What we need to do is a systematic epidemiological study of CJD,” said Dr.
Bernardino Ghetti, a neuropathologist and researcher at the Indiana
University School of Medicine in Indianapolis. The CDC “says that there is
one case per million, but we really don’t know.”

Ghetti supports the bill, arguing that the more states that pass CJD
reporting bills, the easier it will be for researchers to study the disease
and perhaps begin to understand how it is acquired or transmitted.

Part of the difficulty of studying CJD is that it can only be definitively
diagnosed during an autopsy, Ghetti said, and fewer autopsies are being
conducted, in part because of the high cost. Orentlicher wonders whether
some cases diagnosed as Alzheimer’s disease while the patient was alive may
actually have been CJD, which could mean the incidence of the disease is
actually much higher.

Studying the disease is also complicated because it’s one of a group of
diseases caused by a form of protein called a prion. About 10 percent of CJD
cases result from a genetic flaw passed from generation to generation. Most
of the rest are Classic CJD – including the northeast Indiana cases – that
usually strikes older people. In the mid-1990s, British researchers
described a new form of the disease they called Variant CJD, which they
linked to consumption of beef from cattle that had bovine spongiform
encephalopathy or BSE – mad cow disease – the prion-related brain disease
that strikes cattle.

Though BSE is often diagnosed in cattle in Great Britain and Europe, there
have been just two confirmed cases in American cattle, and the U.S.
Department of Agriculture believes more extensive testing of American cattle
isn’t necessary.

Given the vast number of unanswered questions, requiring Indiana health
officials to report CJD cases may someday help unravel the mystery.

http://www.fortwayne.com/mld/journalgazette/news/editorial/17350284.htm

USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.


snip...

64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.


snip...

http://www.seac.gov.uk/minutes/95.pdf


3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western
Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than
classical BSE in humans.***


6:30 Close of Day One


http://www.healthtech.com/2007/tse/day1.asp


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html

There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535


BRITISH MEDICAL JOURNAL


BMJ


vCJD in the USA * BSE in U.S.
15 November 1999


http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406


BMJ


U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
well...
2 January 2000


http://www.bmj.com/cgi/eletters/320/7226/8/b#6117


JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.


Volume 3, Issue 8, August 2003, Page 463


“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD)
and have been searching for answers ever since. What I have found is that we
have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem.”
............................


http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf


see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408


sporadic CJD, the big lie

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276


TSS






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