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Questions linger in U.S. CJD cases

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Questions linger in U.S. CJD cases
By STEVE MITCHELL
Senior Medical Correspondent

WASHINGTON, Oct. 21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical test widely regarded as the only way to determine precisely the nature of his disease, United Press International has learned.

The case of Patrick Hicks, who died last November from his condition, has remained murky from the beginning. Dr. Ron Bailey, of Riverside, Calif., the man's neurologist, had suspected the 49-year-old Hicks of having contracted variant Creutzfeldt Jakob disease -- a fatal, brain-wasting illness humans can contract from eating beef products contaminated with the mad cow pathogen -- and both he and the family wanted an autopsy conducted to determine if Hicks had succumbed to the disorder.

Bailey became concerned that Hicks might have contracted vCJD because he initially had exhibited psychiatric symptoms, his illness appears to have lasted for more than one year and he showed normal brain-wave patterns via EEGs until the late stages -- all consistent with the disease. In addition, Hicks's relatively young age raised concerns, because nearly all of the more than 150 cases of vCJD detected worldwide have occurred in people under age 55.

The first hint of oddness began when, according to both Hicks's brother and mother, a team of six doctors, who they suspect were with the Centers for Disease Control and Prevention in Atlanta, visited Patrick last October while he was still alive and under care at Loma Linda University Medical Center in Loma Linda, Calif.

They said they were asked to leave when the doctors arrived to examine Patrick.

CDC officials would not confirm to UPI whether they had investigated the case, but the agency's policy does require examining all suspected cases of vCJD in anyone under 55.

The family also said Loma Linda refused to released Hicks's medical records to them.

The oddities continued after Hicks's death. Bailey found it almost impossible to get an autopsy conducted on Hicks, the only way to determine conclusively whether he had variant or sporadic CJD -- a version of the disease not related to mad cow. One county coroner's office referred him to another and both refused to conduct the procedure, he said.

Then, the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio -- which was established by the CDC to investigate potential vCJD cases in the United States -- dispatched a mobile autopsy company called 1-800-Autopsy, but the company failed to follow the center's protocol and did not collect frozen sections of brain, which are required for tests to determine whether the disease is vCJD or sCJD. Instead, the autopsy company fixed the entire brain in formalin.

The NPDPSC, however, considers the collection of frozen brain tissue essential to distinguishing vCJD from other forms of CJD.

"Only frozen brain tissue examination definitely confirms or excludes the diagnosis of prion disease and provides the information to identify the type of prion disease," the center's Web site says. Prions are abnormal proteins thought to play a role in causing vCJD and sCJD.

The problem raised enough concern that both Bailey and Hicks's family sought a second opinion.

Experts had told them that animal-injection studies could be done with formalin-fixed tissue, so the family arranged to have a sample of Patrick's brain sent to Dr. Jean Jacques Hauw at the Laboratoire De Neuropathologie at the Groupe Hospitalier Pitie-Salpetriere in Paris, who they thought had agreed to do the studies.

The NPDPSC, however, delayed sending the sample to France for two months after the family's request last March. During the delay, Pierluigi Gambetti, the NPDPSC's director, sent a letter to Hicks's wife.

"We can definitely rule out the diagnosis of variant CJD," the letter stated.

Gambetti's strong conclusion sounded strange to Bailey, because the NPDPSC had not conducted further tests since January, when they had said vCJD was unlikely but that they were unable to rule it out entirely.

After examining the brain tissue, Hauw's team told the family the disease was consistent with sCJD, but to date they have not explained why they did not conduct the animal-injection studies -- the family's reason for sending samples of his brain to France.

Asked the reasons for not following the family's wishes and conducting the animal studies, Hauw told UPI, "I cannot answer your question," citing French regulations that prohibited him from providing information about a specific patient.

He did say, however, that "animal injection is not needed for the routine diagnosis of Creutzfeldt-Jakob disease and its various variants, at least in France and in the United Kingdom."

That may be true, but it remains unclear why he accepted the case in the first place, knowing that is what the family wanted.

Moreover, this was not a "routine diagnosis." If Hicks suffered from vCJD, he potentially would have been the first person in the United States to have acquired the disease domestically, a development with significant domestic and international ramifications.

In addition, other experts, such as Dr. Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, have said the only way to know conclusively whether the disease is due to sCJD or vCJD is through animal-injection studies.

"From what I gather, the result was merely rubber stamped," Bailey told UPI. "I guess we will never really know for sure."

The handling of the case is noteworthy, because the NPDPSC currently is investigating nine potential sCJD cases in Idaho. Experts suspect some of those cases could be vCJD.

Bailey and some patient advocates said they are now skeptical of the NPDPSC's behavior.

"How could my experience with the Hicks case ... and the interaction with NPDPSC not lessen my confidence?" Bailey asked. "I anticipate that all of the Idaho cluster of CJD patients will turn out to have sCJD. I cannot for a minute see their results indicating anything but this. After all, if any patient were to have vCJD, it would have been Patrick Hicks. The results of NPDPSC are not definitive in excluding Hicks as not having vCJD. There certainly will always be that question in my mind."

Terry Singletary, a patient advocate whose mother died of a form of the disease called Heidenhain variant, told UPI he likewise had lost confidence in the NPDPSC.

"I do not trust them," Singletary said. "It's all going to be sporadic. This is the way they want it. They do not want to find out all the routes and sources of this agent."

Both vCJD and mad cow disease are politically sensitive issues because they can impact international trade. Dozens of nations closed their borders to American beef after a lone U.S. cow tested positive for the disease in 2003, resulting in more than $4.7 billion in losses for the industry, and the U.S. Department of Agriculture delayed doing confirmatory tests for seven months on what turned out to be a second case of mad cow.

The NPDPSC did not respond to UPI's phone call requesting comment about the Idaho cases. The CDC referred UPI to Idaho officials.

Of the nine Idaho cases, three people have tested positive for a CJD-like illness, but officials are conducting further tests to determine whether the disease is sCJD. Two others tested negative and four were buried without autopsies.

The cases could just be a statistical fluke, but the state averages about 1.2 sCJD cases per year and has never had more than three in a single year. The disease is rare and generally is thought to occur at the rate of one case per million people.

Several CJD clusters in other states have far exceeded that rate, however. These included:

--southern New Jersey (2000-2003),

--Lehigh, Pa. (1986-90),

--Allentown, Pa. (1989-92),

--Tampa, Fla. (1996-97),

--Oregon (2001-02), and

--Nassau County, N.Y. (1999-2000).

Some of the clusters involved as many as 18 deaths, and ranged from a rate of four to eight cases per million people.

A group of J.P. Morgan analysts issued an advisory last year on the impact the clusters could have on the beef industry, and said that some of the cases could be due to vCJD.

"The existence of clusters raises the question of 'contamination' or 'infection,' and also raises the hypothesis that rather than cases of sCJD, these might have been cases of vCJD," the advisory said. "Given that sCJD occurs randomly in one out of 1 million cases, it is a statistical rarity to find an sCJD cluster -- let alone six."

If that assessment is accurate, another cluster in Idaho would be even more unlikely.

Another possibility is some of the Idaho cases could be due to chronic wasting disease, which is similar to mad cow disease and currently is epidemic among deer and elk in several states, including Idaho's neighbors Wyoming and Utah.

No human cases of CWD have ever been confirmed, but the disease has been shown to infect human cells in a lab dish. Also, a team of researchers led by Jason Bartz of Creighton University in Omaha, Neb., report in the November issue of the Journal of Virology they had experimentally transmitted CWD to squirrel monkeys --the first reported transmission of CWD to primates.

If CWD is capable of infecting humans, it is unknown whether the resulting disease would resemble sCJD, vCJD or a novel disorder. If the disease looks like sCJD, cases could be going undetected or misdiagnosed.

--

E-mail: [email protected]





© Copyright 2005 United Press International, Inc. All Rights Reserved


http://www.upi.com/HealthBusiness/view.php?StoryID=20051019-090103-6576




Questions linger in US CJD cases
Science Daily (press release) - 12 hours ago
... 21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical ...


http://www.sciencedaily.com/upi/index.php?feed=Science&article=UPI-1-20051021-21481300-bc-us-cjdcases.xml



Questions linger in US CJD cases
Middle East North Africa Financial Network, Middle East - 9 hours ago
21 (UPI) -- French researchers have ruled out the human form of mad cow disease in a deceased California man, even though they did not conduct the critical ...


http://www.menafn.com/qn_news_story.asp?StoryId=Cq1HNWeidDxmTy2PKy2fZzxm


a cluster forgotten ;

Creutzfeldt-Jakob Disease in Northeast Texas, J.A. Rawlings,*1 K.A.
Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of
Health, 1Austin and 2Tyler, Texas
Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform
encephalopathy, is caused by prions composed of proteinaceous material
devoid of nucleic acid. CJD occurs sporadically (generally 1
case/1,000,000 population per year) in older patients (average age of
65) and is characterized by rapidly progressive dementia, accompanied by
severe muscle spasms and incoordination. Death usually occurs within 3
to 12 months (average 7 months). CJD activity in Texas, which has a
population of nearly 19 million, appeared to be typical. The statewide
death rate for 1995 and 1996 was just under 1/1,000,000. In April of
1997, the Texas Department of Health became aware of an increased number
of possible CJD cases in a 23-county area of NE Texas with a population
of just over one million. After review of medical and pathology records,
four patients were identified with definite classic CJD and three were
identified with probable CJD. Dates of death for the eight patients were
from April, 1996 through mid-July 1997. The patients were from 46
through 65 years of age; four were male and three were female. A
case-control study to identify risks for CJD in NE Texas has been initiated.

http://www.jifsan.umd.edu/tse/Rawlings.htm




a few other cases of interest;


##################### Bovine Spongiform Encephalopathy #####################

CJD WATCH MESSAGE BOARD
TSS 2005
USA -- 28-year-old son, Joe, suffers from Creutzfeldt-Jakob
Thu Oct 20, 2005 13:15
70.110.83.65


Alexian closes Hospice
BY PATRICK CORCORAN
STAFF WRITER

Alexian Brothers Medical Health Center in Elk Grove Village is closing its hospice center -- the only one of its kind in the northwest suburbs -- and will make new accommodations for patients in the hospital, officials announced Friday.

But as the hospital network shifts away from traditional hospice care services, caught in the middle is at least one family that is dependent on the resources and services provided by Alexian Brothers' Hospice House, the only local hospice facility directly affiliated with a hospital.

The move means hospice care -- affecting about five patients on average -- will no longer be in a single, permanent location. Instead, Alexian Brothers will place terminally ill patients throughout the hospital, integrating them with patients who need similar levels of care and observation.

Josephine Mulczynski, whose 28-year-old son, Joe, suffers from Creutzfeldt-Jakob disease, a variant of mad cow disease that strikes humans, said the support provided by hospice care to her and her son is indispensable.

"My husband died 18 years ago. He went through hospice care. Without hospice, I wouldn't have made it. Now I am going through it again -- they offer so much support for the families and they are wonderful with the patient," she said.

Roger Johnson, Alexian Brothers CEO, said the change is predicated on his belief that hospice care -- which was developed in the mid-1970s -- is an outdated way of treating terminally ill patients.

"Today, rarely are patients told they have six months to live and when they do they want to know, 'How can I turn that six months into two or three years?'" he said. "More than dealing with end-of-life issues, patients want to research their illness, pursue clinical trials, undergo innovative procedures and become involved in aggressive treatment. They have a strong desire to tackle the disease. Hospice care does not provide the types of tracks that allow patients to address those needs."

The hospice unit can accommodate 10 patients, but rarely is it full. More often than not, there are five or fewer patients there, Johnson said.

Mulczynski said the comfort of hospice care, especially individualized attention, is often as valuable to family members as it is for the patient.

"As someone who is experienced in this, sometimes you feel like you are really losing it and it is stressful to be doing this all by yourself. Hospice will take him in for the weekend and it gives you a chance to get away for a little bit and try to get it together," she said.



http://www.pioneerlocal.com/cgi-bin/ppo-story/localnews/current/eg/10-20-05-718699.html



http://www.pioneerlocal.com/cgi-bin/ppo-story/localnews/current/he/10-20-05-720254.html



TSS

#################### https://lists.aegee.org/bse-l.html ####################



##################### Bovine Spongiform Encephalopathy #####################

Registered Nurse's request for help posted on CJD foundation . . . . . http://www.cjdfoundation.org/viewguestbook.asp



VIRGINIA



Posted : 10/17/2005 8:58:08 PM

Name : Wendy DeGray

E-Mail : [email protected]

Referred By: Search Engine

Location : Bluefield , Virginia

Comments :






"I know as an RN there are so many cases not being reported . I can find exact numbers but there is something very wrong here.Too many people have died of CJD in Bluefield Va.This doesn seem to be a "rare" disease they claim. We need to be checked out.Please help us. "



****************************************************************************************************


TSS

#################### https://lists.aegee.org/bse-l.html ####################


IN light of Asante/Collinge et al findings 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;

-------- Original Message -------- Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To:
"'[email protected]'"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.



I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.



Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

[email protected] (until 9/12/02)

New e-mail: [email protected] (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm


AND the new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease


http://www.pnas.org/cgi/content/abstract/0305777101v1


Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate;

http://www.pnas.org/cgi/content/full/041490898v1

Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471


ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally, of all ages. ...TSS
 

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