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From: TSS (
Subject: Re: Patient with mad-cow-like brain ailment dies Gambetti said, he's pretty sure it's a prion disease
Date: August 20, 2004 at 2:21 pm PST

In Reply to: Patient with mad-cow-like brain ailment dies Gambetti said, he's pretty sure it's a prion disease posted by TSS on August 20, 2004 at 6:34 am:

-------- Original Message --------
Subject: Re: [CJDVoice] Re: Nation's experts work to identify mystery disease
Date: Fri, 20 Aug 2004 16:24:38 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy
References: <> <> <>


something about this reminded of something but i could not put
my finger on it, then i hit me. this bit about about trying to find
the identity of this misterious prion disease now in this person
from Alaska was pulled before during 'THE FEAST'. had to
do some digging but i don't think they never did come to a final
conclusion on those three. it went from CJD to PICKS and then
back to what? but one of those poor folks was from Alaska too.
hell, it's all spontaneous here in the USA and we don't have BSE/TSE
in the USA cattle herd either. maybe this is a new strain of
spontaneous/sporadic PICKS just in Alaska;-(TSS)

Subject: Fatal Degenerative Neurologic Illnesses in Men Who Participated
in Wild Game Feasts - Wisconsin, 2002
Date: Thu, 3 Apr 2003 15:58:57 -0600
From: "Terry S. Singeltary Sr."
Reply-To: BSE-L


Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild
Game Feasts - Wisconsin, 2002

from Morbidity & Mortality Weekly Report
Posted 04/02/2003


Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder in
humans. CJD is one of a group of conditions known as transmissible
spongiform encephalopathies (TSEs), or prion diseases, that are believed
to be caused by abnormally configured, host-encoded prion proteins that
accumulate in the central nervous tissue.[1] CJD has an annual incidence
of approximately 1 case per million population in the United States[1]
and occurs in three forms: sporadic, genetically determined, and
acquired by infection. In the latter form, the incubation period is
measured typically in years. Recent evidence that prion infection can
cross the species barrier between humans and cattle has raised
increasing public health concerns about the possible transmission to
humans of a TSE among deer and elk known as chronic wasting disease
(CWD).[2] During 1993-1999, three men who participated in wild game
feasts in northern Wisconsin died of degenerative neurologic illnesses.
This report documents the investigation of these deaths, which was
initiated in August 2002 and which confirmed the death of only one
person from CJD. Although no association between CWD and CJD was found,
continued surveillance of both diseases remains important to assess the
possible risk for CWD transmission to humans.
Case Reports

Case 1. In December 1992, a Wisconsin man aged 66 years with a history
of seizures since 1969 sought treatment for recurring seizures,
increasing forgetfulness, and worsening hand tremors.
Electroencephalographic (EEG) examination demonstrated focal
epileptiform activity and nonspecific diffuse abnormalities, but no
specific diagnosis was made. In February 1993, he was hospitalized for
increasing confusion, ataxia, and movement tremors of his extremities. A
magnetic resonance image (MRI) demonstrated mild, nonspecific
enhancement along the inferior parasagittal occipital lobe. A repeat EEG
showed bifrontal intermittent, short-interval, periodic sharp waves,
suggesting a progressive encephalopathy; a diagnosis of CJD was
suspected. The man died later that month; neuropathologic examination of
brain tissue during autopsy indicated subacute spongiform
encephalopathy, compatible with CJD.

The man was a lifelong hunter who ate venison frequently. He hunted
primarily in northern Wisconsin but also at least once in Montana. He
hosted wild game feasts at his cabin in northern Wisconsin from 1976
until shortly before his death. Fixed brain tissue obtained during the
autopsy was sent for analysis to the National Prion Disease Pathology
Surveillance Center (NPDPSC) and reexamined at the institution where the
autopsy was conducted. Histopathologic examination did not substantiate
the diagnosis of prion disease. In addition, 27 brain tissue sections
were negative for prions by immunostaining despite positive antibody
reactions against other proteins (controls), which indicated that other
epitopes in the tissue samples were preserved.

Case 2. In May 1999, a Minnesota man aged 55 years with no previous
history of a neurologic disease sought evaluation and treatment
following a 3-month history of progressive difficulty in writing and
unsteadiness of gait. A computerized tomography (CT) scan and MRI
examination of his head did not indicate any abnormality. In June 1999,
he was hospitalized following onset of dementia, speech abnormalities,
and myoclonic jerking. An EEG indicated left-hemispheric periodic sharp
waves and moderate generalized background slowing; CJD was diagnosed
clinically. In July 1999, following worsening symptoms and development
of right upper extremity dystonia, the patient died. Neuropathologic
evaluation of brain tissue during autopsy demonstrated widespread
subcortical spongiform lesions, consistent with CJD.

The man was not a hunter but had a history of eating venison. He made an
estimated 12 visits to the cabin where the wild game feasts were held,
but he participated in only one feast during the mid-1980s. Sections of
fixed and frozen brain tissue obtained during autopsy were analyzed at
NPDPSC, and prion disease was confirmed by immunohistochemical and
Western blot testing. The Western blot characteristics and prion disease
phenotype in this patient were consistent with the most common form of
sporadic CJD, classified as M/M (M/V) 1.[3] Subsequent genetic typing
confirmed the presence of methionine homozygosity (M/M) at codon 129 of
the patient's prion protein gene.

Case 3. In June 1992, a Wisconsin man aged 65 years sought treatment for
progressive slowing of speech, worsening memory, and personality
changes. By January 1993, his speech was reduced to one-word utterances.
Neurologic examination showed a flat affect, decreased reflexes, and
apraxia. A CT head scan showed mild atrophy, and an EEG was normal.
Pick's disease was diagnosed. By May, he was unable to perform any daily
living activities; he died in August 1993. Neuropathologic evaluation of
brain tissue during autopsy showed symmetrical frontal lobe cerebral
cortical atrophy and mild temporal lobe atrophy. No Pick's bodies or
spongiform lesions were observed.

The man had a history of eating venison and participated regularly in
wild game feasts held at the cabin owned by patient 1. He was a lifelong
hunter and hunted mostly in Wisconsin but also in Wyoming and British
Columbia. No game was brought to the wild game feasts from his hunting
trips outside of Wisconsin. Examination of fixed brain tissue sent to
NPDPSC demonstrated no lesions indicative of CJD, and
immunohistochemical testing with antibody to the prion protein did not
demonstrate the granular deposits seen in prion diseases.
Epidemiologic Investigation

Wild game feasts consisting of elk, deer, antelope, and other game that
occurred at a cabin in northern Wisconsin owned by patient 1 began in
1976 and continued through 2002. These feasts typically involved 10-15
participants and usually occurred on weekends before or during hunting
seasons in the fall and occasionally in the spring. Wild game brought to
these feasts usually were harvested in Wisconsin, but three men who
attended these feasts reported hunting in the western United States and
bringing game back to Wisconsin. These activities took place in Colorado
(near the towns of Cortez, Trinidad, Collbran, Durango, and Meeker),
Wyoming (near the towns of Gilette and Cody), and Montana (near the town
of Malta). CWD was not known to be endemic in these areas at the time
that these hunting activities took place.

Information was obtained for 45 (85%) of 53 persons who were identified
as possibly participating in the wild game feasts; all were male.
Information was obtained by direct interview or from family members of
decedents. Of the 45 persons, for whom information was obtained, 34 were
reported to have attended wild game feasts. Seven of the 34 feast
attendees were deceased, including the three patients. None of the four
other decedents had a cause of death attributed to or associated with a
degenerative neurologic disorder. None of the living participants had
any signs or symptoms consistent with a degenerative neurologic disorder.

Reported by:JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD, R Wierzba,
Div of Public Health, State of Wisconsin Dept of Health and Family Svcs.
P Gambetti, National Prion Disease Pathology Surveillance Center, Case
Western Reserve University, Cleveland, Ohio. L Schonberger, MD, R
Maddox, MPH, E Belay, MD, Div of Viral and Rickettsial Diseases,
National Center for Infectious Diseases; V Hsu, MD, EIS Officer, CDC.

Section 1 of 2 Continue

Bizarre link in Male Fatal Degenerative Neurologic Illnesses
from Morbidity & Mortality Weekly Report

Editorial Note

CWD was first described in the United States in the 1960s and classified
as a TSE in 1978. Previously localized to a contiguous endemic area in
northeastern Colorado and southeast Wyoming, since 2000, CWD has been
found in free-ranging deer or elk in Illinois, Nebraska, New Mexico,
South Dakota, Wisconsin, and outside the previously known endemic areas
of Colorado and Wyoming. CWD has been identified also in captive deer or
elk in Colorado, Kansas, Minnesota, Montana, Nebraska, Oklahoma, South
Dakota, and Wisconsin.[4] Because a variant form of CJD, with specific
neuropathologic and molecular characteristics that distinguish it from
sporadic CJD, has been associated with eating cattle products infected
with a prion that causes bovine spongiform encephalopathy,[5] concern
has been raised about the possibility that the prion associated with CWD
might be transmitted to humans in a similar way.

In this investigation, because only one of the three cases in Wisconsin
had neuropathologic confirmation of a prion disease, no association
could be made between case participation in the wild game feasts and the
development of CJD. Although patient 2 had confirmed CJD, he was
unlikely to have eaten CWD-infected venison at these feasts because
venison and other game from outside Wisconsin that was served at these
feasts did not originate from known CWD-endemic areas, and the man
participated in the feasts only once. In addition, the prion disease in
this case was consistent with the most common form of sporadic CJD,
without apparent unusual neuropathologic or molecular characteristics
that might occur if the prion related to CWD had been responsible for
the disease.

The findings in this report are subject to at least two limitations.
First, not all members participating in wild game feasts could be
identified, and not all persons listed as participating could be
contacted for interviews. Second, interviews that were conducted
required recall of events that occurred up to 25 years ago, limiting the
detail or accuracy of events. However, the similar responses obtained
from different sources support the accuracy of the investigation findings.

A previous investigation of unusually young CJD patients in whom the
transmission of CWD was suspected also did not provide convincing
evidence for a causal relationship between CWD and CJD.[2] However,
limited epidemiologic investigations cannot rule out the possibility
that CWD might play a role in causing human illness. Ongoing
surveillance of CJD, particularly in states with CWD, is important to
assess the risk, if any, for CWD transmission to humans. Because the
confirmation of CJD and the detection of a new prion disease require
neuropathologic study of brain tissue, physicians are encouraged to
contact NPDPSC (; telephone, 216-368-0587)
to confirm diagnoses of CJD and to distinguish its various subtypes.
Because of the known severity of TSEs in humans and the possibility that
the CWD prion can affect humans, animals with evidence of CWD should be
excluded from the human food or animal feed chains. Hunters and wild
venison consumers should follow precautionary guidelines available from
the Wisconsin Department of Agriculture, Trade, and Consumer Protection
( to prevent potential
exposures to the CWD agent.

Section 2 of 2 Previous

MMWR 52(7):125-127, 2003. © 2003 Centers for Disease Control and
Prevention (CDC)



Date: Tue, 08 Apr 2003 09:01:22 -0500
From: "Terry S. Singeltary Sr."

Hunters' deaths invite theories about disease


Since late 2001, a human brain-wasting disease, similar to mad cow
disease, has killed at least three deer and elk hunters with links to
Washington state.

Two of the men were acquaintances from the same Washington town. The
third was an Alaska man, with no known connection to the other two, who
was treated at a Seattle hospital. Doctors would not release the men's
names or home towns for privacy reasons.

All three died from Creutzfeldt-Jakob disease, or CJD, a fatal illness
thought to be caused by prions: rogue proteins concentrated in the
nervous system. Doctors think prions also cause mad cow disease and
chronic wasting disease, a similar illness in mule deer, white-tailed
deer and elk. All three diseases open small holes in brain tissues and
so are called "spongiform encephalopathies" -- sponge-like brain diseases.

Many scientists think that people can get these brain-wasting diseases
by eating beef from cattle with mad cow disease. Similarly, some think
that eating venison from animals with chronic wasting disease could
cause CJD-like illness in people. No biologists or doctors have proved
these theories.

Doctors who treated the Alaskan and one Washington man said autopsies
revealed CJD. But there is no way to tell from the hunters' bodies
whether their diseases came from eating venison affected with wasting
disease, they said.

None of the animals killed by the Washington friends were tested for
wasting disease. It's not clear what areas they hunted in or whether
they ever shared a venison meal, said Dr. Natalia Murinova, a
neurologist with the University of Washington and Seattle Veterans
Administration Medical Center.

She and Dr. Ali Samii said they are discussing the cases, which they
presented last week at a neurology conference in Hawaii, to make both
doctors and hunters more aware of the possibility that prion diseases
can spread through eating venison. That way, doctors treating hunters
with dementia can know to consider a possible prion disease, and doctors
treating CJD patients will know to ask whether they ate venison.

"We are not saying there's a direct link between this," disease and
eating venison, Samii said. "We're just saying we should pay attention
to this issue."

Samii said one Washington man came to the Seattle VA in September 2001,
and the Alaska man in March 2002. Both men had the hallmarks of CJD, he
said: a "rapidly progressing form of dementia" and trouble moving, which
eventually confined and killed them. Autopsies revealed they had CJD.
Murinova said the other Washington man also had his case confirmed by

Creutzfeldt-Jakob disease is very rare, hitting about one person in
every million. But Murinova said "there is a significant increase in
incidence" in the United States since 1997. The increase is unexplained,
and may not be linked to meat, either beef or venison. "It might just be
that physicians are more aware" of CJD after mad cow disease hit Britain
in the late 1990s, and are sending more brain biopsies for diagnosis,
she said.

There is no biological proof that mad cow disease spreads to humans,
Murinova said. The evidence that beef can cause a human illness, "new
variant" CJD, is based on more than 130 cases of the disease found in
U.K. residents following the 1990s outbreak of mad cow disease that
infected more than 175,000 British cattle. The U.S. Centers for Disease
Control says "strong epidemiologic and laboratory evidence" links eating
the infected beef with new variant CJD cases.

Not all prion diseases pass between species. Humans seem immune to the
sheep prion disease scrapie, for instance. The pressing question for
hunters and scientists is whether prions can cross the "species barrier"
from deer or elk to people, Murinova said.

While that seems unlikely, two friends getting CJD is also very
uncommon, said Marcia Goldoft, an epidemiologist with the Washington
State Department of Health.

"Rare things do happen," she said. "The question is, which of the rare
things is this?"

To be safe, Murinova said deer and elk hunters can take advantage of
programs that test samples of nerve tissue from deer and elk. Both
Oregon and Washington run such programs, at no charge to hunters,
through their Fish and Wildlife departments.

Washington and Oregon game officials said they had not heard of the
hunters' deaths. But they emphasized that no proof exists that chronic
wasting disease can pass to humans.

"A lot of research is going on, as far as the link between chronic
wasting disease and Creutzfeldt-Jakob disease, and so far they're really
struggling to find any evidence of any kind of connection," said Kristin
Mansfield, a veterinarian with Washington's Fish and Wildlife
department. She noted that Colorado, which has many confirmed cases of
wasting disease in animals, has lower-than-average incidence of CJD.

Wasting disease has never been found in Oregon or Washington animals,
despite hundreds of tests.

Jerry Nelson, Washington's deer and elk section manager, said the state
tested 785 samples of deer or elk brain tissue taken in 2001. Washington
officials took more than 1,000 samples for testing last year. He has
results back for about half of those samples, he said, "and they're all

Oregon biologists sent 832 samples gathered last year to a Colorado lab
for testing, and "we're expecting to hear at any time" what the results
are, said Richard Green, who handles sample testing for Oregon's
wildlife department.

Biologists have found chronic wasting disease in wild elk or deer in
Colorado, Illinois, Nebraska, New Mexico, South Dakota, Wisconsin,
Wyoming and the Canadian province of Saskatchewan, according to the
Chronic Wasting Disease Alliance, a coalition of deer- and
elk-conservation groups.

Wasting disease has also struck farmed elk in Colorado, Kansas,
Minnesota, Montana, Nebraska, Oklahoma, South Dakota, Wisconsin and the
provinces of Alberta and Saskatchewan, according to the alliance.


Terry S. Singeltary Sr. wrote:

> ######## Bovine Spongiform Encephalopathy
> #########
> greetings,
> that seattle times article was most unclear. was there prion deposits
> detected by antibody or not? genetics done? what was unusual about
> ihc? in
> my opinion gambetti is on very thin ice offering risk reassurances
> about a
> new strain type. esp for surgical tools used brain to brain.
>>> previously expected that this would rise to as many as 3,800. This
>>> estimate was derived from examinations of appendix and tonsil tissues
>>> removed from 12,674 people during routine surgery, of which three were
>>> found to contain abnormal prion proteins. However, this estimate
>>> assumed
>>> that only those with the less common set of markers were susceptible.
> Indeed, this case would have been missed not only for its met/val at 129
> but also because appendix and tonsil were negative. So 40% are met met,
> because 2.5 x 40% = 100%, need to consider 2.5 x 3800= 9500, then perhaps
> double that to allow for ones where tonsils and appendix are false
> negaitves, = 19,000. and then start thinking about how many of these
> 19,000
> have given blood etc.
>>> besides prion markers. The research indicates that there are at least
>>> seven genes affecting the susceptibility of mice, and Collinge said
>>> that
>>> it would be ìpretty surprisingî if it were not also the case in humans.
> right. in other words, the early met met had other genetic risk factors
> that made them early onset. so there will be more met met later. but not
> in the USA if the CDC/NIH et al have anything to do with it, kinda like
> the mad cow in TEXAS that was rendered to cover-up after testing was
> refused.
> Terry S. Singeltary Sr. wrote:
>>> But Gambetti said the risk to those patients is slight.
>>> "The chances that this disease is transmissible are very, very low," he
>>> said.
>> ???
>> 1: Brain. 1990 Dec;113 ( Pt 6):1891-909.
>> Spongiform encephalopathy transmitted experimentally from
>> Creutzfeldt-Jakob and familial Gerstmann-Straussler-Scheinker diseases.
>> Baker HF, Duchen LW, Jacobs JM, Ridley RM.
>> Division of Psychiatry, MRC Clinical Research Centre, Harrow,
>> Middlesex, UK.
>> A comparison was made of the effects of experimental intracerebral
>> inoculation into marmosets of brain homogenates from a case of
>> Creutzfeldt-Jakob disease (CJD) and from a member of the Wo. family with
>> cerebral amyloid and spongiform encephalopathy--the
>> Gerstmann-Straussler-Scheinker (GSS) syndrome. All the inoculated
>> marmosets developed spongiform encephalopathy (SE) after incubation
>> times of 20-23 months in the CJD group and 25-32 months in the GSS
>> group. Subsequent passage from 1 affected animal in each group resulted
>> in SE developing after 17 months incubation. In every animal inoculated
>> with CJD or GSS material and in the 2 passage experiments the most
>> severely affected region of the brain was the thalamus which in all
>> cases was almost totally occupied by vacuoles. Other grey matter masses
>> were less severely and less consistently affected. Vacuolation was
>> observed in the cerebellar granule cell layer as well as in the
>> molecular layer and the brain stem was finely vacuolated in all cases.
>> There were only minor and inconsistent differences between the disease
>> transmitted from CJD compared with GSS and some differences between the
>> original transmissions and the SE caused by passaged inocula. Severe
>> astrocytic gliosis accompanied the spongiform changes but no amyloid was
>> identified in any of the marmosets with experimentally transmitted
>> disease. The pathogenesis of the spongiform change in the thalamus was
>> studied in a series of marmosets by light and electron microscopy 3-22
>> months after the intracerebral inoculation of CJD or GSS homogenates and
>> was compared with controls. Dilated irregularly-shaped cisternae and the
>> large complex vacuoles typical of SE, present in abundance after 18 and
>> 22 months incubation, were considered most probably to be derived from
>> cisternae of neuronal smooth endoplasmic reticulum.
>> Publication Types:
>> * Case Reports
>> Transfusion
>> Volume 43 Issue 12 Page 1687 - December 2003
>> doi:10.1046/j.0041-1132.2003.00586.x
>> Similar levels of infectivity in the blood of mice infected with
>> human-derived vCJD and GSS strains of transmissible spongiform
>> encephalopathy
>> Larisa Cervenakova, Oksana Yakovleva, Carroll McKenzie, Svetlana
>> Kolchinsky, Lisa McShane, William N. Drohan, and Paul Brown
>> The possible transmission of variant CJD (vCJD) through blood
>> transfusion or use of plasma-derived products prompted this study
>> comparing infectivity in murine models of vCJD and
>> Gerstmann-Sträussler-Scheinker (GSS) disease, a non-vCJD form of
>> transmissible spongiform encephalopathy (TSE).
>> RIII/Fa/Dk (RIII) or Swiss-Webster (Swiss) mice were inoculated
>> intracerebrally (IC) with mouse-adapted strains of vCJD or GSS
>> (Fukuoka-1) of similar infectivity. Groups of RIII mice were euthanized
>> 17 weeks after inoculation (during the incubation period), and another
>> 23 weeks after inoculation (when symptomatic). Blood was collected,
>> separated into components, and inoculated into groups of healthy mice;
>> brains and spleens from all mice were harvested and tested for the
>> presence of PrPres by Western blot using 6H4 MoAb.
>> Levels of 20-30 infectious doses per mL were present in buffy coat and
>> plasma during both the incubation and symptomatic stages of disease; PLT
>> pellet infectivity was lower (10 ID/mL) and RBCs were not infectious.
>> The disease was transmitted more efficiently by IV than IC inoculation
>> of plasma, but there was no difference observed with inoculation of
>> buffy coat. The incubation period was shorter after IC inoculation of
>> GSS- than vCJD-brain inocula. The amount of PrPres in spleens was
>> similar for both TSE agents, but was slightly lower in brains of vCJD
>> than GSS mice.
>> Infectivity was detected in blood components of mice infected with a
>> human-derived strain of vCJD during both the preclinical and clinical
>> phases of disease in a similarly low range of concentrations as in mice
>> infected with a human-derived nonvariant strain (GSS, Fukuoka-1). Other
>> measures of virulence, including brain infectivity titers, incubation
>> periods, and the accumulation of PrPres in spleens and brains, were also
>> comparable in both experimental models.
>> TSS
>> Terry S. Singeltary Sr. wrote:
>>> Wednesday, August 18, 2004 - Page updated at 12:00 A.M.
>>> Nation's experts work to identify mystery disease
>>> By Sandi Doughton
>>> Seattle Times staff reporter
>>> The nation's top experts in mad-cow-like diseases are trying to
>>> identify
>>> a strain discovered this summer in a patient at Harborview Medical
>>> Center in Seattle.
>>> The disease is clearly not the human form of mad-cow, nor does it
>>> appear
>>> to be Creutzfeldt-Jakob Disease (CJD), a closely related condition,
>>> said
>>> Dr. Pierluigi Gambetti, director of the National Prion Disease
>>> Pathology
>>> Surveillance Center.
>>> "We really are puzzled at this time," he said yesterday. "This is
>>> unusual."
>>> The condition of the patient was not available.
>>> Blood tests conducted over the next two to three weeks should provide
>>> more information on the mysterious ailment, said Gambetti, whose
>>> Cleveland lab analyzes most of the so-called prion diseases
>>> diagnosed in
>>> the United States.
>>> Prions are the misshapen proteins believed to cause a family of deadly,
>>> brain-wasting diseases that include human forms such as CJD and animal
>>> versions such as mad-cow and scrapie. Scientists think the human
>>> form of
>>> mad-cow disease, also called variant CJD, is probably caused by eating
>>> infected beef. But the causes of other prion diseases are not
>>> well-understood, though genetic factors play a role in some cases.
>>> Because they are proteins, not microbes, prions are resistant to most
>>> ordinary sterilization techniques. Surgical instruments used to take a
>>> biopsy from the Seattle patient's brain were used subsequently in up to
>>> 12 other brain surgeries before being subjected to the supercleaning
>>> prescribed for prion diseases, hospital officials said.
>>> Human prion disease
>>> There are five known human prion diseases:
>>> Sporadic Creutzfeldt-Jakob disease is believed to arise
>>> spontaneously in
>>> one person in a million and is more common in the elderly. Some cases
>>> also have been caused by contaminated surgical instruments or
>>> contaminated brain implants, corneal transplants and blood transfusion.
>>> Variant Creutzfeldt-Jakob disease is believed to be caused by eating
>>> beef infected with mad-cow disease and mainly strikes young people.
>>> Kuru was discovered in New Guinea, where it was spread by the practice
>>> of cannibalizing human brains.
>>> Fatal Familial Insomnia, marked by progressive sleeplessness, is
>>> genetically linked and has been found only in nine extended families.
>>> Gerstmann-Straüssler-
>>> Scheinker also is caused by genetic mutations and occurs in only about
>>> 50 extended families.
>>> Sources: CDC; Medscape
>>> But Gambetti said the risk to those patients is slight.
>>> "The chances that this disease is transmissible are very, very low," he
>>> said.
>>> The hospital is reviewing its records and will decide whether the
>>> patients should be notified once the disease is positively identified,
>>> said Johnese Spisso, Harborview's chief operating officer.
>>> "We need to sit down and review all the facts so we know what to tell
>>> people," she said. "We need to make sure we give them the best
>>> information possible so we don't unduly alarm people."
>>> There are only six cases on record of a prion disease  specifically
>>> CJD
>>>  being transmitted through contaminated surgical instruments or brain
>>> electrodes, said Dr. Ermias Belay of the U.S. Centers for Disease
>>> Control and Prevention. And all of those cases occurred before 1976,
>>> when common sterilization techniques were improved, said Belay, who
>>> helped write international guidelines for prion sterilization.
>>> It's possible the strain from Harborview is a type that can't be passed
>>> from one person to another, even through a direct dose to the brain,
>>> Gambetti said.
>>> Preliminary tests show that it most closely resembles an extremely rare
>>> prion disease named GSS for the three German scientists who discovered
>>> it (Gerstmann-Straüssler-Scheinker). GSS can be triggered by a dozen
>>> different genetic mutations, Gambetti said, and only one of those forms
>>> has been shown to be transmissible in laboratory animals.
>>> But even if the risk is minuscule, the diseases are so horrible that
>>> hospitals always should err on the side of caution when it comes to
>>> sterilizing instruments, said Florence Kranitz, president of the CJD
>>> Foundation, a support and advocacy group.
>>> Invariably fatal, CJD and its close cousins progressively destroy the
>>> brains of their victims, robbing them of their memories and their
>>> ability to move or speak.
>>> "I sure don't want them doing surgery on anyone I know and love with
>>> instruments that have been contaminated by prions," Kranitz said.
>>> The CDC recommends that hospitals superclean instruments used for brain
>>> surgery on all patients where prion diseases haven't been ruled out,
>>> Belay said. Another option is to "quarantine" the instruments, not
>>> using
>>> them again until a final diagnosis eliminates the possibility of prion
>>> disease.
>>> But Harborview officials say two preliminary tests on the patient were
>>> negative for CJD, which, though it strikes only about 300 Americans a
>>> year, is the most common human prion disease.
>>> "In the opinion of the neurologist, this was not CJD," said Dr. Tom
>>> Montine, the hospital's director of neuropathology.
>>> So the hospital used its normal sterilization techniques: Soaking the
>>> instruments in an enzyme solution, followed by a high-pressure wash and
>>> a 272-degree vacuum autoclave.
>>> Two weeks later, when the patient's biopsy results hinted at the
>>> possibility of a prion disease, the hospital supersterilized the
>>> instruments according to CDC's guidelines, which include an hourlong
>>> soak in sodium hydroxide  the powerful caustic also called lye 
>>> before
>>> the high-temperature autoclave.
>>> "We feel confident we followed the recommended national sterilization
>>> standards based on the patient's clinical information," said Harborview
>>> spokeswoman Susan Gregg-Hanson.
>>> She said the hospital is reviewing the case and may change its
>>> procedures if the review finds flaws.
>>> Similar cases over the past few years have raised concern about
>>> sterilization techniques in the U.S., Canada and the United Kingdom.
>>> Doctors at a Denver hospital operated on six people in 2001 with the
>>> same instruments used to take a brain biopsy from a woman who later
>>> died
>>> of CJD. Two of the patients are suing the hospital.
>>> Several patients settled lawsuits filed against Tulane University
>>> Hospital in New Orleans after a similar occurrence in 2000 may have
>>> exposed eight people to CJD.
>>> In 2001, Madigan Army Medical Center in Pierce County closed its
>>> operating rooms two days for extra sterilization after discovering a
>>> woman who had undergone surgery there four months before had CJD. The
>>> hospital notified five people who might have been exposed.
>>> Britain, where a mad-cow epidemic wiped out herds and infected more
>>> than
>>> 100 people, is spending more than $350 million to upgrade sterilization
>>> equipment.
>>> Sandi Doughton: 206-464-2491 or
>>> Copyright © 2004 The Seattle Times Company
>>> TSS
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