Emory October 12th Press Release
Emory October 1st Press Release
Creutzfeldt-Jakob Disease Information
National Laboratory Confirms Rare Diagnosis of Creutzfeldt-Jakob Disease
A national laboratory in Cleveland, Ohio, has confirmed the CDC's preliminary diagnosis of an extremely rare condition
known as Creutzfeldt-Jakob disease (CJD) following the September 10 brain biopsy of a patient at Emory University Hospital.
Emory officials emphasized the extreme unlikelihood of any other patients being affected by this rare case.
In the medical literature, there has not been a single reported case of CJD transmitted by surgical instruments
in the nearly three decades since 1976 and the widespread adoption of modern surgical sterilization techniques.
Those sterilization techniques have been standard procedure at Emory for all operations for three decades and were followed after the original brain biopsy in this case.
CJD, a degenerative brain disease, occurs in the population worldwide at the rate of about one person per million per year for reasons that are usually unknown. The illness is not the variant condition known popularly as "mad cow disease."
The hospital said it shared the laboratory findings with surgical patients first before making them public.
In the wake of the initial preliminary CDC finding, the Hospital re-sterilized all of its neurosurgical instruments to an enhanced standard recommended by some national and international authorities for cases of CJD. Later, after a continuing review of the situation, the Hospital re-sterilized all of its surgical instruments to the same enhanced standard. Hospital officials say the re-sterilization of all surgical instruments appears to exceed what other hospitals have done in the same situation.
Emory officials have advised 98 brain and spine surgery patients who were operated on following the biopsy that the Hospital is seeking written guidance from an advisory committee of the Department of Health and Human Services on whether they should be blood donors in the future. If HHS advises against donation, the surgical patients would fall in the same extremely low-risk deferred-donor pool as persons who spent three months or more in the United Kingdom between 1980 and 1996, or members of the Armed Services who resided at U.S. military bases in Northern Europe for six months or more between 1980 and 1996.
"We continue to seek the best available guidance and information from experts at other medical centers and from agencies such as CDC and HHS," said William Bornstein, MD, PhD, chief quality officer for Emory Healthcare. "The extreme rarity of this disease and the nature of the potential exposure, which is so small that it cannot be quantified, make it challenging to find published guidelines which are applicable to our situation. Nevertheless, we are fully committed to finding and sharing with our patients the best available information."
Emory Hospital Suspects Case of Rare CJD: Notifying Surgical Patients
Emory officials believe Creutzfeldt-Jakob disease (CJD), an
extremely rare degenerative disease, is the probable diagnosis of an Emory
University Hospital patient following a brain biopsy, and are taking steps
on the basis of that assumption. Given the rarity of this disease, the
definitive test is done by a national laboratory in Cleveland, Ohio. Due to
time requirements and the complexity of analysis, the results are not
expected for several weeks.
Nevertheless, the Hospital is in the process of notifying 98 brain and spine
surgery patients of the remote possibility they may have been exposed to the
protein that causes CJD, a very rare disease that occurs at the rate of only
one person per million per year worldwide.
Emory physicians said potential exposures might have occurred following the
September 10 brain biopsy of a patient who later received a preliminary
diagnosis of CJD.
After the biopsy, the surgical equipment used was sterilized according to
the Hospital's normal procedures, which call for instruments to be cleaned
in a solution and heated to 270 degrees Fahrenheit for four minutes in a
prevacuum surgical autoclave.
There have been no known cases of CJD transmitted by surgical instruments in
the past 28 years since the routine use of these sterilization techniques.
However, certain enhanced sterilization measures are recommended
specifically for instruments used in cases of CJD by bodies such as the
World Health Organization (WHO).
As a first precaution, on September 15, following receipt of preliminary
biopsy results, all neurosurgical equipment was re-sterilized according to
WHO guidelines for CJD. On Monday, September 27, all hospital surgical
equipment was re-sterilized according to the same enhanced guidelines as a
"Although we believe the chances of an exposure are extremely small, we
cannot guarantee they are zero. That is why Emory is taking every possible
step to deal with this matter," said Allan Levey, MD, PhD, Chair of
Neurology, Emory School of Medicine.
William Bornstein, MD, PhD, Chief Quality Officer, Emory Healthcare said,
"All sterilized surgical equipment in the hospital has been resterilized
using the enhanced procedures recommended for this rare disease. We have
also instituted a new policy that exceeds hospital norms and calls for
treating every brain biopsy as a potential case of CJD and sterilizing the
instruments using the enhanced process, no matter how unlikely CJD may
appear at the time."
Hospital officials have also written to 418 non-neurosurgical patients who
were operated on in the hospital between September 10-27, describing the
events to them and explaining that any risks to non-neurosurgical patients
are even lower, if any. There are only six known cases of CJD transmitted
by surgical equipment, and all six of these patients had brain procedures.
All of those cases occurred in the 1970s before current standards of
sterilization, such as those used by Emory Hospital, were adopted.
CJD is an unusually rare, progressive degenerative disease of the brain
usually presenting as premature dementia and gradual loss of muscular
coordination. It is believed to be caused by the accumulation of an
abnormal protein, called a prion, found in nerve cells. Sporadic CJD, which
has no identified underlying cause, occurs at a rate of one case per million
people per year worldwide. It is not the disease known popularly as "mad
"It is Emory's policy to notify patients when we become aware of these types
of issues. It is also true, unfortunately, that there is no diagnostic test
to determine whether they have been exposed, nor are there any known means
of prevention or treatment," says Dr. Bornstein. "We believe the risk is
extremely low, but we also believe we have an obligation to share our
initial findings with our patients."
"The well-being of our patients is always our first concern," said John Fox,
President and Chief Executive Officer of Emory Healthcare. "We want to
assure our patients that we are taking every step possible to ensure quality
The Emory Health Connection, a call center staffed by registered nurses, is
available to speak with patients who would like more information. Emory is
also making physicians available to patients and their families.
About Emory Hospitals
Emory Hospitals include Emory University Hospital, a 579-bed hospital
located on the Emory University campus in northeast Atlanta, Emory Crawford
Long Hospital, a 511-bed, community-based hospital in midtown and Wesley
Woods Hospital, a 100-bed geriatric hospital located on the Emory campus.
Emory Hospitals are components of Emory Healthcare, the largest and most
comprehensive health care system in Georgia. Other components of Emory
Healthcare are: The Emory Clinic, the Emory Children's Center, the jointly
owned Emory-Adventist Hospital, and EHCA, LLC, a limited liability company
created in collaboration with HCA Healthcare.
What is Creutzfeldt-Jakob Disease?
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. Typically, onset of symptoms occurs at about age 60. There are three major categories of CJD: sporadic CJD, hereditary CJD, and acquired CJD. There is currently no single diagnostic test for CJD. The first concern is to rule out treatable forms of dementia such as encephalitis or chronic meningitis. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that is can be examined by a neurologist. Because a correct diagnosis of CJD does not help the patient, a brain biopsy is discouraged unless it is need to rule out a treatable disorder. While CJD can be transmitted to other people, the risk of this happening is extremely small.
Is there any treatment?
There is no treatment that can cure or control CJD. Current treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks.
What is the prognosis?
About 90 percent of patients die within 1 year. In the early stages of disease, patients 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.
What research is being done?
The leading scientific theory at this time maintains that CJD is caused by a type of protein called a prion. The harmless and the infectious forms of the prion protein are nearly identical, but the infectious form takes a different folded shape than the normal protein. Researchers are examining whether the transmissible agent is, in fact, a prion and trying to discover factors that influence prion infectivity and how the disorder damages the brain. Using rodent models of the disease and brain tissue from autopsies, they are also trying to identify factors that influence the susceptibility to the disease and that govern when in life the disease appears.
225 North Michigan Avenue
Chicago, IL 60601-7633
Tel: 312-335-8700 800-272-3900
Centers for Disease Control and Prevention (CDCP)
U.S. Department of Health and Human Services
1600 Clifton Road, N.E.
Atlanta, GA 30333
Tel: 800-311-3435 404-639-3311/404-639-3543
Creutzfeldt-Jakob (CJD) Foundation Inc.
P.O. Box 5312
Akron, OH 44334
Fax: 330-668-2474 305-893-9050