CREUTZFELDT-JAKOB DISEASE FACT SHEET - 1/1999
BY DEBORAH ONEY, MASTER OF SOCIAL WORK
CO-FOUNDER, BLOOD RECALL/WITHDRAWAL - CJD
Creutzfeldt-Jakob Disease (CJD) is an infectious rapidly progressive
fatal brain-deteriorating disease for which there is no treatment or cure.
Most scientists believe CJD is caused by a prion, which is a protein. People
in the United States and throughout the world get CJD. One strain nvCJD is
thought to be related to bovine spongiform encephalopathy (i.e. Mad Cow
Disease) in the United Kingdom. To date no nvCJD cases have been observed in
the United States. Normal sterilization procedures do not inactivate the CJD
infectious agent. The incubation period varies greatly with symptoms
emerging in known cases in 15 months to 30 years after exposure. There is no
preclinical test to determine if infection took place after a possible
exposure.
WHO GETS CJD AND HOW?
CJD most frequently appears in people ages 50-75. However, there are
victims in their 40s, 30s and even 20s. The officially stated mortality rate
of about one person per million per year appears to be an understatement
since CJD often escapes detection. In a Yale University study 13% of
clinically-diagnosed Alzheimer patients were found on autopsy to have CJD.
There are three forms of CJD: familial (about 5-10% of cases), sporadic
(cause unknown, about 90-95%) and iatrogenic (caused by a medical procedure
such as contaminated cadaver-derived growth hormones, dura mater implants,
and cornea transplants, less than 1%). Athletes who use cadaver-derived
growth hormones are at risk for CJD.
People receive blood products in transfusions, vaccines, diagnostic
tests and InVitro Fertilization cultures. The Centers for Disease Control is
currently conducting a study to determine if CJD is transmitted to humans by
blood. The infectious agent has been found in blood, but there have been no
documented cases of blood transmission in humans. As a precaution blood
relatives of CJD victims and people at risk for iatrogenic or familial CJD
should not donate blood.
Blood product withdrawals due to theoretical CJD risk have resulted in
many people receiving blood product withdrawal notifications. These
notifications can cause a great deal of anxiety for recipients.
WHAT ARE THE SYMPTOMS OF CJD AND HOW IS IT DIAGNOSED?
Classical CJD symptoms are a rapidly progressive presenile dementia;
involuntary, irregular jerking movements; and progressive motor dysfunction.
Early symptoms can include failing memory, behavior changes, visual
disturbances, and lack of coordination. The duration of CJD from the onset
of symptoms to death is usually less than a year. 14-3-3 spinal fluid tests
and brain biopsies are tests used to support a clinical diagnosis of CJD in
symptomatic patients. However, an autopsy is the only definitive way of
diagnosing CJD.
RESOURCES USED IN WRITING THE FACT SHEET
Brainum, Jerry. Growth Hormone: Certain Forms Could Be Deadly: GH Derived
from Cadavers Could Incubate Lethal Brain Cancer - And It's Still Available
in Europe! Muscular Development. September, 1998; 146.
The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob
Disease Program: A Congressional Mandate. This is available at this url:
http://members.aol.com/larmstr853/cjdvoice/cdcprog.htm
Click Here
Dealler, Dr. Steven, "Terms used by the scientists and explained as simply as
possible." This is available at: http://www.airtime.co.uk/bse/defb.htm
Click Here
The Food and Drug Administration Center for Biologics Evaluation and Research
"Revised Precautionary Measures to Reduce the Possible Risk of Transmission
of Creutzfeldt-Jakob Disease (CJD) by Blood and Blood Products - December 11,
1996." This is available at: http://www.fda.gov/cber/bldmem/mem121196.txt
Click Here
Hill, Andrew F.; Melanie Desbruslais; Susan Joiner, Katie C. L. Sidle, Ian
Gowland; John Collnge; Lawrence J. Doey; and Peter Lantos. "The same prion
strain causes vCJD and BSE". Nature.389 (October 2, 1997). 448 - 450.
Hsich, Gary; Kimbra Kenney; Clarence J. Gibbs, Jr.; Kelvin H. Lee; Michael G.
Harrington. The New England Journal of Medicine -- September 26, 1996 --
Volume 335, Number 13. "The 14-3-3 Brain Protein in Cerebrospinal Fluid as a
Marker for Transmissible Spongiform Encephalopathies."
Manuelidis, Elias E. and Laura Manuelidis, "Suggested Links between Different
Types of Dementias: Creutzfeldt-Jakob Disease, Alzheimer Disease, and
Retroviral CNS Infections" Alzheimer Disease and Associated Disorders_ 3
(1989): 100-109
National Institute of Neurological Disorders and Stroke. "Creutzfeldt-Jakob
Disease: Fact Sheet". This is available at this url:
http://www.ninds.nih.gov/patients/disorder/creutjab/cjd.htm
Click Here
National Institute of Neurological Disorders and Stroke, "Creutzfeldt-Jakob
Disease for Health Care Workers and Morticians", July, 1997. This is
available at this url: http://members.aol.com/larmstr853/cjdvoice/ninds.htm
Click Here
Ricketts, Maura N., Neil R. Cashman, Elizabeth E. Stratton, Susie ElSaadany,
Laboratory Centre for Disease Control, Health Canada, Ottawa, Ontario,
Canada; Montreal Neurological Institute, Montreal, Canada "Is
Creutzfeldt-Jakob Disease Transmitted in Blood?" 05/14/97 Emerging Infectious
Diseases National Center for Infectious Diseases Centers for Disease Control
and Prevention Atlanta, GA. This is available at this url:
http://www.cyber-dyne.com/~tom/blood_cjd.html
Click Here
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