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Creutzfeldt-Jakob Disease: More Common Than Thought

DebbieOney at aol.com DebbieOney at aol.com
Tue Jun 9 12:45:51 EST 1998

Since I posted on Creutzfeldt-Jakob Disease a few months ago people have asked
me for a more detailed posting.  Therefore, I have revised and added a great
deal of information to the following posting.

If you teach please include Creutzfeldt-Jakob Disease and prions in the

Creutzfeldt-Jakob Disease (CJD) has greater public health consequences than
the mere number of reported case might lead one to believe because it is not a
reportable disease in most places, is often misdiagnosed, is infectious and is
not killed by normal sterilization.  CJD is a horrendious infectious fatal
brain-deteriorating disease for which there is no treatment or cure.  It is
caused by a prion. One strain of CJD is linked to bovine spongiform
encephalopathy (i.e. Mad Cow Disease) in England.

In the United States and throughout the world people get CJD through 3 means:
familial (genetic), sporadic (don't know how) and iatrogenic (through a
medical procedure such as human pitutitary growth hormones, contaminated
surgical equipment and dura mater and cornea transplants.)   CJD can take
decades after exposure for the patient to show symptoms.  However, once a
person shows symptoms their decline is rapid and they die within a year of
first showing symptoms.

CJD should be considered whenever a patient develops a rapid dementia and
myoclonus. The initial symptoms are subtle and ambiguous and include insomnia,
depression, confusion, personality and behavioral changes, strange physical
sensations, and  memory, coordination and visual problems.  Rapidly
progressive dementia and usually myoclonus (involuntary, irregular jerking
movements) develop as CJD progresses.  Also,  language, sight, muscular
weakness, and coordination problems worsen. The patient may appear startled
and become rigid. In the final stage the patient loses all mental and physical
functions. The patient may lapse into a coma and usually dies from an
infection like pneumonia precipitated by the bedridden, unconscious state. The
duration of CJD from the onset of symptoms to death is usually one year of
less.  A 14-3-3 spinal fluid test is over 95% effective in diagnosing CJD when
symptoms are present.  (For information on the test contact Dr  Clarence
Joseph Gibbs, Jr.; National Institutes of Health, (30l) 496-4821 or 6321.)
CJD patients often die at home and therefore have home health service and have
family members as caregivers.

Since early signs of CJD are often psychological, victims will often receive
psychological treatment.  In addition, people, including school-age children
of Creutzfeldt-Jakob Disease victims who die in their 40s, often require
counseling to deal with the death.  And, then there's the recipients of
medical treatments such as human pituitary growth hormones and dura mater
transplants who are at high risk of CJD and the people with a genetic mutation
for familial CJD who must live with CJD hangiing over their heads.  Another
group includes people who receive withdrawal notices that the blood they or,
worst yet, their children, received came from a pool which included a donor
that died of CJD.  While the question of whether CJD is passed through blood
products is yet to be resolved, receiving this type of notice causes great
anxiety in people.  

CJD is more common than reported.  In one study of Alzheimer patients 13% when
autopsied were found to really have CJD.

Also, since normal sterilization methods do no kill the CJD infectious agent
and , it can therefore be spread by surgical instruments, it is more of a
danger to public health than mere number of cases would suggest..

Whether CJD is spread by human blood is controversial,.  Pooled blood products
are withdrawn as a precaution if it is found after the product has been
released that a person in the donor pool has died of CJD of is at higher risk
of contracting CJD.  However, by this point it has often already been used in
humans.  While the controversy remains as to whether CJD can be passed through
blood products, blood products continue to be used in vaccines such as rabies
and allergy shots; in InVitro Fertilization (IVF) cultures; and in medical
test fluids.  At the end of this e-mail I have included the U. S.
Congressional Mandate requesting the Centers for Disease Control to conduct a
study to assess whether the CJD infectious agent is spread through blood

CJD Voice is an e-mail discussion group.  Most members have lost a loved one
to CJD or currently have a loved one with CJD.  Other members include people
who took human pituitary growth hormones as children and therefore are at
higher risk of getting CJD and people who received notices that the blood
products their children received were from a pool that included a person who
died from CJD.  It provides support to these people as well as tries to
increase funding for CJD research so treatments and a cure can be found.  The
CJD webpage has a message board, chat room, links to other websites with CJD
information and a list of CJD researchers accepting financial contributions.

The address for the CJD Voice Webpage is
Please feel free to visit the website and to refer other people to it.

Feel free to distribute this information to whomever you choose.

DebbieOney at aol.com

 The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob
     Disease Program

     A Congressional Mandate

     In response to concerns that CJD may be transmitted through blood or
     blood products, the U.S. Congress requested that the
     CDC conduct a study to assess whether CJD is a threat to the safety of
     the nation's blood supply. Researchers believe that
     the risk of transmission to humans through blood products is very
     small since there have been no known cases of CJD
     contracted by humans in this manner. However, precautions are being
     taken to assure that the blood supply is safe from this
     infectious agent. Current blood safety policy requires that any blood
     products made from blood donated by a person who
     later develops CJD, or is found to have risk factors for CJD, must be
     withdrawn. Until the question of transmissibility is
     resolved, the availability and the price of blood products will
     continue to be adversely impacted by shortages caused by
     recalls and the destruction of blood products which, to date, has cost
     over $100 million.

     The Program: How You Can Help

     Because the signs and symptoms of CJD may not develop for up to 30
     years, a person could be infected and not show any
     symptoms during his or her lifetime. Furthermore, there is no
     screening test available for CJD and the only sure way to test
     for CJD is by analyzing brain tissue after death. The CJD program asks
     families of individuals who have received blood
     products to donate brain tissue after their death. The brain tissue
     will be shipped to Stephen J. Armond, M.D., Ph.D.,
     Professor of Neuropathology, University of California, San Francisco
     to test for evidence of CJD. Other brain tissue will be
     stored at CDC in Atlanta for analysis in the future when more is known
     about the cause of CJD.

     Participation in the CJD program is voluntary. The CDC is aware that
     the death of a family member is a difficult time to make
     important decisions. Therefore, the CDC is working through your
     regional coordinator, physician, and treatment center staff to
     provide support to you and your family as you discuss brain tissue
     donation. To help with making an informed and rational
     decision, the CDC has developed information packets which answer
     commonly asked questions about the program including:
     how to authorize the donation; how confidentiality will be assured;
     the ability to retrieve the brain tissue without disfigurement
     and without affecting funeral arrangements; and the time frame for
     receiving test results.

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