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Off Topic: Of Mad Cows, and Men



This is a description of disease mediated by a controversial
particle called the prion, which is now gaining credibility.

Thought you might be interested in the development of a
new particle theory - much less common in other fields than
physics.

[Abstracted from SCIENCE-WEEK A Weekly Email Digest of the
News of Science at prismx@scienceweek.com

"A journal devoted to the improvement of communication
between the scientific disciplines, and between scientists,
science educators, and science policy makers."

January 21, 2000 -- Vol. 4 Number 3]





Creutzfeldt-Jakob disease was first described clinically and
pathologically in the 1920s. The disease occurs worldwide with an
incidence of 0.5 to 1.5 cases per million population per year.

There is no seasonal distribution, no evidence of changing
incidence over the years, and no convincing geographic clustering
except for areas with large numbers of familial cases. 80 percent
of sporadic cases (i.e., non-familial cases) of Creutzfeldt-Jakob
disease are diagnosed in persons 50 to 70 years of age, with a
mean survival time of only 5 months. 80 percent of patients with
sporadic disease die within one year.

Surgical transmission of Creutzfeldt-Jakob disease in humans has
been documented. A dramatic demonstration occurred when two
young patients developed the disease 16 and 20 months after they
underwent surgery to excise epileptic foci.

At the time of surgery, stereotactic electroencephalographic
exploration was undertaken with silver electrodes that had been
previously implanted in a patient with known Creutzfeldt-Jakob
disease, the electrodes having been "sterilized" with 70 percent
alcohol and formaldehyde vapor.

Two years later, after recognition of the possible transmission
of Creutzfeldt-Jakob disease to the epilepsy patients, the
electrodes were retrieved and implanted into a chimpanzee in
which the disease subsequently developed. Contaminated
neurosurgical instruments have been suspected as modes of
transmission in other patients.

Studies of animal and human prions indicate that the agents are
resistant to treatments that inactivate nucleic acids and viruses
(e.g., alcohol, formalin, ionizing radiation, proteases, nucleases),
but they are inactivated by treatments that disrupt proteins
(e.g., autoclaving, phenol, detergents, and extremes of pH).

--------------------------------------------------------------------
brian whatcott <inet@intellisys.net>
Altus OK