Isn't this just special...
05/26/99
Form of Fatal Insomnia Discovered
By KATHARINE WEBSTER=Associated Press Writer=
Researchers have discovered a brain-wasting disease that
begins with severe insomnia and ends in hallucinations and death.
The disease, sporadic fatal insomnia, is caused by the same
type of deformed proteins, known as prions, that cause mad cow disease
and its human variant, Creutzfeldt-Jakob disease, according to two
studies.
There is no known treatment or cure. It is not clear what
causes the disease, but scientists know it is not inherited. And unlike
one type of Creutzfeldt-Jakob disease, it is not believed to come
from infected meat.
Instead, scientists suspect it is caused by a spontaneous
mutation in a single brain or nerve cell.
So far, they have identified only six cases of the disease,
but there could be others that were misdiagnosed as other
mind-destroying illnesses such as Alzheimer's disease.
Prions are proteins with Jekyll-and-Hyde personalities that
cluster in the brain. When their molecules are folded into the
correct shape, prions are benign, though no one knows their
function.
Folded the wrong way, prions induce other proteins to mimic
them. The misfolded prions then accumulate in parts of the brain,
causing the tissue to break down and become full of holes, like a
sponge.
Different prion diseases attack different parts of the brain,
causing characteristic types of dementia and death.
Some are infectious, like mad cow disease in cattle and
scrapie in sheep and goats. In humans, prion diseases are either
inherited, caught from eating contaminated meat, or spontaneous.
Researchers have previously identified an inherited prion
disorder whose main symptom is sleeplessness, called fatal familial
insomnia.
In a case outlined in Thursday's New England Journal of
Medicine, a 44-year-old patient had all the symptoms of the
inherited disease, including prions with the same ``signature,''
but did not have the inherited mutation. The case was reported by
Dr. James Mastrianni, a neurologist at the University of Chicago.
The spontaneous, or sporadic, versions of the prion diseases
are rare, but more common than their inherited counterparts.
Prions are still controversial. Some researchers believe it
is impossible for proteins to replicate and cause disease, since they
do not contain genetic material. These researchers believe the
diseases are instead caused by as-yet-undiscovered viruses.
However, the new studies add to evidence the infection is
caused by the abnormal shape of the prions, not another agent, Drs.
Pierluigi Gambetti and Piero Parchi of Case Western Reserve
University said in an accompanying editorial.
Gambetti and Parchi have described five similar cases of
non-inherited fatal insomnia in a study scheduled to appear in the
journal Neurology.
--
Judith Richards, London, Ontario, Canada
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