THE POSSIBLE ASSOCIATION OF TSE WITH SQUIRRELS - FO Bastian
I was indirectly involved and am mentioned in the New Yorker article regarding whether
squirrels could be a reservoir for Creutzfeldt-Jakob disease (CJD) in humans. The young
neurologist who reported the association was a trainee at USA (University of South AL where I
was the neuropathologist. At a conference. I presented a case of CJD from Foley AL who
routinely ate fresh squirrel brains. I also mentioned that the CJD case at Baylor wherein I
discovered the spiroplasma inclusion by electron microscopy (reported in 1979) was a patient
who was an avid squirrel brain eater. I also had received a consultation regarding a man in West
VA who was a survivalist who ate fresh squirrel brains mixed with fresh eggs.
As you know the New Yorker Magazine made fun of the Lancet report by the Kentucky
doctors suggesting that Kentuckians are in danger of getting CJD from road kill. Unfortunately,
the senior author of the Lancet article said it was reported in jest. The New Yorker also
interviewed me since I was mentioned by the young neurologist. I said that any claim can only
be made by experimentation.
The question can now be addressed since we have had a major breakthrough in our
laboratory wherein we have consistently isolated an extreme thermoacidophilic bactertum from
TSE-affected tissues. We can now test the idea by attempting to grow the bacterium in Brucella
media which allows growth of the novel TSE spiroplasma isolate because the special media has
low oxygen tension. Our success in isolating the bacterium from TSE tissues has been 100% and
now that we can grow the agent in cell-free culture, we can develop a live test for TSE and could
make an instant diagnosis from a dead squirrel.
Our research is attempting to deal with the chronic wasting disease (CWD) epizootic in
cervids including deer, elk and moose. The CWD epizootic, which is essentially mad cow
disease in cervids, is widely distributed in North America involving 26 states and 3 Canadian
provinces. As you know this has gone global wherein CWD outbreaks have occurred in South
Korea and Europe where reindeer are affected. The disease has even affected camels.
Zoos are in trouble.
Another misconception is the incidence of CJD. I am a Neuropathologist who has spent
most of my career in diagnosing CJD. One year 7 cases were found in New Orleans. The past
information regarding incidence of CJD was based on a French study done in the 1960s, and in
my opinion is out of date. The disease is under reported due often to lack of expertise by general
pathologists. I reported that 15% of Alzheimer disease cases are actually CJD. I made a plea to
the CDC to make CJD mandatory reportable disease and was turned down. People are dying of
CJD in the prime of life and there is no live test available and definitely no treatment options.
Detection of prion amyloid is only a test that can be applied postmortem or in a biopsy specimen.
In conclusion, there is an association of CJD cases with the practice of eating squirrel
brains. With recent advances in our research studying TSEs, we are now able to answer the
question by direct isolation of the novel spiroplasma that is likely the causative agent of TSE.
My young neurologist protege had the courage to report this association, which may prove
important in understanding the epidemiology of the TSE diseases.