spongiform encephalopathy in young humans

[MATTHEW FRANCIS WETTLAUFER]

Jorg Kirberg (kirberg at bii.ch) wrote:
: In article <47h4pd$cbe at news.csus.edu>, mattw at sfsu.edu (MATTHEW FRANCIS
: WETTLAUFER) wrote:

: > VANCAMPE at UWYO.EDU wrote:
: > : Dear Anyone:  I've heard a rumor through the grapevine that 3 cases of
: > : spongiform encephalopathy have been diagnosed in young (teenage)
: humans in the
: > : U.K.  There is some mention of possible links to the ingestion of
: unpasteurized
: > : milk and bovine brains by two of these patients.  The report is
: supposed to be
: > : in Lancet.  Anyone have a reference or can confirm?  Thank you in
: advance, Hana
: > I haven't read any articles about it but it would make sense if you
:                                            ^^^^^^^^^^^^^^^^^^^ ???* 
: > consider the outbreak of "mad cow disease" in the U.K. during the 
: > mid-1980's when up to 130,000 head of cattle had to be destroyed because 
: > they had become infected with prions from sheep mulch.
: > Spongiform encephalopathies have very long incubation periods, 5 to 8 
: > years, so there was and still is speculation over whether a species leap 
: > could take place between cows and humans (as it did between sheep and cows).
: > Don't know if any of this info. helps.
: > Matt Wettlaufer

: * how can it make sense if man never got it from sheep before ? I would
: think that one still has to wait for more cases before a link between BSE
: and humans eating whatever from the U.K. cattles can be made.
: jorg

: ******************************************************************
: Joerg Kirberg                     EMAIL: kirberg at bii.ch
: Basel Institute for Immunology    FAX:   41-61-605 13 72
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: Switzerland
I agree with you that no connection can be made between BSE and human 
spongiform encephalopathy unless more cases arise.  But I do not think 
that should rule speculation out.  PrP proteins that cause BSE are closer 
to PrP proteins sequenced for humans than they are to those that have 
been sequenced for sheep (scrapies).  And as the others of this panel 
have mentioned, it is worrisome when two cases of noninherited CJD arise 
in the same country at the same time in two young people (when CJD 
usually developes in elderly people and at an incidence rate of one per 
million).  No connection can be proven at this point unless other cases 
arise, but speculation about this is not unwarranted given this pathogen's 
unpredictable etiology.
Matt Wettlaufer