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help on cruetzfelft jacobs disease (sp?)

Ged Haydon g.w.haydon at surveying.salford.ac.uk
Wed Nov 22 15:33:26 EST 1995

In article <48h9hg$3af at treasure.coastalnet.com> "William T. Honeycutt" <cn1606 at coastlanet.com> writes:
>From: "William T. Honeycutt" <cn1606 at coastlanet.com>
>Subject: Re: help on cruetzfelft jacobs disease (sp?)
>Date: 17 Nov 1995 06:19:28 GMT

>CReutzfeldt-Jakob Disease
>(Subacute Spongiform Encephalopathy)

>"A progressive, inevitably fatal, slow virus disease of the CNS, 
>charcterized by progressive dementia and myocolinic seizures and affecting 
>adults in mid-life"  (The Merck Manual, Sixteeth edition, copyright 1992, 
>pg. 209.

>"little is known about its (the viruses mode) of transmission."

>The Merck Manual further recommends standard Universal Precautions when 
>dealing with people with Creutzfeldt-jakob's, and recommends Steam 
>Autoclaving of instruments used with patient's with Creutzfeldt-Jakob's.  
>It also warns that formalin does not appear to be a effective means of 

>If memory serves me correctly, the only noted "possibly" transmission of 
>Creutzfeldt-Jakob is via direct contact with infected bodily fluids, such 
>as blood, or cerebral spinal fluid, or infected bodily tissues such as 
>brain dura matter.  However, I also remember that eating sheep eyes as is 
>a custom in some Arabic countries is also a high risk "behavior" for the 
>transmission of Creutzfeldt-Jakobs. I unfortunately do not recall if 
>sexual contact is included in possible modes of transmission, but would 
>recommend the use of a condom, as part of the general Universal 

>Unfortunately, like so many viruses, little is know about this one. 

>William T. Honeycutt, RN.
In the UK this is very topical due to the BSE (Bovine Serum Encephalitus) 
epidemic amongst the UK cattle population.

First came to light about 10 years ago. Then believed to be due to feeding 
cattle on the remains of sheep infected with "scapie".

UK government did b*gger all to record and traclk infected animals.

No a serious problem. One survey showed 25% of people would not eat beef any 
more and 60% were worried.

Has been shown to cross the species barrier.

Government states that it "does not infect humans" - one politician fed a 
hamburger to his kids on TV.

10 year (or there abouts) incubation period.

Little solid evidence at present.

Reckoned to be 600 infected cattle consumed each week - no tests sensitive (at 
least in common use) enough to show low level BSE infection.

Guidelines for slaughter houses are that they remove the spinal cord and offal 
from beef carcasses. However, in practice this is not particularly effective.

60 cases of CJD reported last year.

Believed to be caused by "Prion protein". I believe prions were originally 
considered to be proteins with an infective capability - not DNA or RNA.

It's no thought that the prion protein that causes the disease exists in two 
forms (perhaps isomers ?). The protein is a normal constituent of nerve tissue.

Its thought that contact between the disease protein form and the normal form 
causes the normal form to adopt the disease form structure. The cells control 
mechanisms continue to produce more protein because the levels appear to be 
too low to the cell - eventually the cells are damaged -> dementia etc.

Not destroyed by cooking.

Hope that helps

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