Variant Creutzfeldt-Jakob

[TheCritic]

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For random reasons, I have been looking at this topic in the past few days and have not been able to answer a few of my own questions.

Apparently there have been 1 to 3 confirmed cases ( 1 per the CJD surveillance website at Case Western) of vCJD in the US, the kind you get from eating BSE infected meat. 160 or so in the UK since 1997 and various others around the world.

I wonder if anyone has any autopsy experience with suspected CJD (any kind) cases. I read somewhere that most pathologists have justified aversions for such cases for obvious reasons and I am curious what probably happens with these cases. Autopsy is never done? Autopsy is done somewhere else? I also wonder if many labs are equipped for the diagnosis at Autopsy with the appropriate stains etc.

 

[yaah]

I don't know if you can refuse...but here I think they have tried to talk people out of it unless it is really necessary. When it does they take extra precautions with every step and limit personnel involved (i.e. often just the diener and attending). Then they quickly take brain tissue with specialized instruments, freeze it, and send it to somewhere in Iowa. Iowa does a specialized test. I don't know if it is U of Iowa or some specialized lab...

Honestly, though, I can't remember offhand how the rest of the case goes. I don't know if it is treated like a surgical biopsy which gets long fixation in formalin then fixation in formic acid then formalin again before processing. Or maybe they just stop the case there with just the samples. I will have to look it up, we have a procedure manual somewhere. I just haven't been on autopsy in years.

 

[TheCritic]

I don't know if you can refuse...but here I think they have tried to talk people out of it unless it is really necessary. When it does they take extra precautions with every step and limit personnel involved (i.e. often just the diener and attending). Then they quickly take brain tissue with specialized instruments, freeze it, and send it to somewhere in Iowa. Iowa does a specialized test. I don't know if it is U of Iowa or some specialized lab...

Honestly, though, I can't remember offhand how the rest of the case goes. I don't know if it is treated like a surgical biopsy which gets long fixation in formalin then fixation in formic acid then formalin again before processing. Or maybe they just stop the case there with just the samples. I will have to look it up, we have a procedure manual somewhere. I just haven't been on autopsy in years.

Are you sure it's Iowa, or Ohio? Cleveland? Check out:
http://www.cjdsurveillance.com/abouthpd-acquired.html

 

[ataktos]

I actually asked about this during a forensic rotation at the county ME's in a major city. The attending I was working with said that they refuse such cases. I'm sure there has to be a longer answer than that....perhaps Mindy will give her input on this question?

 

[DarksideAllstar]

At my program the vCJD cases get special treatment, with only a specific autopsy attending and one of the neuropathologists who studies CJD (I think he worked w/ Prusiner in the past) do the case by themselves.

 

[CameronFrye]

We do CJD autopsies for Virginia and several of the surrounding states (a lot of places refuse to do them). The tissue is sent to Case Western for confirmation. I think we have around 8-10 potential cases a year, and about half of them come back positive. Residents are allowed to opt out if they have concerns.

 

[Horusman]

First, there has only been one case of vCJD diagnosed in the US (well documented as being "imported" from UK).

Second, worry far more about Hep C than CJD in terms of exposure from cutting yourself during an autopsy.

Third, worry far more about the case of CJD which arrives at your autopsy table WITHOUT a clinical diagnosis [which you would do happily] than the only that arrive labeled as such [which you would turn away].

Many countries (US, EU, etc) have a single national reference lab which handles the diagnostic tests for prion disease (Western blotting, immunohistochemistry, sequencing); the US one is at Case Western in Cleveland. As mentioned above, the accepted protocol for tissue processing involves formalin fixation, formic acid treatment and additional fixation. With this treatment, the tissue becomes non-infectious (the method was developed at NIH in the 1990s). Clean-up of instruments, table, etc requires lots of bleach (what most places use anyway).

For the "mature" members of the group:
This conversation is reminiscent of the mid-80's when the "I don't want to do AIDS cases" complaint arose (although those occurred face-to-face rather than hidden by screen names!). Yet now, on path services all over the country, residents, technicians and staff handle tissue from HIV-positive patients without a particular thought.

 

[yaah]

Similar in a way to AIDS cases in the past, but more currently similar to TB cases. No one wants to do autopsies on patients with active TB either. I feel much more comfortable with a blood borne or tissue based disease because precautions can help. Airborne diseases, though, not so much.

Guess I was wrong about Iowa. Strange, thought it was.

 

[djmd]

First, there has only been one case of vCJD diagnosed in the US (well documented as being "imported" from UK).

Really only one case? So the autopsy I did was the only positive one?
Some how I doubt that...
And yes, it was confirmed by Case Western's lab.

 

[Horusman]

vCJD in the US:
One case was diagnosed in US by autopsy in Florida (2004).

One person was living in US when symptoms developed but returned to UK where the diagnosis was made. This was confirmed by autopsy in UK apparently.

One person is assumed to have acquired the disease in Saudi Arabia, became ill while in US and had diagnosis confirmed by biopsy at UCSF (lymphoid tissue and brain). This person is either still living or didn't have an autopsy.

(http://www.cdc.gov/ncidod/dvrd/vcjd/qa.htm#UScases)

 

[yaah]

Really only one case? So the autopsy I did was the only positive one?
Some how I doubt that...
And yes, it was confirmed by Case Western's lab.

Correct me if I'm wrong, but there is a difference between vCJD and CJD. He was talking about the one acquired from eating meat. There are other forms of the disease which are more common and which warrant biopsy more often.

 

[Horusman]

Exactly -- that is why it is called VARIANT CJD (vCJD). Variant CJD appeared in the UK in the wake of "Mad Cow disease" (Bovine Spongiform Encephalopathy) and represents the consequence of transmission of the prion disease to humans following oral exposure to contaminated beef. There are fewer than a total of 200 cases know world-wide, with nearly all in the UK.

Sporadic CJD (sCJD) is a world-wide disorder with an incidence of about 1 case per 1,000,000 per year. It presents as a rapid dementing illness with typical course from onset to death of less than 1 year (often just a few months).

Other forms of prion disease include familial CJD (fCJD) associated with mutations in the prion gene. This includes the GSS syndrome (complicated and hard to spell German names).

There is also iatrogenic CJD (iCJD) associated with exposure to dural grafts (mostly in Japan), to electrical recording depth electrodes (a handful of cases) or to growth hormone from the pre-recombinant DNA era. There are a few hundred cases of iCJD world wide.

And then there is kuru...

 

[TheCritic]

Exactly -- that is why it is called VARIANT CJD (vCJD). Variant CJD appeared in the UK in the wake of "Mad Cow disease" (Bovine Spongiform Encephalopathy) and represents the consequence of transmission of the prion disease to humans following oral exposure to contaminated beef. There are fewer than a total of 200 cases know world-wide, with nearly all in the UK.

Sporadic CJD (sCJD) is a world-wide disorder with an incidence of about 1 case per 1,000,000 per year. It presents as a rapid dementing illness with typical course from onset to death of less than 1 year (often just a few months).

Other forms of prion disease include familial CJD (fCJD) associated with mutations in the prion gene. This includes the GSS syndrome (complicated and hard to spell German names).

There is also iatrogenic CJD (iCJD) associated with exposure to dural grafts (mostly in Japan), to electrical recording depth electrodes (a handful of cases) or to growth hormone from the pre-recombinant DNA era. There are a few hundred cases of iCJD world wide.

And then there is kuru...

And scrapie. Yes there is a big difference between CJD and vCJD. Trust me, if you find a case of vCJD that originated in the US, it would be a huge freaking deal to the point that the US Department of Agriculture and the US beef industry would literally **** their pants all at the same time. Just think about the implications.

 

[CameronFrye]

Yeah, I probably should've specified that our cases have all been for "normal" CJD.