CJD from sheep sources

[Adrian.Philbey at SMTPGWY.AGRIC.NSW.GOV.AU]

          Bruce Phillips (bap at med.pitt.edu) wrote:

          >... is it conceivable that ingestion of Haggis ... could
          >passage the agent to humans

          >... the current U.K. CJD cases were first described from
          >... Edinburgh, Scotland

          Traditional haggis consists of offal such as heart and liver
          of sheep minced with suet and oatmeal or barley and
          contained within the first stomach (rumen) of the sheep.
          Therefore, if an agent was present in the offal that could
          be transmitted to humans, haggis would be a potential
          source.

          Scrapie in sheep is considered to be the pathological
          equivalent of bovine spongiform encephalopathy (BSE) in
          cattle and Creutzfeldt-Jakob disease (CJD) in humans.
          Scrapie occurs in the UK, Europe and many other parts of the
          world. There has been no epidemiological association between
          the occurrence of scrapie in sheep and the occurrence of CJD
          in humans, despite consumption of sheep products from
          affected areas. Therefore, there is no evidence that the
          scrapie agent has been transmitted directly to humans or
          causes CJD and haggis is unlikely to have been a source of
          the agent causing the newly identified CJD-like disease in
          humans in the UK.

          It is hypothesised that BSE in cattle resulted from
          exposure to the scrapie agent from sheep in meat meals that
          had been improperly inactivated during the rendering process
          at abattoirs. This hypothesis is plausible, but cannot be
          proven conclusively and there are alternative possibilities,
          such as spread of an agent of bovine origin throughout the
          cattle population of the UK. Similarly, there is no proof
          that the newly identified CJD-like disease in humans in the
          UK has been caused by exposure to either the BSE agent of
          cattle or the scrapie agent of sheep.

          The reason that the newly identified CJD-like disease has
          been reported from Edinburgh, Scotland, is that there is a
          CJD Surveillance Unit in Edinburgh that has been collating
          cases of CJD reported from throughout the UK by clinical
          neurologists and neuropathologists. I do not have details of
          where in the UK the cases of the newly identified CJD-like
          disease are located, but they are not all clustered in
          Scotland.

          Adrian W Philbey
          Veterinary Research Officer
          Elizabeth Macarthur Agricultural Institute
          Private Mail Bag 8
          Camden  NSW  2570
          Australia
          
          Telephone: 61-46-293332
          Facsimile: 61-46-293429
          email: philbea at agric.nsw.gov.au