On food and cooking the science and lore of the kitchen ( PDFDrive ) 249
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begantocausebraindiseaseinthecattle.
Humansarenotsusceptibletosheep
scrapie.Butthere’samainlyhereditary
humanbraindiseasesimilartoscrapieand
causedbyasimilarprion;itiscalled
Creutzfeldt-Jakobdisease(CJD),typically
strikesoldpeoplewithlossofcoordination
andthendementia,andeventuallykillsthem.
In1995and1996,tenrelativelyyoungBritons
diedfromanewvariantofCJD,andtheprion
agentfoundintheirbodieswascloselyrelated
totheBSEprion.Thisstronglysuggeststhat
humanscancontractadevastatingdiseaseby
eatingmeatfromBSE-infectedcattle.The
cattlebrain,spinalcord,andretinaare
thoughttobethetissuesinwhichprionsare
concentrated,buta2004reportsuggeststhat
theymayalsobefoundinmusclesandthusin
commoncutsofbeef.
BSEappearstohavebeeneliminatedin
Britainthankstothecullingofaffectedherds,
changesinfeeding,andsurveillance.But
diseasedcattlehaveturnedupelsewherein
Europe,aswellasintheUnitedStates,
Canada,andJapan.Asaprecautionary
measure,anumberofcountrieshave
suspendedsometraditionalpracticesatleast
temporarily.Theseincludeeatingflavorful
meatfromolderanimals(whicharemore
likelytocarryBSE),aswellasbeefbrains,
sweetbreadsandspleen(immune-system
organs),andintestines(whichcontain
immune-systemtissues).Somecountriesalso
forbidtheuseof“mechanicallyrecovered
meat”—tinyscrapsremovedfromthe
skeletonbymachineandincorporatedinto
groundbeef—fromtheheadandspinal
column.Theseruleswillprobablybe
modifiedasrapidtestsfortheanimaldisease
aredevelopedandimplemented,andaswe
learnmoreabouthowitistransmittedto
people.
Todate,theknownhumandeathtollfrom
BSE-infectedbeefnumbersinthelow
