Veterinary Record2013;172:455 doi:10.1136/vr.f2613 
 
 
Letters
 
 
Ruminant Health
 
 
 
Transmission of classical scrapie via goat milk
 
 
 
Timm Konold1, Hugh A. Simmons1, Paul R. Webb1, Peter J. Bellerby1, Steve A. 
C. Hawkins1 and Lorenzo González2
 
 
+ Author Affiliations 
 
 
 1AHVLA – Weybridge, New Haw, Addlestone, Surrey KT15 3NB 2– Lasswade, 
Pentlands Science Park, Bush Loan, Penicuik, Midlothian EH26 0PZ e-mail: 
timm.konold@ahvla.gsi.gov.uk 
 
 
 
FOLLOWING reports that ovine scrapie (referred to here and subsequently as 
classical scrapie) can be transmitted from dams to lambs via milk (Konold and 
others 2008, Ligios and others 2011), we subsequently carried out a study to 
investigate whether caprine scrapie could also be transmitted via milk, using 
material collected from a field outbreak of scrapie in goats in the UK (González 
and others 2009). Lambs were selected as milk recipients from a closed flock of 
known scrapie-free status (Simmons and others 2009) because an assured 
scrapie-free source of goats was not available. 
 
 
Due to the lack of published information about the susceptibility of sheep 
to caprine scrapie, a pilot study was conducted to determine whether sheep were 
susceptible to … 
 
 
 snip... 
 
 
The study is still ongoing but the current results confirm that the scrapie 
agent can also be transmitted via milk from goats. This reinforces the validity 
of the decision by the European Parliament in 2009 to prohibit the feeding of 
milk or milk products from classical scrapie-infected flocks or herds to small 
ruminants in general. 
 
 
Timm Konold, Hugh A. Simmons, 
 
 
please see full text @
 
 
 
 
 
Sunday, February 10, 2013 
 
Scientific Opinion on the risk of transmission of classical scrapie via in 
vivo derived embryo transfer in ovine animals 
 
 
 
 
Saturday, February 11, 2012 
 
PrPSc Detection and Infectivity in Semen from Scrapie-Infected Sheep 
 
 
 
 
 
Wednesday, January 18, 2012
 
BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE 
 
February 1, 2012 
 
 
 
 
 
 
Envt.18: Mother to Offspring Transmission of Chronic Wasting Disease 
 
 
 
Candace K. Mathiason,† Amy Nalls, Kelly Anderson, Jeanette Hayes-Klug, 
Jenny G. Powers, Nicholas J. Haley and Edward A. Hoover 
 
 
 
Colorado State University; Fort Collins, CO USA†Presenting author; Email: 
ckm@lamar.colostate.edu 
 
 
 
We have developed a new cervid model in small Asian muntjac deer (Muntiacus 
reevesi) to study potential modes of vertical transmission of chronic wasting 
disease (CWD) from mother to offspring. Eight of eight (8/8) muntjac doe orally 
infected with CWD tested PrPCWD lymphoid positive by four months post infection. 
Ten fawns were born to these CWD-infected doe— four of the fawns were viable, 
five were non-viable and one was a first trimester fetus harvested from a 
CWD-infected doe euthanized at end-stage disease. The viable fawns have been 
monitored for CWD infection by immunohistochemistry and sPMCA performed on 
serial tonsil and rectal lymphoid tissue biopsies. PrPCWD has been detected in 
one fawn by IHC as early as 40 days of age. Moreover, sPMCA performed on rectal 
lymphoid tissue has yielded positive results on another fawn at ten days of age. 
In addition, sPMCA assays have demonstrated amplifiable prions in fetal 
placental or spleen tissue of three non-viable fawns and mammary tissue of the 
dams. 
 
 
 
Additional pregnancy related fluids and tissues from the doe as well as 
tissue from the nonviable fawns are currently being probed for the presence of 
CWD. In summary, we have employed the muntjac deer model, to demonstrate for the 
first time the transmission of CWD from mother to offspring. These studies 
provide the foundation to investigate the mechanisms and pathways of maternal 
prion transfer. 
 
 
 
 
 
=========================== 
 
 
 
 
 
PPo3-18: A Possible Case of Maternal Transmission of the BSE Agent within 
Captive Cheetah Affected with Feline Spongiform Encephalopathy 
 
 
 
 Anna Bencsik, Sabine Debeer, Thierry Petit and Thierry Baron 
 
 
 
Afssa; Unité ATNC; Lyon, France; Zoo de la Palmyre; Les Mathes, France 
 
 
 
Key words: BSE, FSE, vertical transmission 
 
 
 
Introduction. Feline spongiform encephalopathy (FSE) is considered to be 
related to bovine spongiform encephalopathy (BSE). It has been reported in 
domestic cats as well as in captive wild cats including cheetahs, first in the 
United Kingdom (UK) and then in other European countries. In France, several 
cases were described in cheetahs either imported from UK or born in France. Here 
we report details of two other FSE cases in captive cheetah. These cases are of 
particular interest since the 2nd case of FSE in a cheetah born in France, 
appears most likely due to maternal transmission.1 
 
 
 
Results. Complete PrPd study showed the close likeness between the two 
cheetah cases. The TgOvPrP4 mouse brains infected with cattle BSE and cheetah 
FSE revealed similar vacuolar lesion profiles, PrPd brain mapping with 
occurrence of typical florid plaques. 
 
 
 
Materials and Methods. Using immunohistochemistry (IHC), pathological form 
of PrP(PrPd) was analyzed in the brains and peripheral organs of these two 
cheetahs. Transmission studies to the TgOvPrP4 mouse line were also performed, 
for comparison with the transmission of cattle BSE. Lesion profiles of the 
infected transgenic mice were analyzed as well as type and brain distribution of 
PrPd. 
 
 
 
Conclusion. Collectively, these data indicate that both FSE cases harbor 
the same strain of agent as the cattle BSE agent. Because this is most probably 
a case of maternal transmission of the disease, this new observation may have 
some impact on our knowledge of vertical transmission of BSE agent-linked TSEs 
such as in human variant Creutzfeldt Jakob disease. 
 
 
 
References 
 
 
 
1. Bencsik et al. PLoS One 2009; 4:6929. 
 
 
 
 ========================= 
 
 
 
 
 
PPo3-40: Mother to Offspring Transmission of Chronic Wasting Disease 
 
 
 
Candace K. Mathiason, Amy V. Nalls, Kelly Anderson, Jeanette Hayes-Klug, 
Nicholas Haley and Edward A. Hoover 
 
 
 
Colorado State University, Department of Microbiology, Immunology and 
Pathology, Fort Collins, CO USA 
 
 
 
Key words: Chronic wasting disease, vertical transmission, muntjac deer 
 
 
 
We have developed a new cervid model in small Asian muntjac deer (Muntiacus 
reevesi) to study potential modes of vertical transmission of chronic wasting 
disease (CWD) from mother to offspring. Eight of eight (8/8) muntjac doe orally 
infected with CWD tested PrPCWD lymphoid positive by 4 months post infection. 
Six fawns were born to these CWD-infected doe. Six fawns were born to 6 
CWD-infected doe; 4 of the fawns were non-viable. The viable fawns have been 
monitored for CWD infection by immunohistochemistry and sPMCA performed on 
serial tonsil and rectal lymphoid tissue biopsies. PrPCWD has been detected in 
one fawn as early as 40 days of age. Moreover, sPMCA performed on rectal 
lymphoid tissue has yield positive results on another fawn at 10 days of age. In 
addition, sPMCA assays have also demonstrated amplifiable prions in maternal 
placental (caruncule) and mammary tissue of the dam. 
 
 
 
Additional pregnancy related fluids and tissues from the doe as well as 
tissue from the nonviable fawns are currently being probed for the presence of 
CWD. In summary, we have employed the muntjac deer model, to demonstrate for the 
first time the transmission of CWD from mother to offspring. These studies 
provide the foundation to investigate the mechanisms and pathways of maternal 
prion transfer. 
 
 
 
PRION 2011 
 
 
 
landesbioscience.com 
 
 
 
International Prion Congress: From agent to diseaseSeptember 8–11, 
2010Salzburg, Austria 
 
 
 
 
 
 
 
 
Saturday, December 3, 2011 
 
Isolation of Prion with BSE Properties from Farmed Goat Volume 17, Number 
12—December 2011
 
 
 
 
 
 
 
PRION 2010 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Friday, December 23, 2011 
 
Detection of PrPres in Genetically Susceptible Fetuses from Sheep with 
Natural Scrapie 
 
 
 
 
 
Monday, November 22, 2010
 
 
 
Saturday, April 12, 2008
 
Evidence of scrapie transmission via milk 
 
 
 
 
 
Wednesday, January 18, 2012 
 
Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural 
Scrapie Isolates Similar to CH1641 Experimental Scrapie 
 
Journal of Neuropathology & Experimental Neurology: February 2012 - 
Volume 71 - Issue 2 - p 140–147 
 
 
 
 
 
Wednesday, February 16, 2011 
 
IN CONFIDENCE 
 
SCRAPIE TRANSMISSION TO CHIMPANZEES 
 
IN CONFIDENCE 
 
 
 
 
 
Sunday, December 12, 2010 
 
EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 
December 2010 
 
 
 
 
 
Sunday, April 18, 2010 
 
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010 
 
 
 
 
 
Thursday, December 23, 2010 
 
Molecular Typing of Protease-Resistant Prion Protein in Transmissible 
Spongiform Encephalopathies of Small Ruminants, France, 2002-2009 
 
Volume 17, Number 1 January 2011 
 
 
 
 
 
Thursday, November 18, 2010 
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform 
encephalopathy following passage in sheep 
 
 
 
 
 
Monday, April 25, 2011 
 
Experimental Oral Transmission of Atypical Scrapie to Sheep 
 
Volume 17, Number 5-May 2011 
 
 
 
 
 
Friday, February 11, 2011
 
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues 
 
 
 
 
 
Thursday, March 29, 2012 
 
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012 
 
NIAA Annual Conference April 11-14, 2011San Antonio, Texas 
 
 
 
 
 
Wednesday, April 4, 2012
 
20120402 - Breach of quarantine/Violation de la mise en quarantaine of an 
ongoing Scrapie investigation
 
 
 
 
 
Michigan and California have had a high spike in Goat Scrapie cases, 
compared to elsewhere ??? 
 
 
 
Tuesday, February 01, 2011 
 
Sparse PrP-Sc accumulation in the placentas of goats with naturally 
acquired scrapie 
 
(Figure 6) including five goat cases in FY 2008 that originated from the 
same herd in Michigan. This is highly unusual for goats, and I strenuously urge 
that there should be an independent investigation into finding the common 
denominator for these 5 goats in the same herd in Michigan with Scrapie. ... 
 
 
 
 
 
Thursday, February 23, 2012
 
Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012 
 
 
 
 
 
RESEARCH 
 
Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011 
 
 
Experimental Oral Transmission of Atypical Scrapie to Sheep 
 
 
Marion M. Simmons, S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. 
Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, 
Melanie J. Chaplin, and John Spiropoulos 
 
To investigate the possibility of oral transmission of atypical scrapie in 
sheep and determine the distribution of infectivity in the animals’ peripheral 
tissues, we challenged neonatal lambs orally with atypical scrapie; they were 
then killed at 12 or 24 months. Screening test results were negative for 
disease-specifi c prion protein in all but 2 recipients; they had positive 
results for examination of brain, but negative for peripheral tissues. 
Infectivity of brain, distal ileum, and spleen from all animals was assessed in 
mouse bioassays; positive results were obtained from tissues that had negative 
results on screening. These fi ndings demonstrate that atypical scrapie can be 
transmitted orally and indicate that it has the potential for natural 
transmission and iatrogenic spread through animal feed. Detection of infectivity 
in tissues negative by current surveillance methods indicates that diagnostic 
sensitivity is suboptimal for atypical scrapie, and potentially infectious 
material may be able to pass into the human food chain. 
 
SNIP... 
 
Although we do not have epidemiologic evidence that supports the effi cient 
spread of disease in the fi eld, these data imply that disease is potentially 
transmissible under fi eld situations and that spread through animal feed may be 
possible if the current feed restrictions were to be relaxed. Additionally, 
almost no data are available on the potential for atypical scrapie to transmit 
to other food animal species, certainly by the oral route. However, work with 
transgenic mice has demonstrated the potential susceptibility of pigs, with the 
disturbing fi nding that the biochemical properties of the resulting PrPSc have 
changed on transmission (40). The implications of this observation for 
subsequent transmission and host target range are currently unknown. 
 
How reassuring is this absence of detectable PrPSc from a public health 
perspective? The bioassays performed in this study are not titrations, so the 
infectious load of the positive gut tissues cannot be quantifi ed, although 
infectivity has been shown unequivocally. No experimental data are currently 
available on the zoonotic potential of atypical scrapie, either through 
experimental challenge of humanized mice or any meaningful epidemiologic 
correlation with human forms of TSE. However, the detection of infectivity in 
the distal ileum of animals as young as 12 months, in which all the tissues 
tested were negative for PrPSc by the currently available screening and confi 
rmatory diagnostic tests, indicates that the diagnostic sensitivity of current 
surveillance methods is suboptimal for detecting atypical scrapie and that 
potentially infectious material may be able to pass into the human food chain 
undetected. 
 
Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011 
 
 
 
 
 
why do we not want to do TSE transmission studies on chimpanzees $ 
 
 
 
5. A positive result from a chimpanzee challenged severly would likely 
create alarm in some circles even if the result could not be interpreted for 
man. I have a view that all these agents could be transmitted provided a large 
enough dose by appropriate routes was given and the animals kept long enough. 
Until the mechanisms of the species barrier are more clearly understood it might 
be best to retain that hypothesis. 
 
snip... 
 
R. BRADLEY 
 
 
 
 
1: J Infect Dis 1980 Aug;142(2):205-8
 
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to 
nonhuman primates.
 
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
 
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep 
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were 
exposed to the infectious agents only by their nonforced consumption of known 
infectious tissues. The asymptomatic incubation period in the one monkey exposed 
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus 
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the 
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. 
Careful physical examination of the buccal cavities of all of the monkeys failed 
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru 
has remained asymptomatic during the 39 months that it has been under 
observation. 
 
snip... 
 
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie 
by natural feeding to squirrel monkeys that we have reported provides further 
grounds for concern that scrapie-infected meat may occasionally give rise in 
humans to Creutzfeldt-Jakob disease.
 
PMID: 6997404 
 
 
 
 
Recently the question has again been brought up as to whether scrapie is 
transmissible to man. This has followed reports that the disease has been 
transmitted to primates. One particularly lurid speculation (Gajdusek 1977) 
conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and 
transmissible encephalopathy of mink are varieties of a single "virus". The U.S. 
Department of Agriculture concluded that it could "no longer justify or permit 
scrapie-blood line and scrapie-exposed sheep and goats to be processed for human 
or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is 
emphasised by the finding that some strains of scrapie produce lesions identical 
to the once which characterise the human dementias"
 
Whether true or not. the hypothesis that these agents might be 
transmissible to man raises two considerations. First, the safety of laboratory 
personnel requires prompt attention. Second, action such as the "scorched meat" 
policy of USDA makes the solution of the acrapie problem urgent if the sheep 
industry is not to suffer grievously.
 
snip... 
 
76/10.12/4.6 
 
 
 
 
Nature. 1972 Mar 10;236(5341):73-4. 
 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). 
 
Gibbs CJ Jr, Gajdusek DC.
 
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
 
Transmission of Scrapie to the Cynomolgus Monkey (Macaca 
fascicularis)
 
C. J. GIBBS jun. & D. C. GAJDUSEK
 
National Institute of Neurological Diseases and Stroke, National Institutes 
of Health, Bethesda, Maryland
 
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey 
(Macaca fascicularis) with an incubation period of more than 5 yr from the time 
of intracerebral inoculation of scrapie-infected mouse brain. The animal 
developed a chronic central nervous system degeneration, with ataxia, tremor and 
myoclonus with associated severe scrapie-like pathology of intensive astroglial 
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of 
grey matter. The strain of scrapie virus used was the eighth passage in Swiss 
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral 
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, 
Berkshire). 
 
 
 
 
 
 
 
 
 
Thursday, December 20, 2012 
 
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe 
WITH BOVINE MAD COW DISEASE 
 
 
 
 
 
 
Monday, November 30, 2009 
 
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH 
CODE 
 
 
 
 
 
 
 
Wednesday, April 24, 2013 
 
Chimpanzees Released After 30 Years Of Testing, Brace Yourself For Smiles 
 
 
 
 
 
 
 
THE OIE, USDA, CFIA, DEFRA, MAFF, $$$ POLICY OF SPREADING THE TSE PRION 
DISEASE GLOBALLY, THE LEGAL TRADING OF ATYPICAL AND POSSIBLY TYPICAL SCRAPIE AS 
A COMMODITY. ... 
 
 
 
 
absolutely insane, crazy, absurd, NEGLIGENT, take your pick. ...
 
 
 
 
TSS