Mother to Offspring Transmission of Chronic Wasting Disease in Reeves’
Muntjac Deer
Amy V. Nalls1, Erin McNulty1, Jenny Powers2, Davis M. Seelig3, Clare
Hoover1, Nicholas J. Haley1, Jeanette Hayes-Klug1, Kelly Anderson1, Paula
Stewart4, Wilfred Goldmann4, Edward A. Hoover1, Candace K. Mathiason1*
1 Department of Microbiology, Immunology and Pathology, Colorado State
University, Fort Collins, Colorado, United States of America, 2 Biological
Resource Management Division, National Park Service, Fort Collins, Colorado,
United States of America, 3 Department of Veterinary Clinical Sciences,
University of Minnesota, Saint Paul, Minnesota, United States of America, 4 The
Roslin Institute and Royal (Dick) School of Veterinary Studies, University of
Edinburgh, Easter Bush, Midlothian, Scotland, United Kingdom
Abstract
The horizontal transmission of prion diseases has been well characterized
in bovine spongiform encephalopathy (BSE), chronic wasting disease (CWD) of deer
and elk and scrapie of sheep, and has been regarded as the primary mode of
transmission. Few studies have monitored the possibility of vertical
transmission occurring within an infected mother during pregnancy. To study the
potential for and pathway of vertical transmission of CWD in the native cervid
species, we used a small cervid model–the polyestrous breeding, indoor
maintainable, Reeves’ muntjac deer–and determined that the susceptibility and
pathogenesis of CWD in these deer reproduce that in native mule and white-tailed
deer. Moreover, we demonstrate here that CWD prions are transmitted from doe to
fawn. Maternal CWD infection also appears to result in lower percentage of live
birth offspring. In addition, evolving evidence from protein misfolding cyclic
amplification (PMCA) assays on fetal tissues suggest that covert prion infection
occurs in utero. Overall, our findings demonstrate that transmission of prions
from mother to offspring can occur, and may be underestimated for all prion
diseases.
Introduction Transmissible spongiform encephalopathies (TSEs), or prion
diseases, are fatal neurodegenerative diseases that affect a variety of species,
including humans (Kuru; variant Creutzfeldt-Jakob disease [vCJD]), cattle
(bovine spongiform encephalopathy [BSE]), sheep (scrapie), mink (transmissible
mink encephalopathy [TME]), domestic and nondomestic cats (feline spongiform
encephalopathy [FSE]) and cervids (chronic wasting disease [CWD]).
The transmission of prions, while remaining poorly understood, has obvious
public health significance and requires sustained re-appraisal of present
concepts for both TSE spread and intervention strategies. It is clear that TSEs
are spread by horizontal means of transmission via oral ingestion (Kuru, BSE,
vCJD, TME, FSE, CWD) and environmental (scrapie, CWD) contact with infectious
prions, as well as by medical means including blood transfusion (vCJD, CWD,
scrapie), or iatrogenic exposure (vCJD, sCJD). While less studied, supporting
evidence has emerged for an additional path of transmission– that from mother to
offspring.
A variant form of the human prion disease CJD, known as variant CJD (vCJD),
was attributed to the ingestion of BSE-infected meat products [1], [2]. It is
known that vCJD can be transmitted from human to human via blood transfusion
from both symptomatic and asymptomatic donors [3]–[6] and that a prolonged
asymptomatic incubation period occurs in 129 Met/Val individuals [7]. It is
estimated that up to 1 in 4,000 individuals in the UK are asymptomatic carriers
of vCJD [8]–[10].
To date, 125 children have been born to women who later developed CJD [11].
This is concerning because PrPCJD has been detected in the fetal and pregnancy
related tissues of a woman infected with CJD [12]. Although decades may pass
before the onset of clinical effects associated with such transmission due to a
long subclinical carrier state, the probability that these individuals harbor
infectious prions remains high.
Animal TSEs suggest similar disease patterns. Scrapie prions have been
detected in maternal and fetal sheep tissues [13]–[22], and there is a higher
incidence of clinical disease during the lambing season [14], [23]–[27]. It is
known that BSE incidence is higher in calves born to BSE-infected cattle [28],
[29] and that BSE has been transmitted to the offspring of BSE-infected female
mice expressing bovid PrP [30]. A cheetah cub, born to a FSE-infected cheetah,
was housed in a TSE-free environment and fed a TSE-free diet, yet succumbed to
FSE at 7 years of age [31], suggesting maternal exposure.
Importantly, infectious prions are present in the brain, bodily fluids and
excreta of animals showing no clinical signs of TSE [32]–[37]. For example, the
blood, saliva and urine from deer subclinically infected with CWD contain
infectious prions [32], [38], [39]. Race and coworkers [40] found a long-term
subclinical carrier state in mice infected with hamster scrapie. Atypical BSE
(BASE) can be transmitted from non-clinical cattle to primates [41] and cattle
exposed to large oral doses of BSE can remain free of clinical signs but are
found to harbor infectious prions upon bioassay into bovid PrP transgenic mice
[42]. Taken together, these findings suggest that carriers without clinical
signs exist and may transmit infection to their offspring via milk,
transplacental trafficking or blood.
Chronic wasting disease, known for its efficient transmission among
cervids, is now recognized in 22 states, two Canadian provinces and South Korea
(http://www.nwhc.usgs.gov/disease_information/chronic_wasting_disease/index.jsp).
Studies proved that CWD can be horizontally transmitted among cervids either by
direct contact with infected animals [43], their secreta [32], [33], [38], [44]
or indirectly through the environment they inhabit [39], [45]. As observed in
other animal TSEs, even cervids in the subclinical phase of disease harbor
transmissible infectious prions [32], [39], [46]–[49]. Although there is a
growing body of evidence that identifies the sources of infectious prions, less
defined are the mechanisms by which prions are shed by infected hosts and taken
up by their naïve counterparts. One such mechanism may include that from mother
to offspring, either in utero (vertical transmission), or shortly after birth in
colostrum or milk (maternal transmission).
To address the possibility of CWD mother to offspring transmission we
utilized Reeves’ muntjac deer (Muntiacus reevesi) [50]. This small Asian deer
reaches sexual maturity at 6 months of age, is polyestrous, and can be bred and
housed conveniently in indoor controlled laboratory conditions, thus providing a
suitable in vivo model for cervid vertical/maternal transmission studies
[51].
We undertook this study to determine: 1) whether Reeves’ muntjac deer are
susceptible to CWD, 2) whether CWD is transmitted from mother to offspring, and
3) whether offspring born to CWD-infected mothers develop clinical TSE disease.
snip...
Implications Associated with Transmission of CWD to Offspring
The facile transmission of CWD seen in herds of free ranging and captive
cervids has led to questions regarding the modes of transmission. While direct
contact with infected cervids, as well as indirect contact with infectious
bodily fluids and contaminated environments are suspected to account for much of
this transmission, a third path of transmission, the one from mother to
offspring, may be underappreciated. PrPCWD placental accumulation and CWD in
utero acquirement would help explain the surprisingly high rates of CWD among
cervids. Moreover, CWD positive nonviable offspring carcasses 1) would
contribute to environmental prion contamination [77] and 2) would be available
for consumption by scavenger species, thus increasing the potential for
cross-species transmission [53,78,79]. Viable offspring may spread disease by 1)
direct cervid-to-cervid contact, 2) multigenerational mother to offspring
transmission, as well as 3) shedding infectious prions in bodily secretions
(urine, feces, saliva, blood) during their entire lifespan.
Here, in an experimental model of CWD, we have demonstrated the
transmission of infectious prions from clinical and subclinical mothers to
full-term viable, nonviable and in utero harvested offspring, revealing that the
transmission of TSEs from mother to offspring can occur and may be
underestimated for all prion diseases.
Citation: Nalls AV, McNulty E, Powers J, Seelig DM, Hoover C, et al. (2013)
Mother to Offspring Transmission of Chronic Wasting Disease in Reeves’ Muntjac
Deer. PLoS ONE 8(8): e71844. doi:10.1371/journal.pone.0071844
Editor: Ilia V. Baskakov, University of Maryland School of Medicine, United
States of America Received May 1, 2013; Accepted July 3, 2013; Published August
14, 2013
Copyright: 2013 Nalls et al. This is an open-access article distributed
under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited.
Funding: This work was supported by NIH grants N01-AI-25491 and
R01-AI-093634. The funders had no role in study design, data collection and
analysis, decision to publish, or preparation of the manuscript.
Competing Interests: The authors have declared that no competing interests
exist. * E-mail: Candace.Mathiason@colostate.edu
snip...
Sunday, August 25, 2013
Prion2013 Chronic Wasting Disease CWD risk factors, humans, domestic cats,
blood, and mother to offspring transmission
snip...
Oral.08: Mother to offspring transmission of chronic wasting disease in
Reeve's Muntjac deer Amy Nalls,1 Erin McNulty,1 Jenny Powers,2 Davis Seelig,1
Clare Hoover,1 Nicholas Haley,1 Jeanette Hayes-Klug,1 Kelly Anderson,1 Paula
Stewart,3 Wilfred Goldmann,3 Edward A. Hoover1 and Candace K. Mathiason1
1Colorado State University; Fort Collins, CO USA; 2National Park Service; Fort
Collins, CO USA; 3The Roslin Institute and Royal School of Veterinary Studies;
Edinburgh, UK To investigate the role mother to offspring transmission plays in
chronic wasting disease (CWD), we have developed a cervid model employing the
Reeve's muntjac deer (Muntiacus reevesi). Eight muntjac doe were orally
inoculated with CWD and tested PrPCWD lymphoid positive by 4 mo post infection.
Fourteen fawns were born to these eight CWD-infected doe-3 were born viable, 6
were born non-viable and 5 were harvested as fetuses from early or end-stage
CWD-infected doe. All three viable fawns have demonstrated CWD IHC lymphoid
biopsy positivity between 43 d post birth and 11 mo post birth. Two of these
three CWD positive viable offspring have developed clinical signs consistent
with TSE disease (28-33 mo post birth). Moreover, CWD prions have been detected
by sPMCA in 11 of 16 tissues harvested from 6 full-term non-viable fawns and in
7 of 11 fetal tissues harvested in utero from the second and third trimester
fetuses. Additional tissues and pregnancy related fluids from doe and offspring
are being analyzed for CWD prions. In summary, using the muntjac deer model we
have demonstrated CWD clinical disease in offspring born to CWD-infected doe,
and in utero transmission of CWD from mother to offspring. These studies provide
basis to further investigate the mechanisms of maternal transfer of prions.
snip...
Sunday, August 25, 2013
Prion2013 Chronic Wasting Disease CWD risk factors, humans, domestic cats,
blood, and mother to offspring transmission
>>> Here, in an experimental model of CWD, we have demonstrated
the transmission of infectious prions from clinical and subclinical mothers to
full-term viable, nonviable and in utero harvested offspring, revealing that the
transmission of TSEs from mother to offspring can occur and may be
underestimated for all prion diseases. <<<
EFSA Journal 2013;11(2):3080
Suggested citation: EFSA Panel on Biological Hazards (BIOHAZ); Scientific
Opinion on the risk of transmission of classical
scrapie via in vivo derived embryo transfer in ovine animals. EFSA Journal
2013;11(2):3080. [15 pp.]
doi:10.2903/j.efsa.2013.3080. Available online: www.efsa.europa.eu/efsajournal
© European Food Safety Authority, 2013
SCIENTIFIC OPINION
Scientific Opinion on the risk of transmission of classical scrapie via in
vivo derived embryo transfer in ovine animals1
EFSA Panel on Biological Hazards2, 3
European Food Safety Authority (EFSA), Parma, Italy
ABSTRACT
The risk of transmission of classical scrapie via the transfer of in vivo
derived embryo in ovines was assessed, taking into account the scientific
information made available since the last EFSA opinion on this topic (2010) (see
http://www.efsa.europa.eu/en/efsajournal/pub/1429.htm).
The potential impact of PrP genotype of the embryo and/or of the ram and donor
ewe on this risk was also assessed. The new data made available over the last
three years further reinforce the view that classical scrapie could be
vertically transmitted in sheep. Since the possibility of such vertical
transmission was already considered in the previous opinion, its conclusions and
recommendations relating to the risk of classical scrapie transmission via
embryo transfer remain valid. In ovines, the susceptibility to classical scrapie
infection in sheep is strongly influenced by certain polymorphisms of the PrP
gene. Under natural exposure conditions, animals that are heterozygous or
homozygous A136R154R171 display respectively a low or negligible risk of being
infected. The genetic control of the susceptibility to classical scrapie is also
likely to impact on the risk of transmitting the disease via embryo transfer.
Irrespective of the embryo’s genotype, embryos derived from rams and dams
carrying at least one ARR allele would significantly decrease this risk
(compared to an embryo from parents of unknown genotypes). The use of homozygous
ARR embryos would provide the highest level of safety regarding the risk of
transmitting classical scrapie through embryo transfer (in vivo derived
embryos). The use of heterozygous ARR embryos would ensure a higher level of
safety compared to Q171/Q171 embryos. Finally, it was concluded that, providing
the OIE recommendations and procedures relating to embryo transfer are adhered
to, the risk of transmitting classical scrapie due to the transfer of homozygous
or heterozygous ovine ARR embryos can be considered negligible.
© European Food Safety Authority, 2013
KEY WORDS
Classical scrapie, ovine, sheep, embryo transfer, transmission risk
1 On request from the European Commission, Question No EFSA-Q-2012-00647,
adopted on 24 January 2013.
2 Panel members: Olivier Andreoletti, Dorte Lau Baggesen, Declan Bolton,
Patrick Butaye, Paul Cook, Robert Davies, Pablo S. Fernandez Escamez, John
Griffin, Tine Hald, Arie Havelaar, Kostas Koutsoumanis, Roland Lindqvist, James
McLauchlin, Truls Nesbakken, Miguel Prieto Maradona, Antonia Ricci, Giuseppe Ru,
Moez Sanaa, Marion Simmons, John Sofos and John Threlfall. One member of the
Panel did not participate in the discussion on the subject referred to above
because of potential conflicts of interest identified in accordance with the
EFSA policy on declarations of interests. Correspondence: biohaz@efsa.europa.eu
3 Acknowledgement: The Panel wishes to thank the members of the Working
Group on the risk of transmission of classical scrapie via in vivo embryo
transfer in ovine animals: Olivier Andreoletti, Nora Hunter and Ciriaco Ligios
for the preparatory work on this scientific opinion and the hearing experts:
Michel Thibier and Pascale Chavatte-Palmer, and EFSA staff: Pablo Romero Barrios
for the support provided to this scientific opinion.
snip...
CONCLUSIONS AND RECOMMENDATIONS
CONCLUSIONS Since the 2010 opinion only two relevant studies have been
published. None of them suggest a need to revise the previous conclusion adopted
by the BIOHAZ Panel in January 2010 i.e ‘Based on the data currently available
the risk of TSE transmission associated with embryos collected from Classical
scrapie incubating ewes and she-goats ranges from negligible to low. However,
data are insufficient to conclude that such a risk is negligible.’ In sheep the
susceptibility to classical scrapie infection is strongly influenced by the
polymorphisms of the PrP gene. Under natural exposure conditions, animals that
are heterozygous or homozygous ARR show respectively a low or negligible risk of
being infected by classical scrapie. This genetic control of the susceptibility
to classical scrapie infection directly influences the risk of transmitting the
disease via embryo transfer:
– Irrespective of the embryo’s genotype, the use of embryos derived from
rams and dams carrying at least one ARR allele would significantly decrease the
risk of transmitting classical scrapie via embryo transfer (by comparison to an
embryo from parents of unknown genotypes).
– The use of homozygous ARR embryos would provide the highest possible
level of safety with regard to the risk of transmitting classical scrapie
through embryo transfer (in vivo derived embryos).
– The use of heterozygous ARR embryos would ensure a higher level of safety
by comparison with Q171/Q17110 embryos.
– The risk of transmitting classical scrapie by implantation of a Q171/Q171
embryo collected from a dam with an unknown classical scrapie status cannot be
considered negligible. Providing that the OIE recommendations and procedures
relating to embryo transfer are adhered to, the risk of transmitting classical
scrapie by the implantation of homozygous or heterozygous ovine ARR embryos can
be considered negligible.
RECOMMENDATIONS The recommendations relating to the risk of transmitting
classical scrapie via embryo transfer that were formulated in the 2010 opinion
remain valid. In particular, the presence of infectivity in ovine embryos
collected from scrapie infected dams bearing susceptible genotypes needs to be
assessed before a definitive assessment of the risk of transmitting classical
scrapie via the use of embryos bearing a susceptible genotype can be made.
10 By convention Q171 refers to ARQ, VRQ, and AHQ alleles.
>>>Finally, it was concluded that, providing the OIE
recommendations and procedures relating to embryo transfer are adhered to, the
risk of transmitting classical scrapie due to the transfer of homozygous or
heterozygous ovine ARR embryos can be considered negligible.<<<
‘’LOL’’ ! ...tss
Tuesday, April 30, 2013
Transmission of classical scrapie via goat milk
Veterinary Record2013;172:455 doi:10.1136/vr.f2613
Friday, May 10, 2013
Evidence of effective scrapie transmission via colostrum and milk in
sheep
Thursday, April 26, 2012
Maternal Transmission of the BSE and Birth Cohorts
December 14, 2011
Detection of PrPres in Genetically Susceptible Fetuses from Sheep with
Natural Scrapie
María Carmen Garza1, Natalia Fernández-Borges2, Rosa Bolea1, Juan José
Badiola1, Joaquín Castilla2,3, Eva Monleón1,4*
1 Centro de Investigación en Encefalopatías Espongiformes Transmisibles y
Enfermedades Emergentes, Universidad de Zaragoza, Zaragoza, Spain, 2 CIC
bioGUNE, Parque Tecnológico de Bizkaia, Derio, Spain, 3 IKERBASQUE, Basque
Foundation for Science, Bilbao, Spain, 4 Producció Animal, Universitat de
Lleida, LLeida, Spain
Scrapie is a transmissible spongiform encephalopathy with a wide PrPres
dissemination in many non-neural tissues and with high levels of
transmissibility within susceptible populations. Mechanisms of transmission are
incompletely understood. It is generally assumed that it is horizontally
transmitted by direct contact between animals or indirectly through the
environment, where scrapie can remain infectious for years. In contrast, in
utero vertical transmission has never been demonstrated and has rarely been
studied. Recently, the use of the protein misfolding cyclic amplification
technique (PMCA) has allowed prion detection in various tissues and excretions
in which PrPres levels have been undetectable by traditional assays. The main
goal of this study was to detect PrPres in fetal tissues and the amniotic fluid
from natural scrapie infected ewes using the PMCA technique. Six fetuses from
three infected pregnant ewes in an advanced clinical stage of the disease were
included in the study. From each fetus, amniotic fluid, brain, spleen,
ileo-cecal valve and retropharyngeal lymph node samples were collected and
analyzed using Western blotting and PMCA. Although all samples were negative
using Western blotting, PrPres was detected after in vitro amplification. Our
results represent the first time the biochemical detection of prions in fetal
tissues, suggesting that the in utero transmission of scrapie in natural
infected sheep might be possible.
Citation: Garza MC, Fernández-Borges N, Bolea R, Badiola JJ, Castilla J, et
al. (2011)
Detection of PrPres in Genetically Susceptible Fetuses from Sheep with
Natural Scrapie.
PLoS ONE 6(12): e27525. doi:10.1371/journal.pone.0027525
Editor: Jason Bartz, Creighton University, United States of America
Received: July 20, 2011; Accepted: October 18, 2011; Published: December
14, 2011
Copyright: © 2011 Garza et al. This is an open-access article distributed
under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited.
Funding: This study was supported by a national grant from Spain
(AGL2009-11553-C02-01), a Basque government grant (PI2010-18) and the Department
of Industry, Tourism and Trade of the Government of the Autonomous Community of
the Basque Country (Etortek Research Programs 2011/2013), from the Innovation
Technology Department of the Bizkaia County. Dr. Garza was supported by a FPU
doctoral grant from the Spanish Ministry of Education (AP2007-03842). The
funders had no role in study design, data collection and analysis, decision to
publish, or preparation of the manuscript.
Competing interests: The authors have declared that no competing interests
exist.
* E-mail: emonleon@unizar.es
SEMEN AND TSE INFECTIVITY
Saturday, February 11, 2012
PrPSc Detection and Infectivity in Semen from Scrapie-Infected Sheep
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
PRION 2010
Wednesday, December 30, 2009
Is there evidence of vertical transmission of variant CJD ?
J Neurol Neurosurg Psychiatry doi:10.1136/jnnp.2009.172148
Monday, November 22, 2010
SHEEP WITH MASTITIS TRANSMIT INFECTIOUS PRIONS THROUGH THE MILK
Monday, November 22, 2010
SHEEP WITH MASTITIS TRANSMIT INFECTIOUS PRIONS THROUGH THE MILK
J. Virol. doi:10.1128/JVI.02022-10 Copyright (c) 2010, American Society for
Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.
Saturday, December 3, 2011
Isolation of Prion with BSE Properties from Farmed Goat
Volume 17, Number 12—December 2011
Prion disease spreads in sheep via mother's milk
communicated by: Terry S Singeltary Sr [
These research confirms experimentally previous observations by others (such as,
Lacroux C et al: Prions in Milk from Ewes Incubating Natural Scrapie. PLoS
Pathog 4(12): e1000238.doi:10.1371/journal.ppat.1000238;
SNIP...
-------- Original Message --------
Subject: POSITION STATEMENT MATERNAL TRANSMISSION OF vCJD SEAC
Date: Fri, 04 Feb 2005 09:54:58 –0600
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy
CC: cjdvoice@yahoogroups.com, Agriculture@mail.house.gov
1
POSITION STATEMENT MATERNAL TRANSMISSION OF vCJD
Issue
1. The Chief Medical Officer for England asked SEAC to consider current
evidence and comment on the potential transmission of vCJD from mother to child
via human breast milk. In utero transmission was also considered. The committee
also commented on the scientific basis of a risk reduction measure for possible
transmission of vCJD via banked breast milk.
Background
2. No diagnostic test is currently available for the detection of abnormal
PrP in milk. Research is under way to develop tests to screen for the possible
presence of abnormal prion protein (PrP) in milk samples from cattle
experimentally infected with BSE1. These modified tests may also be applicable
to human milk. However, it is not yet clear when/if a reliable test will be
available.
3. A small number of breast milk banks in the UK supply highly vulnerable
premature babies for whom no milk may be available from the mother. A model
developed by the Department of Health to assess the effect of pooling breast
milk from multiple donors on the possible risks of transmission of vCJD via
breast milk banks was considered.
4. There is some, albeit limited, published epidemiological and
experimental research on maternal transmission of prion diseases. There are also
unpublished surveillance data of children born to vCJD cases from the National
CJD Surveillance Unit and UK surveillance of neurological illness in children
which might inform on potential risks of maternal transmission.
1 A joint FSA/SEAC milk working group is monitoring and providing advice
on this research carried out at the Veterinary Laboratories Agency.
2 Breast milk banks
5. There is no evidence that vCJD infectivity has ever been transmitted
through breast milk. However, a theoretical risk exists. Modelling studies
clearly show that the practice of pooling breast milk increases the number of
donors to which a recipient is exposed and thereby increases the potential risk
of an infant receiving milk contaminated with vCJD infectivity. The theoretical
risk of infection can be minimised by not pooling the milk, by the use of
individual hand operated breast milk pumps for single donors, and by the use of
single-use sterilised bottles for collection. In addition, available evidence
suggests that infection/inflammation of the breast results in increased
lymphocytes in milk and therefore increased risk of infectivity. This risk would
be minimised if milk from donors showing signs of infection is not used.
6. The committee suggested that, if practicable, milk could be stored for
an appropriate period of time to allow the health status of donors to be
monitored, before it is released. However, information was not available to the
committee on whether long-term storage of human milk is detrimental to its
nutritional quality.
Maternal transmission
7. There is evidence from animal studies for low level maternal
transmission of prions in cattle and sheep. This transmission may occur in
utero, via milk and/or perinatally. However, the possibility that this putative
maternal transmission might have been due to another mode of transmission, for
example through a contaminated environment or feed, cannot be ruled out.
8. In contrast, in humans there is no evidence for maternal transmission
in cases of familial prion disease, other than the transfer of a mutant form of
the PrP gene, and there is no evidence of maternal transmission of Kuru.
However, compared with other human prion diseases vCJD may pose a greater risk
because of the greater involvement of the lymphoreticular system in vCJD
pathogenesis. Although, breast tissue (and placenta) from a single vCJD case
tested negative for PrPvCJD, transfer of infectivity to breast milk may depend
on the physiological status of the mammary gland. Similar tests or infectivity
bioassays have not been conducted on breast tissue from lactating patients with
vCJD. 3
9. A published study suggesting transmission of sCJD in colostrum2 was
considered unreliable because tissues not normally associated with high levels
of infectivity (blood and placenta) showed equivalent infectivity to that of the
brain in this study.
10. Analysis of prospective surveillance data of UK children born to
mothers with, or that had subsequently developed clinical vCJD, provide no
evidence for maternal transmission of vCJD. However, the number of cases is very
small and the incubation period of vCJD, if transmitted from mother to child, is
unknown and so the children may yet be too young to have developed symptoms.
11. The phenotype of BSE infection in humans expressing PrP genotypes
other than M/M at codon 129 is not known. Given recently published studies in
mice expressing the human PrP gene3, which suggest that the human PrP genotype
may affect disease phenotype, the committee considered it very important that
undiagnosed neurological diseases be carefully monitored. In this respect,
amongst others, it is recommended that the careful monitoring of neurological
illnesses through the PIND surveillance of children4 continue.
Conclusions
12. In summary, there is currently no epidemiological evidence for
maternal transmission of vCJD, including transmission via breast milk. However,
there is a hypothetical risk. Although available evidence is limited and mostly
indirect rather than direct, this risk, if any, appears to be low. As a risk
cannot be excluded, a watching brief should be maintained.
SEAC
January 2005
2 Tamai Y et al. Demonstration of the transmissible agent in tissue from a
pregnant woman with CJD. New Eng J Med 1992 327, 649.
3 Wadsworth et al. Human prion protein with valine 129 prevents expression
of variant CJD phenotype. Science. 2004 306, 1793-1796.
4 Devereux G et al. Variations in neurodegenerative disease across the UK:
findings from the national study of Progressive Intellectual and Neurological
Deterioration (PIND). Arch Dis Child. 2004 89, 8-12.
Date: Thu 20 Jan 2005
From: Terry S. Singeltary Sr.
Chronic Lymphocytic Inflammation Specifies the Organ Tropism of Prions
----------------------------------------------------------------------[
The following is the summary of a paper by Mathias Heikenwalder and 8
others, published in Science online, 10.1126/science.1106460, Thu 20 Jan 2005
This paper describes work that illustrates that chronic inflammatory
conditions may affect and expand the natural and iatrogenic transmission of
prions - Mod.CP]Prions typically accumulate in nervous and lymphoid tissues.
Because proinflammatory cytokines and immune cells are required for lymphoid
prion replication, we tested whether inflammatory conditions affect prion
pathogenesis. We administered prions to mice with 5 inflammatory diseases of
kidney, pancreas or liver. In all cases, chronic lymphocytic inflammation
enabled prion accumulation in otherwise prion-free organs. Inflammatory foci
consistently correlated with lymphotoxin upregulation and ectopic induction of
PrPC-expressing FDC-M1+ cells, whereas inflamed organs of mice lacking
lymphotoxin-alpha or its receptor accumulate neither PrPSc nor infectivity upon
prion inoculation. By expanding the tissue distribution of prions, chronic
inflammatory conditions may act as modifiers of natural and iatrogenic prion
transmission.******[4]Date: Thu 20 Jan 2005
From: Terry S. Singeltary Sr.
Source: Reuters News Agency, Thu 20 Jan 2005 [edited]
Study Finds that Illness May Promote Spread of Mad Cow Prion
------------------------------------------------------------
The agent that transmits mad cow disease and related diseases may spread
further in the body of an animal suffering from certain illnesses, scientists
said on Thu 20 Jan 2005. Their finding raises the question of whether measures
aimed at curbing the spread of mad cow disease, or bovine spongiform
encephalopathy (BSE), are adequate, the researchers said.Tests on mice showed
that prions, the protein-like fragments that transmit BSE and related diseases
[e.g. variant Creutzfeldt-Jakob disease in humans], can show up in organs they
are not supposed to if the mouse has an inflammatory condition. Scientists have
believed that BSE-causing prions are limited to the brain, spleen, spinal cord
and lymph tissue, although some tests have suggested blood and muscle tissue may
also harbor the prions. The latest study, published in the journal Science,
suggests prions may also sometimes be found in the kidney, pancreas and liver.
"We administered prions to mice with 5 inflammatory diseases of kidney, pancreas
or liver," wrote the researchers, led by top prion expert Dr. Adriano Aguzzi of
the University Hospital of Zurich in Switzerland.Aguzzi and colleagues in
Britain and the United States inoculated specially bred mice with prions and
checked to see if the prions spread in their bodies when the mice had an
inflammatory condition. This is because other studies had suggested that prions
might be attracted to immune system inflammatory cells. "In all cases, chronic
lymphocytic inflammation enabled prion accumulation in otherwise prion-free
organs," the researchers wrote.BSE peaked in British cattle herds in the
mid-1990s, and a few cases have been reported in other countries. Canada
reported its 3rd case this month. People who eat BSE-infected beef products can
develop a related human brain disease called variant Creutzfeldt-Jakob disease
or vCJD. There is no treatment or cure. [As of 4 Feb 2005, so far in the UK for
the year 2005 there have 8 referrals of suspected CJD; and there have been 8
deaths from sporadic CJC, one from GSS and none from familial, iatrogenic or
variant CJD. - Mod.CP]. It has killed 148 Britons, and 5 [now 6] Britons are
alive with the disease, according to the British Department of Health's monthly
report on the disease. The World Health Organization says it has reports of 6
cases in France, one in Ireland, one in Italy, one in Canada and one in the
United States [and one in Japan: see; ProMED-mail post "CJD (new var.) - Japan:
death 20050204.0381" - Mod.CP] Experts believed BSE first appeared when cattle
were fed improperly rendered remains of sheep infected with scrapie, a related
disease. In 1997, the United States and Canada imposed animal feed bans, and
have mandated the removal of materials believed to carry infectious prions.
These include the skull, brain, nerves attached to the brain, eyes, tonsils,
spinal cord and attached nerves, plus a portion of the small intestine. The
study suggests that even symptom-free animals may also have prions in their
liver, kidney, and pancreas.--
Terry S. Singeltary Sr.flounder@wt.net
****** [5]Date: Fri 21 Jan 2005From: ProMED-mail promed@promedmail.org
Souce: New York Times, Fri 21 Jan 2005 [edited]
Study Finds Broader Reach for Mad Cow Proteins
----------------------------------------------
Mad cow disease has long been thought to occur in just the brains and
nervous systems of infected animals. But scientists are reporting today that the
proteins thought to cause the disease can travel to other organs as well. The
research is based on experiments with mice, but if it is borne out in other
species, it may suggest that no part of an infected animal is safe to eat. The
disease leads to a fatal brain disease in humans [variant Creutzfeldt-Jakob
disease].In the mouse experiments, reported in the journal Science [see [3]
above], researchers in Switzerland found that prions, proteins that are the
infectious agent in mad cow disease, follow immune cells, called lymphocytes, in
the body. When mice were given chronic infectious diseases of the liver, kidney
and pancreas and then inoculated with prions, the prions made their way to the
infected organs. Dr. Adriano Aguzzi, a neuropathologist at the University
Hospital in Zurich, who led the experiments, said this meant that cows and sheep
infected with prions could harbor the disease in any inflamed organ.But Dr.
David R. Smith, a veterinarian at the University of Nebraska, said the research
did not raise alarms about American beef. For one thing, he said, livestock with
obvious signs of systemic infection, like a fever, are not allowed into the food
supply. And most American cattle are slaughtered while they are young and at
reduced risk of infection. Many countries, including the United States, require
the removal of skulls, brains, eyes, spinal cords and other nervous tissues from
slaughtered animals because prions are known to accumulate in those tissues.
Even in countries with mad cow disease, mainly in Europe, meat is considered
safe if those tissues are removed, Dr. Aguzzi said. But the disease could spread
more readily if infections are not obvious or if inspections are sloppily done,
he said.[
Byline: Sandra Blakeslee]--ProMED-mail
******[6]Date: Fri 4 Feb 2005
From: Terry S. Singeltary Sr.
Source: Spongiform Encephalopathy Advisory Committee (SEAC), Position
Paper, January 2005 [edited]
SNIP...SEE FULL TEXT ;
-------- Original Message --------
Subject: re-Mother passes on CJD to unborn baby
SUNDAY TELEGRAPH Sun, 17
Sep 2000 10:09:01 –0700
Date: Sun, 26 Dec 2004 11:32:26 –0600
From: "Terry S. Singeltary Sr."
Reply-To: BSE-L
Greetings list members,
> re-Mother passes on CJD to unborn baby SUNDAY TELEGRAPH Sun, 17 Sep
> 2000 10:09:01 –0700
WHY is it we have heard nothing else about this case?
WHAT is the latest about this child that was supposedly to have been
infected with nvCJD via his mother from birth, after which the mother passed on
with confirmed nvCJD ???
thank you, kind regards,
Terry
Date: Sun, 17 Sep 2000 10:09:01 –0700
Reply-To: BSE-L
Sender: BSE-L
From: "Terry S. Singeltary Sr."
Subject: Mother passes on CJD to unborn baby SUNDAY TELEGRAPH
Bovine Spongiform Encephalopathy
ISSUE 1941 Sunday 17 September 2000
Mother passes on CJD to unborn baby
By Rajeev Syal, Jenny Booth and Chris Hastings Scientists shocked as
disease reveals new deadly traits Tragic inheritance of baby 'born with CJD' BSE
report DOCTORS believe that a baby girl has been born with new variant
Creutzfeldt-Jakob disease, the human form of mad cow disease. Her mother died of
the illness earlier this year. Four specialists who have examined the
11-month-old girl believe that she is exhibiting the symptoms of vCJD and that
she contracted the condition in the womb. The Telegraph knows the identity of
the child, but cannot name her for legal reasons. The specialists have passed on
their findings to the child's grandmother after tests failed to detect any other
ailment in the girl. Only a post-mortem examination, however, can offer
conclusive proof of vCJD. If confirmed, this would be the first known example of
vCJD being transmitted from mother to child, and will heighten fears that the
disease can be transmitted through blood. One leading microbiologist believes
that some of the 67 people who have already died of vCJD may have inherited it
from their mothers, rather than contracting it from eating infected meat. The
baby's 50-year-old grandmother, who is now her legal guardian, said the doctors
suspected that prions - the infectious agents believed to cause the disease -
had been passed on to the baby in the womb and had given her brain damage. She
said: "They don't know if it's gone into incubation. If so, it could be years
before we can finally confirm the disease." The health of the child has been the
subject of speculation since her mother died of vCJD in May, seven months after
giving birth. The girl was found to have brain damage and has been suffering
from fits and convulsions. Doctors have said that she is growing at half the
normal rate for a child of her age and suffers from poor sight and abnormally
stiff limbs. Her appendix has been examined by doctors looking for signs of
vCJD, but the tests proved inconclusive. She will undergo further brain scans
later this year. On Friday, The Lancet reported research by scientists at the
Institute for Animal Health confirming for the first time that BSE can be
transmitted in sheep by infected blood transfusions. The finding increases the
likelihood that a vCJD-infected mother could pass on the disease to her baby.
Richard Lacey, the emeritus professor of medical microbiology at Leeds
University, said that it was "inevitable" that infected mothers would pass on
vCJD through the placenta. He said: "The only thing that is uncertain is the
scale on which it is happening." New variant CJD, a fatal disease of the brain
and nervous system, is believed to have been transmitted to humans through
eating beef infected with bovine spongiform encephalopathy. The Ministry of
Agriculture admitted in 1996 that a pregnant cow could pass BSE to its unborn
calf. Maternal transmission also occurs in sheep, rats and mice. Scientists have
observed that offspring often develop the disease more virulently than the
parent and after a much shorter incubation period. Until now, researchers have
been baffled at the youth of the 67 people known to have died of vCJD. Their
average age is 27. Most victims were aged 18 to 40. Dr Rob Will, of the National
CJD Surveillance Unit in Edinburgh, has suggested that the victims caught the
disease through eating cheap mechanically recovered meat used in school meals or
even baby food. Dr Lacey however suspects that some may have contracted the
illness from their mothers. The Telegraph has also learnt that the Department of
Health is now considering the use of disposable surgical instruments throughout
the NHS because of growing concern that blood and tissue from vCJD carriers
could remain infectious even after sterilisation. As this newspaper revealed
four years ago, tissue from such patients is capable of passing ordinary CJD to
healthy patients, yet current standards for sterilising equipment are not
adequate to destroy the prions.
ISSUE 1941 Sunday 17 September 2000
Tragic inheritance of baby 'born with CJD'
By Rajeev Syal, Chris Hastings and Jenny Booth
Mother passes on CJD to unborn baby THE dark-haired baby attracts admiring
glances wherever she goes. She has her mother's striking blue eyes, says her
adoring grandmother. Medical experts believe, however, that she is the first
child to inherit the disease which killed her mother, variant Creutzfeldt-Jakob
disease, while still in the womb. The child's 50-year-old grandmother, who has
to feed the 11-month-old girl through a tube, said: "Every time I look at her, I
see the agony that my daughter endured in her last days. Seeing my own child die
in agony nearly killed me and now I am terrified that I will also see my
grandchild die in the same way." For months she suspected that her granddaughter
was suffering from the symptoms of vCJD. Over recent weeks, her worst fears have
been confirmed by doctors from the leading London hospital that is treating her
grandchild. The tragedy began in July 1998, when the woman's 22-year-old
daughter - who ran her own catering business - became moody, tired and
constantly tearful. Her daughter's frequent outbursts of temper were untypical;
the two normally lived harmoniously together in a semi-detatched home in a
Warwickshire village. By February last year, the young woman had become
pregnant. She developed severe back trouble and, five months into her pregnancy,
had to give up work. She could hardly move her arms and legs and had to be
helped around the house. Other symptoms included pins and needles in her legs
and swollen and sore lips. Doctors were mystified as to the cause of the
illness. In October, to try to protect mother and child, the baby girl was
delivered by Caesarean section weighing 6lb 4oz. Immediately, however, the
doctors were aware that the little girl had difficulties swallowing and she was
placed in a special baby care unit. The family was told by doctors two days
after the birth that the baby probably had brain damage. The specialists decided
to conduct a series of tests on the child. The family had begun to guess the
truth. The dead woman's mother recalled: "I had spoken to someone who told me
that their relative had died of CJD, and I had seen the news reports on
television about cows. Then it dawned on me; my daughter's moods and the jerky
movements she had begun to suffer were similar. It was an awful moment." By
January, vCJD was confirmed in the mother by a biopsy on her tonsils - a
procedure that is 98 per cent accurate.After this she was warned that her baby
may also have contracted the disease.The young woman was virtually confined to a
wheelchair and her memory was so bad that she sometimes failed to recognise her
mother and her child. The baby's father had moved away from the area. Doctors
tried to discover if her baby also had vCJD, but because the case was unique,
and hampered by the poor health of the child, they were not sure how to reach a
diagnosis. In May, the child's mother died after months of suffering. In the
same month, doctors removed the little girl's appendix and took samples of her
lymph tissue in the hope that an analysis would show whether she was carrying
the prions - aberrant proteins - that caused the disease that killed her mother.
According to the baby's grandmother, the tests carried out by the doctors were
necessarily inconclusive because vCJD infection can only be finally confirmed
after death through a post-mortem. They nevertheless believe that her
granddaughter has been suffering from the effects of vCJD from the time of her
conception. Caring for her dead daughter's child has now become the focus of the
woman's life, and it is proving to be a daunting task. Her granddaughter's
eyesight has been affected, and it is impossible to know how much she can see.
Her limbs are stiff and she needs physiotherapy. She sleeps a lot of the time,
as her mother did when she was ill. Last week she was suffering fits and
convulsions, and doctors have said that she is growing at half the normal rate.
She is to undergo further examinations by vCJD specialists later this year. Her
grandmother said: "The appalling thing is that I am watching my granddaughter
die while the Government fails to warn others that they may be passing the
disease on. I want this baby's case highlighted because no other family should
go through what we have been through,"
ISSUE 1941 Sunday 17 September 2000
Scientists shocked as disease reveals new deadly traits
By Robert Matthews
THE growing concern over the health of the baby born to a mother with
variant CJD and the new evidence, announced last week, that the disease may have
spread through blood transfusions, highlight the disturbing ability of vCJD to
surprise the experts. A conclusive diagnosis of vCJD in the 11-month-old child
would imply a far shorter incubation period than many scientists thought
possible. It would also mean that the disease can be passed down the
generations, not merely acquired through contact with infected tissue.
Scientists have yet to pin down the likely size of the vCJD epidemic, with
estimates for Britain ranging from about 100 cases to more than 100,000; so far
there have been only 82 probable or confirmed cases. The case of the baby girl
means that scientists are now admitting that many predictions may have to be
rethought. According to Dr Neil Ferguson of the University of Oxford, a leading
expert on the mathematics of epidemics, so-called vertical transmission down the
generations has, at least until now, been thought to be relatively unimportant.
Dr Ferguson said: "It very much depends on the probability of it taking place.
If a woman has to be very sick and symptomatic in order to give it to her baby,
then the number of cases it creates are going to be very small, because she is
unlikely to get pregnant." The mother of the baby at the centre of the current
concern is, however, understood to have been showing only minor symptoms of the
disease at the time of conception; only later in her pregnancy did she develop
the classic symptoms of vCJD from which she died in May this year. Richard
Lacey, an emeritus professor of medical microbiology at Leeds University, said
that the Telegraph story had wider implications than its obvious medical
significance. Prof Lacey said: "The only thing that is certain is the scale on
which it is happening. That will take decades to find out. I am aware of other
cases where maternal transmission could be an issue; this isn't the only one.
"This poses difficult ethical problems: to what extent should individuals be
told of the risk? Should such a person be told not to give blood when he or she
is old enough to do so? What effect will this knowledge have on that person's
way of life, on their emotions? There needs to be some sort of discussion about
how we cope with it, but at the moment there is nothing." Epidemiologists are
puzzled by the fact that vCJD is predominantly affecting young people, while
classic CJD is a disease of the old. Prof Lacey suggested that one possible
explanation is that the teenagers and young people who have died from vCJD may
have contracted the disease from their mothers while in the womb. He added that
it is a recognised clinical syndrome that infectivity accumulates when it is
passed on from one generation to the next. Dr Stephen Dealler, an expert on BSE
and vCJD at Burnley Hospital, said that it had been believed that
mother-to-offspring infection could take place in cows, but only with a
considerable incubation period. "The thinking is that it may take place in cows,
but the incubation period is still three to five years," he said. "This would
lead us to expect an incubation period of around seven years in humans. If the
baby does have vCJD, that's a very fast incubation period." Last week's
revelation resulting from animal experiments that the disease may be transferred
through blood transfusions from infected people with no symptoms was being
played down by government scientists, who said that all British blood is
screened to remove cells that may harbour vCJD. The Telegraph has learnt,
however, that the Department of Health is seriously considering the use of
disposable surgical instruments throughout the National Health Service. This
follows growing concern that blood and tissue from asymptomatic carriers of the
disease could remain infectious even after sterilisations.
TSS
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
============================================
Subject: mother-to-child CJD (more downplaying or just hope?)
Date: Thu, 21 Sep 2000 20:56:41 –0700
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de
######### Bovine Spongiform Encephalopathy #########
The first case of mother-to-child transmission of vCJD?
Lancet 2000; 356: 1085 - 1092 Download PDF (109 Kb)
Last week UK newspapers reported that four UK doctors had revealed that
they suspected an 11-month-old girl had variant Creutzfeldt-Jakob disease
(vCJD), which she may have contracted from her mother, who died from the disease
in May.
The child has brain damage, is unable to swallow, and has convulsions and
stiff limbs. Doctors have not been able to give another explanation for her
signs and tests for the abnormal prion protein in appendix and lymph tissue have
been inconclusive. Proof of vCJD in adults requires examination of brain tissue
at necropsy or by brain biopsy.
Prof James Ironside, a neuropathologist from the National Creutzfeldt-Jakob
Disease Surveillance Unit, Edinburgh, UK, says "the position we are in just now
is suspicion, as far as I am aware, and it is not proven that this child has the
disease". In occasional cases of iatrogenic CJD occurring in younger patients
during pregnancy there has been no evidence of maternal transmission, adds
Ironside.
Chris Verity, a paediatric neurologist from Addenbrooke's Hospital,
Cambridge, UK, who is part of a national paediatric vCJD surveillance team,
stresses the need for caution before drawing conclusions from this case. "We
cannot comment on individual cases . . . and there are a whole lot of reasons
why children may have problems with development".
Haroon Ashraf
############ http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
############
so, what about sutures ???
Other US BSE risks: the imported products picture 24 Jul 00 Trade
Statistics: UK to US Compiled by Terry S. Singeltary Sr of Bacliff, Texas
[Opinion (webmaster): The US has focused for years on tracing, containing,
and eradicating live animal imports from the UK or other countries with
acknowledged BSE like Belgium, including some 499 cattle and the Vermont sheep.
This strategy does not acknowledge imports of rendered bovine products from
England during the BSE period nor secondary products such as surgical catgut,
which is to say surgical cowgut, or dairy cattle embryos, vaccines for
veterinarian and human medicines. What has become of these?
Mr. Singeltary, who lost his mother to CJD of unexplained origin a few
years back and went on to became a well-known TSE activist, has tracked down
voluminous pertinent import data through correspondence with UK officials and
searches of government web sites. Imports of such products are frequently cited
by Europeans in rating BSE risks in the US and in shutting out US exports.
Many people's eyes glaze over when reviewing reams of sometimes older trade
statistics. There is no proof that any of the imported products was contaminated
with BSE nor if so, any evidence that any BSE product lead to infection in US
livestock, surgical patients, or what not. Nonetheless, the data obtained by Mr.
Singeltary establish that an appalling variety and tonnage of products that were
imported by the US from the UK and othr BSE-affected countries during the peak
of the BSE epidemic years.
10 January 1990 COMMERCIAL IN CONFIDENCE
NOT FOR PUBLICATION
COMMITTEE ON SAFETY OF MEDICINES WORKING PARTY ON BOVINE SPONGIFORM
ENCEPHALOPATHY
SURGICAL CATGUT SUTURES 2.1 At the first meeting of the Working Party on
Bovine Spongiform Encephalopathy on 6 September 1989, detailed consideration was
given to XXXXX Surgical Catgut. This arose from the Company's response to the
Letter to Licence Holders, indicating that the bovine small intestine source
material was derived from UK cattle, unlike 8 other licenced catgut sutures. In
contrast XXXXX Surgical Catgut was stated to hold over 90% share of the market
for catgut sutures, and to constitute approximately 83% of all sutures used in
U.K. IMPORTS OF SUTURES FROM THE KNOWN BSE COUNTRY;
see full text ;
Tuesday, February 8, 2011
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
Friday, December 23, 2011
Detection of PrPres in Genetically Susceptible Fetuses from Sheep with
Natural Scrapie
Sunday, September 1, 2013
Evaluation of the Zoonotic Potential of Transmissible Mink Encephalopathy
We previously described the biochemical similarities between PrPres derived
from L-BSE infected macaque and cortical MM2 sporadic CJD: those observations
suggest a link between these two uncommon prion phenotypes in a primate model
(it is to note that such a link has not been observed in other models less
relevant from the human situation as hamsters or transgenic mice overexpressing
ovine PrP [28]). We speculate that a group of related animal prion strains
(L-BSE, c-BSE and TME) would have a zoonotic potential and lead to prion
diseases in humans with a type 2 PrPres molecular signature (and more
specifically type 2B for vCJD)
snip...
Together with previous experiments performed in ovinized and bovinized
transgenic mice and hamsters [8,9] indicating similarities between TME and
L-BSE, the data support the hypothesis that L-BSE could be the origin of the TME
outbreaks in North America and Europe during the mid-1900s.
Monday, September 02, 2013
Atypical BSE: role of the E211K prion polymorphism
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Location: Virus and Prion Research Unit
Saturday, July 6, 2013
Small Ruminant Nor98 Prions Share Biochemical Features with Human
Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive
Prionopathy
Research Article
Sunday, August 11, 2013
Creutzfeldt-Jakob Disease CJD cases rising North America updated report
August 2013
Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing
an extreme increase of 48% between 2008 and 2010
Friday, August 16, 2013
Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and
Contaminated blood products induce a highly atypical prion disease devoid of
PrPres in primates
Sunday, March 31, 2013
Creutzfeldt Jakob Disease CJD worlds youngest documented victim, 11 years
old, shall we pray
Monday, January 14, 2013
Gambetti et al USA Prion Unit change another highly suspect USA mad cow
victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes
along with this BSe
Monday, December 31, 2012
Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State,
2006–2011-2012
Saturday, December 29, 2012
MAD COW USA HUMAN TSE PRION DISEASE DECEMBER 29 2012 CJD CASE LAB REPORT
MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...
Tuesday, November 6, 2012
Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and
Sporadic CJD, November-December 2012 update
Tuesday, June 26, 2012
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE
RISE IN NORTH AMERICA
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1
(August 19, 2011) including Texas
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein or just more Prionbaloney ?
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion
poker goes up again $
OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS
TRANSMISSIBLE IN BANK VOLES Nonno
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far but the
possibility that a small proportion of human cases so far classified as
"sporadic" CJD are of zoonotic origin could ***not be excluded. Moreover,
transmission experiments to non-human primates suggest that some TSE agents in
addition to Classical BSE prions in cattle (namely L-type Atypical BSE,
Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic
wasting disease (CWD) agents) might have zoonotic potential.
snip...
see follow-up here about North America BSE Mad Cow TSE prion risk factors,
and the ever emerging strains of Transmissible Spongiform Encephalopathy in many
species here in the USA, including humans ;
Thursday, August 12, 2010
Seven main threats for the future linked to prions
First threat
The TSE road map defining the evolution of European policy for protection
against prion diseases is based on a certain numbers of hypotheses some of which
may turn out to be erroneous. In particular, a form of BSE (called atypical
Bovine Spongiform Encephalopathy), recently identified by systematic testing in
aged cattle without clinical signs, may be the origin of classical BSE and thus
potentially constitute a reservoir, which may be impossible to eradicate if a
sporadic origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases
constitute an unforeseen first threat that could sharply modify the European
approach to prion diseases.
Second threat
snip...
TSS
