Sylvie L. Benestad, Tran, B. Saure, R. Terland, S. Svendsen, K. Dessen, M. Haugum, M. Opheim, K. Handeland, K. Madslien, Ø. Kolbjørnsen, H. Wisløff, T. Moldal, J. Våge, T. Vikøren,, L. Pirisinu, G. Vaccari, R. Nonno
Norwegian Veterinary Institute
6th March 2018, Turin20 convegno degli istituti zooprofilattici sperimentali sulle EST
Chronic Wasting Disease:Chronic Wasting Disease:new experience in Europenew experience in Europe
239 tests 2008-2015
March 2016•Nordfjella mountain
Photo: Norwegian Institute for Nature Research (NINA)
First European CWD case
CWD diagnosed in North America (and South Korea)
2018
2007
Mule deer White tail
deerElk / "wapiti“
Moose Free ranging reindeer
IHC
WB TeSeE bio-Rad
ELISA TeSeE Bio-Rad
CWDclinical signs
Behavior changes
Emaciation
Excessive salivation, urination
Most of the time no signs, or unspecific signs
Terry Spraker, CSU, CO
� Imported from USA
- import life animals
- WTD to Finland 1935
- attractants/lures?
Where does it come from?
SpeciesSpecies Classical type Atypical type
SheepSheep 63 Classical, last 2009 165 Nor98, 13 in 2017
GoatGoat 1 Nor98 in 2005
CattleCattle 1 H-BSE in 2015
CatCat 1 FSE in 1994
ReindeerReindeer 18 CWD, first 2016
MooseMoose 3 atypical CWD, first 2016
Red deerRed deer 1 atypical CWD, in 2017
Norwegian animals TSE
Where does it come from?� Sheep with classical or atypical/Nor98 scrapie?
?
Spontaneous case
in a cervid?
Where does it come from?
?
How does CWD spread within cervids ?
Directly
Via environment
CWD in NorwayCervid species Diagnosed
Free ranging reindeer
18
Moose 3
Red deer 1
Totalt 22
18
2
1
13 CWD spots
Same CWD strain strain in the3 species?
200km
Characterization of a prion strainstrain in vitro
… different reactions to Proteinase K treatment:
Different conformations…
9A2 (98-100) F99F99 (220-225)
12B2 (88-92)
L42 (148-153)N-term C-term
Reindeer
C-termN-term
Moose
�WB: Norwegian moose prions have different characteristics from other CWD isolates
�It is not only host dependent: Norwegian moose was also different from Canadian moose
� New strain? Need for bio-assay
Bio-assay ongoing
Institutions Country Recipient Prion gene from
INRA Toulouse France Transgenic mice Sheep (3 lines), cattle and human
CEA Jouy-en-Josas France Transgenic mice Macaques
ISS Rome Italy Bank voles 2 genotypes
UCL London UK Transgenic mice Human
PRC Fort Collins USA Transgenic mice Cervids and elk
CWRU Cleveland USA Transgenic mice Human
ANSES Lyon France Transgenic mice Sheep
Roslin Edinburgh/Alberta University
UK/Canada
Conventional mice
Mice
Reindeer Moose/red deerAge Young, adult Old
ELISA Positive Positive
Distribution of PrPCWD
Lymph nodes and/or brain
In brain but not in lymph nodes
Distribution of PrPCWD in the brain
Spread, especially in the brain stem
Mostly in neurons, in the whole brain
Western blot Like CWD in North America
Low molecular weigth pattern. Truncated on the N-terminal.
2 populations of reindeer
Semi-domesticated
Wild
The sami people and their reindeer
… no administrative borders
Started 10th August 2017, completed February 2018
Nordfjella, relatively isolated area2000 reindeer / 25 000 total wild reindeer.
Disease absent
Increasing distribution, eradication unlikely
Small number, localized cases, eradication possible
Widespread and abondant distribution; long term management aimed at population suppression and protection
Diseaseabsent
Increasing distribution and abondance: eradication unlikely
Small number, localized cases: ERADICATION ….POSSIBLE
Ent
ry
2 phases:-Hunters giving heads-State culling
2449 reindeer killed and tested since primary case.18 positive cases
Depopulation of Nordfjella in 2017-2018
350mg obex
Primary test: ELISA TeSeE
Opp til total 500mg
+ 100-150mg LN
Obex
+Ln
Repeated ELISA TeSeE 350mg brainstem
bra
in
Obex
+Ln
Ln
250mg Ln
1
2
3
Species Dead Sex Age PrPCWD PrPCWD
obex LN
Rein 1 03.2016 Female 3-4 y POS POS
Rein 2 08.2016 Male 7 y POS POS
Rein 3 09.2016 Female 4 y POS POS
Rein 4 06.2017 Male 4 y not detected POS
Rein 5 08.2017 Female 3 y not detected POS
Rein 6 08.2017 Male 8 y not detected POS
Rein 7 10.2017 Male 2,5 y POS POS
Rein 8 11.2017 Male 2,5 y POS POS
Rein 9 11.2017 Male Adult POS POS
Rein 10 11.2017 Male 5 y POS POS
Rein 11 12.2017 Female Adult not detected POS
Rein 12 12.2017 Female Adult POS POS
Rein 13 01.2018 Male 1,5 y* not detected POS
Rein 14 01.2018 Male Adult not detected POS
Rein 15 02.2018 Male Adult not detected POS
Rein 16 02.2018 Male Adult POS POS
Rein 17 02.2018 Male Adult POS POS
Rein 18 02.2018 Female Adult not detected POS
Moose 1 05.2016 Female 13 y POS not detected
Moose 2 05.2016 Female 14 y POS not available
Moose 3 10.2017 Female 13 y POS not detected
Red deer 10.2017 Female 16 y POS not detected
� CWD: new European disease
� Most contagious of the prion diseases (within cervid population)
� New atypical CWD type in Norwegian moose, Nor16CWD
� Where is the epicenter? What is the situation in Europe?
Take home messages
� Measures to eradicate/ limit spread completed, unpredictable issue (environment)
� Wildlife population, management challenges
� Overlapping cervids populations, both wild and semi domesticated
� Need for sensitive test on live animals (RAMALT) /environment (Rt-QuIC)
� Genetic studies
Thanks to all the Norwegian colleagues:�Veterinary Institute: Pathology technicians�Norwegian Institute for Nature Research�Ministry of Environment�Norwegian Food Safety Authority�The hunters
And thanks to international colleagues:
�Olivier Andréoletti, INRA Toulouse, Fr�Emmanuel Comoy, CEA Jouy-en-Josas, Fr�Romolo Nonno, ISS Rome, It�John Collinge, UCL London, UK�Glenn Telling, Terry Spraker, PRC Fort Collins, Colorado, USA�Qingzhong Kong, CWRU Cleveland, USA�Thierry Baron, ANSES, Lyon, Fr�Vincent Beringue, INRA, Jouy-en-Josas, Fr�Wilfred Goldmann, Roslin Institute, Edinburgh, UK
Thank you for your attention!
https://www.vetinst.no/sykdom-og-
agens/chronic-wasting-disease
http://apps.vetinst.no/skrantesykestatistikk/N
O/
