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Prion diseases in India
https://www.slideshare.net/singh_br1762/prion-diseases-in-india-spatial-and-temporal-distribution-
55539613
Dr. Bhoj R singh, Principal Scientist (VM)
Head Division of Epidemiology
Indian Veterinary Research Institute, Izatnagar-243122,
Bareilly, UP, India.
 Stanley Prusiner coined the word “prion”
for a proteinaceous infectious particle
 PROTEINACEOUS + INFECTIOUS
PRION
 BSE expert RICHARD LACCY state
THAT..
“BSE IS the times bomb of the twentieth
century equivalent to the bubonic plague “
 It is the time to put a special attention on
BSE.….
www.arccjournals.com /
indianjournals.com
 Prion disease or transmissible spongiform
encephalopathy (TSE) are a group of
neurodegenerative disorder which is
characterised by accumulation of the prion
form of the mammalian prion protein (PrPSC)
in central and peripheral nervous system in
both animals and humans population.
 Prion disease are unique in that they can be
inherited, can occur sporadically, or be
infective
 SCRAPIE in sheep and goat.
 Bovine spongiform encephalopathy (BSE) IN
cattle.
 Transmissible mink encephalopathy.
 Chronic wasting disease (deer).
 CREUTZFELDT-JAKOB DISEASE (CJD)
 KURU
 Variant CJD
 Fatal familial insomnia
 Gerstman-Straussler Scheinker syndrome
(GSS)
1. Scrapie in Pargana district of West Bengal
(Garole sheep) [Choudhary S., Gupta N.,
Lethra G. and Gour D. S., 2015]
2. Scrapie in Kamach sheep from Himalayan
region (Choudhary et. al., 2015)
3. Scrapie in Kasauli Himachal Pradesh (1996)
4. vCJD in Mumbai and Bengaluru (IMAHANS 20
definite case of human between 1971-1990)
5. vCJD in Karnatak and Kerala (1991)
Map presenting
Reported cases in
different states of India
Sporadic occurrence of
diseasesvCJD
vCJD
Scrapie
Scrapie
vCJD
4
13
7
10
0
2
4
6
8
10
12
14
1961-1970 1971-1980 1981-1990 1991-2000
2
3
8 8
0
2
4
6
8
10
KARNA(J&K) MANDHYA MALPURA GAROLE(WB)
www.ias.ac.in/jgenet/Vol94No1
 BSE SURVEILLANC … An initiative of IVRI
 A total of 350 suspected brain samples were
collected from Cross bred cattle (10),
buffaloes (315), sheep (21), and Goat (4)
 Not a single confirmed case is found.
official IVRI Website
 Consumption of beef and beef product.
 Lateral transmission is possible by taking bone
meal and blood meal of infected animals.
 Both vertical and horizontal transmission are
possible.
 Placenta, foetal membrane, amniotic fluid may
contaminate the pasture and pen and
surrounding environment (Pattison et al 1972).
 Dissemination of prion by ewes to their suckling
offspring through colostrums and milk (Konold
and Maddision et. al., 1972).
 American regulation mandated that brain
and other nerve tissue be removed from
cattle after they are slaughtered.
 Where as in India no such rule till now.
 As India rank 2nd (next to Brazil) in exporting
beef putting millions of people at risk of mad
cow disease.
 Indian slaughter houses have no laboratory
facilities to diagnose BSE.
 Even some slaughter houses have no
veterinarian for A/M and P/M examination.
 Administration claims that mad cow disease
does not exist in India but----
 People are in millions in India having brain
deterioration, loss of memory and motor
functions and are usually diagnosed as senile or
victims of Alzheimer’s disease.
 Question is, Does there is regular testing for
Prion diseases at least in people with senile
disease? How many cows and buffaloes are
tested for BSE of similar disease in proportion to
total population it is insignificant number?
 Epidemiologically human forms of prion disease
can be classified as familial sporadic and
iatrogenic.
 Human to human transmission via cadaver
derived therapeutics or tissues.
 Corneal transplantation pituitary growth
hormone injection and Dura matter grafting
(Yamada et al 2009).
 RABIES vaccine made from sheep brain can cause
BSE in cattle or CJD in human.
 According to OIE
The spread of BSE from slaughter houses and
rendering plants may be important in some
countries like CHINA, INDIA, JAPAN, PAKISTAN, and
TAIWAN.
HIGH RISK MATERIAL ARE TO Be
TREATED AS BIOHAZARD 4
 High risk material are Brain (including
Dura mater), spinal cord, posterior eye,
pituitary tissue.
 Low risk material are Cerebrospinal fluid,
liver, lymph node, kidney, lung, spleen,
placenta, olfactory epithelium.
 No risk materials are blood milk saliva etc.
 All surgical and steel surfaced instruments to
be sterilised by autoclaving at 134oC for 18
minute.
 Material should be dipped in 1N sodium
hypochlorite and then autoclaved at 121oC for
30 min.
 Lab surface should be sterilized by 1:5/1:10 of
hypochlorite solution for 5 min contact time
 Blood contaminated matter should be
sterilised by 1:10,1:100 (5.25%-6.15%)
sodium hypochlorite solution.
 Mucous membranes disinfected through
irrigation with saline water for several
minutes.
 Contaminated wound must be rinsed with
0.5% sodium hypochlorite.
 Always discard single use devices
wrapped with polythene as biohazard 4
label material.
 1. Name the diseases spreading through
consumptions of animal, fish and poultry
products in ruminants.
 2. How to control Prion diseases?
 3. Legislations in India on feeding animal, fish
and poultry products in ruminants.
 4. Name the diseases which may be similar to
Prion Diseases in ruminants but associated
with known etiology.
 5. Zoonotic importance of Prion Diseases.

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Prion diseases in India

  • 1. Prion diseases in India https://www.slideshare.net/singh_br1762/prion-diseases-in-india-spatial-and-temporal-distribution- 55539613 Dr. Bhoj R singh, Principal Scientist (VM) Head Division of Epidemiology Indian Veterinary Research Institute, Izatnagar-243122, Bareilly, UP, India.
  • 2.  Stanley Prusiner coined the word “prion” for a proteinaceous infectious particle  PROTEINACEOUS + INFECTIOUS PRION
  • 3.  BSE expert RICHARD LACCY state THAT.. “BSE IS the times bomb of the twentieth century equivalent to the bubonic plague “  It is the time to put a special attention on BSE.….
  • 5.  Prion disease or transmissible spongiform encephalopathy (TSE) are a group of neurodegenerative disorder which is characterised by accumulation of the prion form of the mammalian prion protein (PrPSC) in central and peripheral nervous system in both animals and humans population.  Prion disease are unique in that they can be inherited, can occur sporadically, or be infective
  • 6.  SCRAPIE in sheep and goat.  Bovine spongiform encephalopathy (BSE) IN cattle.  Transmissible mink encephalopathy.  Chronic wasting disease (deer).
  • 7.  CREUTZFELDT-JAKOB DISEASE (CJD)  KURU  Variant CJD  Fatal familial insomnia  Gerstman-Straussler Scheinker syndrome (GSS)
  • 8. 1. Scrapie in Pargana district of West Bengal (Garole sheep) [Choudhary S., Gupta N., Lethra G. and Gour D. S., 2015] 2. Scrapie in Kamach sheep from Himalayan region (Choudhary et. al., 2015) 3. Scrapie in Kasauli Himachal Pradesh (1996) 4. vCJD in Mumbai and Bengaluru (IMAHANS 20 definite case of human between 1971-1990) 5. vCJD in Karnatak and Kerala (1991)
  • 9. Map presenting Reported cases in different states of India Sporadic occurrence of diseasesvCJD vCJD Scrapie Scrapie vCJD
  • 11. 2 3 8 8 0 2 4 6 8 10 KARNA(J&K) MANDHYA MALPURA GAROLE(WB) www.ias.ac.in/jgenet/Vol94No1
  • 12.  BSE SURVEILLANC … An initiative of IVRI  A total of 350 suspected brain samples were collected from Cross bred cattle (10), buffaloes (315), sheep (21), and Goat (4)  Not a single confirmed case is found. official IVRI Website
  • 13.  Consumption of beef and beef product.  Lateral transmission is possible by taking bone meal and blood meal of infected animals.  Both vertical and horizontal transmission are possible.  Placenta, foetal membrane, amniotic fluid may contaminate the pasture and pen and surrounding environment (Pattison et al 1972).  Dissemination of prion by ewes to their suckling offspring through colostrums and milk (Konold and Maddision et. al., 1972).
  • 14.  American regulation mandated that brain and other nerve tissue be removed from cattle after they are slaughtered.  Where as in India no such rule till now.  As India rank 2nd (next to Brazil) in exporting beef putting millions of people at risk of mad cow disease.  Indian slaughter houses have no laboratory facilities to diagnose BSE.  Even some slaughter houses have no veterinarian for A/M and P/M examination.
  • 15.  Administration claims that mad cow disease does not exist in India but----  People are in millions in India having brain deterioration, loss of memory and motor functions and are usually diagnosed as senile or victims of Alzheimer’s disease.  Question is, Does there is regular testing for Prion diseases at least in people with senile disease? How many cows and buffaloes are tested for BSE of similar disease in proportion to total population it is insignificant number?
  • 16.  Epidemiologically human forms of prion disease can be classified as familial sporadic and iatrogenic.  Human to human transmission via cadaver derived therapeutics or tissues.  Corneal transplantation pituitary growth hormone injection and Dura matter grafting (Yamada et al 2009).  RABIES vaccine made from sheep brain can cause BSE in cattle or CJD in human.  According to OIE The spread of BSE from slaughter houses and rendering plants may be important in some countries like CHINA, INDIA, JAPAN, PAKISTAN, and TAIWAN.
  • 17. HIGH RISK MATERIAL ARE TO Be TREATED AS BIOHAZARD 4  High risk material are Brain (including Dura mater), spinal cord, posterior eye, pituitary tissue.  Low risk material are Cerebrospinal fluid, liver, lymph node, kidney, lung, spleen, placenta, olfactory epithelium.  No risk materials are blood milk saliva etc.
  • 18.  All surgical and steel surfaced instruments to be sterilised by autoclaving at 134oC for 18 minute.  Material should be dipped in 1N sodium hypochlorite and then autoclaved at 121oC for 30 min.  Lab surface should be sterilized by 1:5/1:10 of hypochlorite solution for 5 min contact time
  • 19.  Blood contaminated matter should be sterilised by 1:10,1:100 (5.25%-6.15%) sodium hypochlorite solution.  Mucous membranes disinfected through irrigation with saline water for several minutes.  Contaminated wound must be rinsed with 0.5% sodium hypochlorite.  Always discard single use devices wrapped with polythene as biohazard 4 label material.
  • 20.  1. Name the diseases spreading through consumptions of animal, fish and poultry products in ruminants.  2. How to control Prion diseases?  3. Legislations in India on feeding animal, fish and poultry products in ruminants.  4. Name the diseases which may be similar to Prion Diseases in ruminants but associated with known etiology.  5. Zoonotic importance of Prion Diseases.