Temporal and Spatial Trends of Prion diseases in

India

Dr.Sudam Chandra Sahoo Division of Veterinary Public Health Bhoj R Singh

Division of Epidemiology

IVRI, Izatnagar-243 122, 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.….

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Mechanism of mis-folding of prion Protein

Prion disease or transmissible spongiform encephalopathy (TSE) are 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

Definition of Prion disease

SCRAPIE in sheep and goat Bovine spongiform encephalopathy (BSE) IN

cattle Transmissible mink encephalopathy Chronic wasting disease (deer)

PRION DISEASES IN ANIMAL

CREUTZFELDT-JAKOB DISEASE (CJD) KURU Variant CJD Fatal familial insomnia Gerstman-Straussler Scheinker syndrome

(GSS)

PRION DISEASES IN HUMAN

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1. Scrapie in Pargna district of West Bengal (Garole sheep) [Choudhary S., Gupta N., Lethra G. and Gour D. S., 2015]

2. Scrapie in Kamach sheep from Himalayan (Choudhary et. al., 2015)

3. Scrapie in Kasuli Himanchala Pradesh(1996)4. vCJD in Mumbai and Bengaluru (IMAHANS 20

definite case of human between 1971-1990)5. vCJD in Karnatak and Kerala (1991)

Reports of prion diseases in India

Map presenting Reported cases in different states of IndiaSporadic occurrence of diseases

vCJD

vCJD

Scrapie

Scrapie

vCJD

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1961-1970 1971-1980 1981-1990 1991-20000

2

4

6

8

10

12

14

Occurrences of prion disease in different years in India

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KARNA(J&K) MANDHYA MALPURA GAROLE(WB)0123456789

Genetic susceptibility different indigenous sheep breeds of India

official IVRI Website

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 cases are found.

Centre for Animal Diseases Research and Diagnosis (CADARD) IVRI in 2000

Consumption of beef and beef product. Lateral transmission may possible by taking

bone meal and blood meal of infected animals. Both vertical and horizontal transmission may

possible. Placenta foetal membrane amniotic fluid may

contaminated the pasture and pen and surrounding (Pattison et al 1972).

Dissemination of prion by ewes to their off springs by suckling colostrum and milk (Konold and Maddision et. al., 1972).

TRANSMISSION IN ANIMALS

American regulation mandate 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.

LEGISLATION RELATED TO PRION DISEASE

Maneka Sanjay Gandhi/tribuneindia.com

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 by sheep brain can cause BSE in cattle or CJD in human.

According to OIE The spread of BSE from slaughter houses and rendering plants in some countries like CHINA, INDIA, JAPAN, PAKISTAN, and TAIWAN.

http://www.shea-online.org/Prion.pdf

HIGH RISK MATERIAL ARE TO 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.

http://www.shea-online.org/Prion.pdf

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 121oC for 30 min.

Lab surface should be sterilized by 1:5/1:10 of hypochlorite solution for 5 min contact time

PROCESES TO DISINFECT THE PRION CONTAMINATED MATERIALS

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.