Epidemiology

In the beginning, mad cow disease (BSE) was experienced by cows but in its
development this disease was transmitted to humans (zoonosis). The first case of this disease
was found on the European continent, namely in England in 1986 with the number of cases
continuing to grow until it reached 170,000 cases identified. The disease continues to spread
to other countries with identified sporadic occurrences in Ireland, Switzerland, Portugal, Italy,
France and other European countries. Mad cow disease then not only spread to the European
continent but eventually spread to other continents in the world (Wiwik, 2018).

Data from the World Health Organization reports that in 2017, the highest number of
cases of mad cow disease are still found in the UK, followed by France, Spain, Ireland and
America and also found in countries such as Italy, the Netherlands, Canada and Portugal. In
addition, countries on the Asian continent are also exposed to mad cow disease, namely in
Japan, Taiwan and Saudi Arabia (Manik et al., 2019).

Based on data obtained from the CDC, in England the incidence of epizootic disease
of cows peaked in 1993 with almost 1000 new cases per week. This figure fortunately
decreased sharply in subsequent years where annual cases in 1995 were 14,562 cases, in
2000, 2005, 2010 and 2015 cases continued to decline with the number of cases each being
1,443 cases, 225 cases, 1 case, and 2 cases. This sharp decline occurred due to the role of
food ban policies and other control measures aimed at eliminating the recycling of BSE
agents and preventing contamination of food and feed with BSE agents so that their spread is
reduced (Kumagai et al., 2019). From this data, accumulated until the end of 2015, the
number of confirmed cases of mad cow disease in the UK reached more than 184,500 from
more than 35,000 cattle. This figure is the highest figure in the world, showing how severe
the incidence of mad cow disease is in England which is also the first country to find this
disease (CDC, 2021). Based on the Decree of the Minister of Agriculture Number
367/KPTs/TN 530/12/2002 Indonesia was declared mad cow disease free. Based on data
from the World Health Organization, there have been no reports of cases of mad cow disease
in Indonesia as well as in Indonesia's neighboring countries, namely Malaysia and Brunei
Darussalam. This achievement was obtained due to Indonesia's policy of prohibiting imports
of meat and animal products which are usually used as raw materials for making instant food
such as sausages and corned beef from countries affected by BSE such as America and
European countries.
 North America is one of the areas affected by BSE. In 2018, BSE surveillance data in
North America reported 26 cases with a distribution of 6 cases occurring in the United States
and 20 cases occurring in Canada. There were 26 cases of BSE identified in North America,
consisting of 7 cases of atypical BSE and 19 cases of classic BSE. The only cases of classic
BSE identified in the United States were imported from Canada (CDC, 2021).

As mentioned above, BSE is divided into two, namely classical and atypical BSE.
People can become infected with classic BSE when they eat certain foodstuffs such as the
brains and spines of animals infected with BSE. The main route for the spread of classic BSE
is through contaminated animal feed. Classic BSE can cause variant Creutzfeldt-Jakob
disease in humans through consumption of contaminated animal meat. The first case
diagnosed as classic BSE occurred in 1986 in England then other cases were one after
another confirmed in other countries. In contrast to classic BSE, in atypical BSE, the cases
that occur are not related to animal feed contamination. This infected animal occurs suddenly
but occurs generally when it has reached the age of 8 years or more. This atypical BSE is
associated with spontaneous changes that occur in prion proteins in adult cattle. This is
something that is still difficult to understand. Therefore, until now scientists are still
researching whether there is a genetic and environmental relationship to the occurrence of
atypical BSE. (Kumagai et al., 2019).

Etiology

BSE is a disease that attacks the central nervous system. This disease is characterized
by the accumulation of an abnormal form of disease-associated prion protein (PrPSc) that
resides in the central nervous system (CNS). PrPSc has generally been described as a
pathological agent of TSEs and may be a posttranslationally modified form of normal cellular
prion protein (PrPC). Based on Stanley Prusiner's research many years ago by identifying
TSEs agents, prions were identified as proteins that have the ability to reproduce without the
need for the role of nucleic acids. Prions are divided into disease-causing prions and normal
native prion proteins. In normal native protein conditions, prions are proteins that can protect
cells from oxidative damage, play a role in regulating the circadian cycle and sleep, and play
a role in creating memory in humans.

Disease-causing prion proteins are proteins that are resistant to heat, radiation and
sterilization measures that aim to kill them. This protein is also highly resistant to pH,
refrigeration and freezing. Bovine spongiform encephalopathy, like other prion diseases, can
be transmitted not only by ingestion, but also by iatrogenic tissue transfer from subclinical
carriers. In cattle infected with BSE prions, the infectious prions are mostly confined to the
central nervous system. However, in patients with vCJD, infectious prions are detectable in
multiple tissues throughout the body, posing a risk of iatrogenic transmission, even through
routine surgical procedures, if adequate prion decontamination procedures are not followed.

The disease-causing prion and native prion protein have identical amino acid
sequences but differ in their folding patterns. Exogenous prion isoforms modify the original
prion protein to replicate disease-causing prion isoforms, these replicating prions then
transform other normal native prions into disease-causing prions continuously or in chains.
These prion pools then spread the infection and prion deposits are usually found in the brains
of infected animals (Costassa et al., 2016).

For many years cattle with BSE disease were identified as infected by 1 strain of BSE
agent, but an analysis identified 3 strains of BSE agent consisting of 1 classic BSE strain and
2 atypical strains, namely the H-type strain and the L-type strain. Based on studies conducted
on mice where mice carry the human prion gene, the results revealed that L-type BSE is
capable of causing disease in humans. Meanwhile, classic BSE agents have been identified as
being able to infect humans through consumption of contaminated meat.

Costassa, E. V., Iulini, B., Mazza, M., Acutis, P., Maurella, C., Meloni, D., Pautasso, A.,
Capucci, L., Bozzetta, E., Simmons, M. M., Zanusso, G., Pocchiari, M., Corona, C., &
Casalone, C. (2016). Pathogenesis and Transmission of Classical and Atypical BSE in
Cattle. 130–134. https://doi.org/10.14252/foodsafetyfscj.2016018

Kumagai, S., Daikai, T., & Onodera, T. (2019). Bovine Spongiform Encephalopathy – A
Review from the Perspective of Food Safety. Food Safety, 7(2), 21–47.
https://doi.org/10.14252/foodsafetyfscj.2018009

Manik, R., Panjaitan, Z., StudiSistemInformasi, P., & Triguna Dharma, S. (2019). Sistem
Pakar Mendiagnosa Penyakit Mad Cow Disease (Sapi Gila) Menggunakan Metode
Certainty Factor. 1–10.

CDC. (2021). About BSE BSE (Bovine Spongiform Encephalopathy). Centers for Disease
Control and Prevention. https://www.cdc.gov/prions/bse/about.html
Wiwik, H. W. (2018). Penyakit Ternak yang Perlu Diwaspadai Terkait Keamanan Pangan.
Cakrawala, 12(2), 208–221.

CDC. (2021). BSE in North America BSE (Bovine Spongiform Encephalopathy) | Prion
Diseases | CDC. Www.cdc.gov. https://www.cdc.gov/prions/bse/bse-north-
america.html
Houston, F., & Andréoletti, O. (2019). Animal prion diseases: the risks to human health.
Brain Pathology, 29(2), 248–262. https://doi.org/10.1111/bpa.12696