MAD COW DISEASE

MAD COW DISEASE/ BOVINE SPONGIFORM ENCEPHALOPATHY (BSE)

Bovine spongiform encephalopathy (BSE) is a progressive, fatal, infectious neurologic

disease of cattle that resembles scrapie of sheep and goats. The economic consequences of the
BSE epidemic have been substantial. Since effective control measures were put into place, the
incidence of BSE has decreased markedly.

ETIOLOGY

In the 1990s, there was intensive debate about the nature of the BSE pathogen. To date,
the most widely accepted hypothesis is that a proteinaceous infectious particle (prion) causes the
disease. Prions are misfolded conformational isoforms of host-encoded proteins that induce
protein misfolding, protein aggregation, and disease upon transmission to other organisms. This
hypothesis is supported by the agent’s apparent resistance to heat, freezing, ultraviolet light, and
chemical disinfectant procedures effective against bacteria and viruses. In addition, the
inoculation of purified prions in animals is able to transmit the disease, confirming that the agent
is only a protein.

Prions are also the cause of scrapie of sheep and goats; chronic wasting disease of deer;
transmissible mink encephalopathy; and kuru, Creutzfeldt-Jakob disease, fatal familial insomnia,
and similar disorders of humans. All of these conditions are also termed prion diseases, and the
related protein is the prion protein PrPSc (in which Sc stands for "scrapie ", the first described
prion disease).

PATHOGENESIS

Classical BSE develops as a result of foodborne exposure to prions via contaminated

animal-source proteins (meat and bone meal) in cattle rations. Calves born to infected cows are
at greater risk of acquiring BSE than are calves born to noninfected cows; however, this mode of
transmission is of minor importance relative to infections acquired through contaminated feed
sources. BSE is not transmitted horizontally by contact or aerosols. There is no sex or breed
predisposition.
Most cases of classical BSE are diagnosed in cattle 3–6 years old. The incubation period
after exposure is ~2–8 years, and animals as young as 22 months have been diagnosed with BSE.
Animals affected with atypical BSE are relatively old, and the incidence rates do not
follow the trends observed for classical BSE. Together, these findings led to the hypothesis that
atypical BSE results from spontaneous prion protein misfolding and is not related to the
ingestion of prion-contaminated feed. The mechanisms that induce the spontaneous prion
formation remain obscure.
CLINICAL SIGNS

Initial clinical signs of BSE are subtle and behavioral; signs progress over weeks to months to
include:
-hyperesthesia,
-nervousness,
-negotiating obstacles,
-reluctance to be milked,
-aggression toward either farm personnel or other animals,
-low head carriage,
-hypermetria,
-ataxia, and
-tremors.
-Weight loss and decreased milk production are common.

However, clinical signs may be nonspecific, and involvement of the nervous system is not
obvious in every case. Most animals reach a terminal state by 3 months after clinical onset.

DIAGNOSIS

Detection of the causative agent (PrPSc) in brain samples by:

-ELISA
-Western immunoblot techniques
-immunohistochemical techniques
-ultrasensitive in vitro amplification techniques

Clinical examinations do not provide a definitive diagnosis of BSE. In case of clinical suspicion
of the disease, the animal should be euthanized and the brain subjected to neuropathologic
examination.

TREATMENT, PREVENTION and CONTROL

-No effective treatment or vaccines are available
-Control measures: banning of meat and bone meal in cattle feeds, active and passive
surveillance, and culling sick animals

There is no effective treatment or vaccine for BSE. Euthanasia is advisable as soon as there is
some certainty of the clinical diagnosis, because animals become unmanageable as the disease
progresses, compromising their welfare and handler safety.

The most effective control measure is to prohibit the feeding of meat and bone meal to cattle.
Meat and bone meal supplements for cattle have been banned in many countries as a
consequence of the BSE epidemic
Public Health Consideration/ Zoonotic Risk

People can get a version of BSE called variant Creutzfeldt-Jakob disease (vCJD). As of
2019, 232 people worldwide are known to have become sick with vCJD, and unfortunately, they
all have died. It is thought they got the disease from eating food made from cows sick with BSE.
Also, research studies have shown that people cannot get BSE from drinking milk or eating dairy
products, even if the milk comes from a sick cow.

Initial signs of vCJD include behavioral changes and abnormal sensations. As the disease
progresses, incoordination and dementia develop, followed by coma and death. There is no cure
for vCJD. Most people die within a year after signs occur.

REFERENCE:

https://www.msdvetmanual.com/nervous-system/bovine-spongiform-encephalopathy/bovine-spo
ngiform-encephalopathy?fbclid=IwAR3wYYVuzui83h01g26KtqxPIapf7E1sq-ohPPtpYSWfvdP
zMvC-m3sKOTo

https://cacmap.fda.gov/animal-veterinary/animal-health-literacy/all-about-bse-mad-cow-disease?
fbclid=IwAR25igtgbsH2j2fGP8A4AqS8zxPbcf9VNRODpofNZeoWCtXujcKkw0a-Wts