Creutzfeldt-Jakob disease (Mad Cow Disease)

Introduction of Creutzfeldt-Jakob disease (Mad Cow

Disease)

Mad cow disease is a neurological disease in which certain abnormal proteins

known as prions induce the formation of lesions/plaques in the brain tissue of the
infected animal. This disease is as old as 300 years, when it was reported in sheep
as 'scrapie'. Other than cows and sheep, this disease can infect cats, deer, and elk
as well. However, the bad news is that the infection is not restricted only to
animal species. Mad cow disease, in humans, is known as the variant CreutzfeldtJakob disease (vCJD), or the new variant Creutzfeldt-Jakob disease (nvCJD), and
there is strong evidence that it is contracted by eating beef that came from
infected cows.

Causes of Creutzfeldt-Jakob disease (Mad Cow Disease)

The classic Creutzfeldt-Jakob disease was described in the 1920s by German
neurologist Hans Gerhard Creutzfeldt and Alfons Maria Jakob, giving the disease
its name. Although it is often called the mad cow disease in human beings, this
is a wrong association. Themad cow diseasewhich is also known as the
bovine spongiform encephalopathy (BSE), CJD, vCJD, and nvCJD are all types of
transmissible spongiform encephalopathy (TSE), which is characterized by
spongy appearance of the brain tissue of the infected organisms.
Creutzfeldt-Jakob diseasecan occur in three different ways. In over 80% of
the cases, it occurs sporadically for no apparent reason. Almost 5% to 10% of
the cases of CJD are hereditary, that are passed on as a result of gene mutation.
A small percentage of classic CJD is contracted through contamination. Although
some researchers dispute the role of the prion, till date, the prion theory is the
most widely accepted cause of the TSE. However, greater characterization of
this protein is required.
The disease in humans is believed to have been caused by consumption of
infected cattle beef, carrying the agent of BSE. Besides the co-relation between
the consumption of infected beef products, the fact that healthy cows when fed
with tissue infected with BSE agent (prion) also contracted the disease has lent
further ground to the theory of the disease being transmitted to human beings
through infected bovine tissue.

Symptoms of Creutzfeldt-Jakob disease (Mad Cow Disease)

Although both are forms of TSE, the effects of mad cow disease are different
from those of classic CJD. In case of classic CJD,dementia and neurological
degradationmake an early appearance whereas, the effects of mad cow
disease start with psychiatric or behavioural problems like depression and
anxiety. As the disease progresses, neurological signs like unsteadiness,
difficulty in walking, involuntary, jerky movements appear. Towards the end,
an individual loses control over his mental and physical functions completely
and dies. BSE has longer illness duration of 13 - 14 months as opposed to 4 - 5
months in the case of classic CJD. The average age of death in case of classic
CJD is 68 years, whereas in case of BSE, it is only 28 years.

Diagnosing of Creutzfeldt-Jakob disease (Mad Cow Disease)

Only a brain biopsy, which can be done after the death of an infected individual,
can confirm BSE. Unless the clinical symptoms have surfaced, there are
nodefinitive diagnostic teststhat can confirm mad cow disease in human
beings. However, magnetic resonance scans, tonsillar biopsy, and cerebrospinal
fluid tests are some diagnostic tests that healthcare professionals rely on, in case
the disease has been suspected in individuals.

Treatments of Creutzfeldt-Jakob disease (Mad Cow Disease)

There is no cure for this disease. Doctors concentrate on reducing pain

and alleviating the symptoms. For preventive measures, WHO recommends
strict regulation of meat and meat products from animals that show signs
of TSE. The international body also suggests that pharmaceutical industries
should avoid making vaccines using materials from animals that can be
infected with prions.
Be it the CJD or the mad cow disease, the problem is its unusually long
incubation period that makes it difficult to trace the source from which the
disease was contracted.Mad cow disease in humansis a relatively new
disease and researchers are trying to gain more insight into the cause and
ways to prevent the disease.