Creutzfeldt-Jakob disease (Mad Cow Disease): Symptoms, causes, diagnosis and treatment

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Creutzfeldt-Jakob disease (Mad Cow Disease)
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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
  Creutzfeldt-Jakob 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.

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Causes of Creutzfeldt-Jakob 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. The mad cow disease which
  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
  disease can 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.

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Causes of Creutzfeldt-Jakob disease
Continue

• 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.

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Symptoms

• 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 degradation make 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.

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Diagnosis of Creutzfeldt-Jakob 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 no definitive diagnostic tests that 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.

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Treatments

• 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
  humans is a relatively new disease and
  researchers are trying to gain more insight into
  the cause and ways to prevent the disease.

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