Creutzfeldt–Jakob disease

Examples of Creutzfeldt–Jakob disease in the following topics:

• Variant Creutzfeldt-Jakob Disease

  • Variant Creutzfeldt–Jakob Disease (vCJD) is a fatal neurological disorder which is caused by prions.
  • Creutzfeldt–Jakob disease, or CJD, is a degenerative neurological disorder (brain disease) that is incurable and invariably fatal.
  • However, given that BSE is believed to be the cause of variant Creutzfeldt–Jakob disease (vCJD) in humans, the two are often confused.
  • The prion that is believed to cause Creutzfeldt–Jakob exhibits at least two stable conformations.
  • The brown staining is for the prion protein responsible for variant Creutzfeldt–Jakob disease prion.

• Intracellular Pathogens

  • A pathogen or infectious agent is a microorganism such as a virus, bacterium, prion, or fungus that causes disease in its host.
  • Their theory was developed to explain the discovery that the mysterious infectious agent causing the diseases scrapie and Creutzfeldt–Jakob disease resisted ionizing radiation.
  • Fungi are the most common cause of diseases in crops and other plants.
  • Some eukaryotic organisms, such as protists and helminths, cause disease.
  • These abnormally-folded proteins are found characteristically in some diseases such as scrapie, bovine spongiform encephalopathy (mad cow disease), and Creutzfeldt–Jakob disease.

• Bovine Spongiform Encephalopathy

  • Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a fatal neurodegenerative disease in cattle that causes a spongy degeneration in the brain and spinal cord .
  • In humans, it is known as new variant Creutzfeldt–Jakob disease (vCJD or nvCJD), and by October 2009, had killed 166 people in the United Kingdom and 44 elsewhere.
  • The research shows, for the first time, that a 10-year-old cow from Alabama with an atypical form of bovine spongiform encephalopathy had the same type of prion protein gene mutation as found in human patients with the genetic form of Creutzfeldt–Jakob disease, also called genetic CJD, for short.
  • This form of Creutzfeldt-Jakob disease is called variant CJD.
  • The disease has progressed so far the animal cannot stand.

• Exceptions to Koch's Postulates

  • Even in Koch's time, it was recognized that infectious agents could be responsible for disease without fulfilling all of the postulates.
  • Even in Koch's time, it was recognized that some infectious agents were clearly responsible for disease, even though they did not fulfill all of the postulates.
  • Currently, a number of infectious agents are accepted as the cause of diseases despite their not fulfilling all of Koch's postulates.
  • Asymptomatic or subclinical infection carriers are now known to be a common feature of many infectious diseases, especially viruses such as polio, herpes simplex, HIV, and hepatitis C.
  • The second postulate may also be suspended for certain microorganisms or entities that cannot (at the present time) be grown in pure culture, such as prions responsible for Creutzfeldt–Jakob disease.

• Prions

  • Prions are responsible for the transmissible spongiform encephalopathies in a variety of mammals, including bovine spongiform encephalopathy (BSE, also known as "mad cow disease") in cattle and Creutzfeldt–Jakob disease (CJD) in humans.
  • All known prion diseases affect the structure of the brain or other neural tissue, are currently untreatable and universally fatal.
  • All known mammalian prion diseases are caused by the so-called prion protein, PrP.
  • Fungal prions do not appear to cause disease in their hosts.
  • But exponential growth of both PrPSc and of the quantity of infectious particles is observed during prion disease.

• Disease Development

  • It may be as short as minutes to as long as thirty years in the case of variant Creutzfeldt–Jakob disease.
  • Whichever is shorter depends on the disease.
  • Depending on the disease, the person may or may not be contagious during the incubation period.
  • Prodromes may be non-specific symptoms or, in a few instances, may clearly indicate a particular disease.
  • Outline the stages of disease: incubation, prodromal, acute and convalescence periods

• Protein Folding, Modification, and Targeting

  • Many of the disease-related mutations in collagen also cause defective folding.
  • A misfolded protein, known as prion, appears to be the agent of a number of rare degenerative brain diseases in mammals, like the mad cow disease.
  • Related diseases include kuru and Creutzfeldt-Jakob.
  • The diseases are sometimes referred to as spongiform encephalopathies, so named because the brain becomes riddled with holes.
  • Therefore, improved understanding of protein folding may lead to new therapies for cystic fibrosis, Creutzfeldt-Jakob, and many other diseases.

• Prions and Viroids

  • Fatal neurodegenerative diseases, such as kuru in humans and bovine spongiform encephalopathy (BSE) in cattle (commonly known as "mad cow disease"), were shown to be transmitted by prions.
  • The disease was spread by the consumption of meat, nervous tissue, or internal organs between members of the same species.
  • Later on in the outbreak, however, it was shown that a similar encephalopathy in humans known as variant Creutzfeldt-Jakob disease (CJD) could be acquired from eating beef from animals with BSE, sparking bans by various countries on the importation of British beef and causing considerable economic damage to the British beef industry.
  • Although a rare disease, individuals that acquire CJD are difficult to treat.
  • Human diseases caused by viroids have yet to be identified.

• Poliomyelitis

  • Roosevelt is perhaps the most famous polio sufferer, though the late onset of the disease and symptoms brings the diagnosis into question.
  • Poliomyelitis was first recognized as a distinct condition by Jakob Heine in 1840.
  • Although major polio epidemics were unknown before the late 19th century, polio was one of the most dreaded childhood diseases of the 20th century.
  • Polio epidemics have crippled thousands of people, mostly young children; the disease has caused paralysis and death for much of human history.
  • Enhanced vaccination efforts led by the World Health Organization, UNICEF, and Rotary International could result in global eradication of the disease.