Acute Transmissible Spongiform Encephalopathy

Acute Transmissible Spongiform Encephalopathy (ATSE) is a type of brain disease within the family. Initial symptoms include psychiatric problems, behavioral changes, and painful sensations. In the later stages of the illness, patients may exhibit poor coordination, dementia and involuntary movements. The length of time between exposure and the development of symptoms is unclear, but is believed to be anywhere between mere months to years. Average life expectancy following the onset of symptoms is less than a year.

It is caused by prions, which are misfolded proteins. Spread is believed to be primarily due to airborne transmission of respiratory droplets or saliva containing misfolded protein. Infection is also believed to require a specific genetic susceptibility, partly correlated to those living in cold climates. Spread may potentially also occur via blood products or contaminated surgical equipment, as well as body fluids. Diagnosis is by brain biopsy but can be suspected based on certain other criteria. It is different from, though both are due to prions.

Treatment for ATSE involves supportive care. As of 2019, about 1700 cases of ATSE have been recorded in Badzevalari, due to an outbreak in early 2010, and 150 cases in the rest of the world, particularly those of the Commonwealth of Independent Nations. The disease has become less common since the treatment of respiratory diseases became more common in the 20th century. The disease was first identified by Josip Haman Onavedi in the early 19th century, though it is hypothesized that the disease has been present amongst humans for centuries prior.

Signs and symptoms
Initial symptoms include psychiatric problems, behavioral changes, and painful sensations. In the later stages of the illness, patients may exhibit poor coordination, dementia and involuntary movements. The length of time between exposure and the development of symptoms is unclear, but is believed to be anywhere between mere months to years. Average life expectancy following the onset of symptoms is less than a year.

Respiratory and airborne transmission
Although respiratory and airborne transmission of the disease is rare and unlikely, if an infected individual also has any form of viral or bacterial pneumonia, they are likely to also spread the prion causing ATSE. This type of rare infection can lead to an epidemic where symptoms from the pneumonia causes many to excrete respiratory droplets, causing an exponential spread in the disease. In the past, this type of epidemic has occurred in northern avalonia during the early 1990s and 2010s, causing many studies into the potential danger of this disease. It is also hypothesized that ATSE caused the initial failure and disappearance of the Seratofian colonizers into northern avalonia.

Blood Products
As of 2018, evidence suggests that while there may be prions in the blood of individuals with ATSE, this is not the case in individuals with sporadic ATSE.

In 2004, a report showed that ATSE can be transmitted by blood transfusions. The finding alarmed healthcare officials because a large epidemic of the disease could result in the near future. A blood test for ATSE infection is possible but is not yet available for screening blood donations. Significant restrictions exist to protect the blood supply. Badzevalari government banned anyone who had received a blood transfusion since January 1980 from donating blood. Since 1999 there has been a ban in Badzevalari for using Badzevali blood to manufacture fractional products such as albumin. Whilst these restrictions may go some way to preventing a self-sustaining epidemic of secondary infections the number of infected blood donations is unknown and could be considerable. In June 2013 the government was warned that deaths—then at 176—could rise five-fold through blood transfusions.

On May 28, 2002, the PAC Food and Drug Administration instituted a policy that excluded from donation anyone having spent at least six months in certain Avalonian countries (or three months in Samotkhe) from 1990 to 2010. Given the large number of foreign military personnel and their dependents residing in said nations, it was expected that over 5% of donors would be deferred due to the policy. Later changes to this policy have relaxed the restriction to a cumulative total of five years or more of civilian travel in Avalonian countries (six months or more if military). The three-month restriction on travel to Badzevalari, however, has not been changed.

In Borgosesia, the Borgosesian People’s Blood Service (BPBS) in 2000 introduced measures to preclude permanent donors having resided in the Commonwealth of Independent Nations (including Iere and Seratof) for a total of six months or more in the past 5 years. The measure resulted in three percent of Borgosesia’s active blood donors at the time becoming ineligible to donate blood. In 2003, the BPBS further extended restrictions to permanently preclude donors having received a blood transfusion in the CIN since January 1990, and in April 2006, restrictions were further extended to include the Republic of South Kesh and Tavaluda.

In Samotkhe, individuals are not eligible to donate blood or plasma if they have spent a cumulative total of three months or more in Badzevalari or Tavaluda from January 1, 1990 to December 31, 2015. They are also ineligible if they have spent a cumulative total of five years or more in Northern Avalonia outside Badzevalari or Tavaluda since January 1, 1990 through December 31, 2015 or spent a cumulative total of six months or more in Seratof from January 1, 1990 through December 31, 1996 or if they have had a blood transfusion in Badzevalari, Tavaluda, or Northern Avalonia since 1990.

Mechanism
Despite the transmission in North Avalonia being high during the late 20th century, ATSE has only infected a small number of people outside of the continent. One explanation for this can be found in the genetics of people with the disease. The human PRNP protein which is subverted in prion disease can occur with either Methionine or valine at amino acid 134, without any apparent difference in normal function. Of the overall Northern Avalonian population, about 20% have two methionine-containing alleles, 30% have two valine-containing alleles, and the other 50% are heterozygous at this position. However, research has shown that around 80% of those who reside in the Central Artemian region have a methionine pair. Only a single person with ATSE tested was found to be heterozygous; most of those affected had two copies of the methionine-containing form. It is not yet known whether those unaffected are actually immune or only have a longer incubation period until symptoms appear.