Transmissible spongiform encephalopathy
| Classification according to ICD-10 | |
|---|---|
| G81.9 | Atypical viral infection of the central nervous system, unspecified |
| ICD-10 online (WHO version 2019) | |
Transmissible spongiform encephalopathy (TSE) (German: "Transmissible spongiform brain disease") or transmissible spongiform encephalopathy , also known as prion disease , is the name for a number of brain diseases ( encephalopathy ) in which there is a spongy change in the brain tissue. Diseases are found in both humans and animals. Prions are assumed to be the cause (hence the name prion disease ). TSEs are among the protein misfolding diseases .
TSEs are always fatal; so far there are no therapeutic options.
Forms of TSE
- The oldest known TSE is scrapie , a spongy brain disease observed in sheep and goats. It was discovered in England in 1732.
- Bovine spongiform encephalopathy (BSE): in cattle, first detected in England at the end of 1984
- Chronic Wasting Disease (CWD): in elk and other North American deer
- Exotic Ungulate Encephalopathy (EUE): in nyalas and greater kudu (African antelope species)
- Feline Spongiform Encephalopathy (FAE): in cats , diagnosed since 1990
- Transmissible Mink Encephalopathy (TME): in mink , first appeared in 1947 on a mink farm in the USA
The following animal species can also be affected by TSE: mules , elk , deer, oryx, chamois, rats, guinea pigs, hamsters, mice (in laboratory tests).
So far, four TSEs are known in humans:
- the Creutzfeldt-Jakob disease (CJD)
- the Gerstmann-Sträussler-Scheinker syndrome (GSSS)
- Kuru
- the fatal familial insomnia (Engl. Fatal Familial Insomnia , FFI)
etiology
The triggers of the diseases are most likely prions . Some TSEs are known and proven to be inherited. For the acquired form, a transmission of the prions in the sense of an infectious agent is assumed.
pathology
There are no signs of an inflammatory or immune response in the patient's brain. Characteristic features are thread-like, protein-containing deposits in the nerve tissue and the sponge-like perforated structure of the brain.
Reporting requirement
Transmissible spongiform encephalopathies are in Austria in accordance with § 1 para. 1, point 1 Epidemics Act 1950 on suspicion, illness and death notifiable . Doctors and laboratories, among others, are obliged to report this ( Section 3 Epidemics Act).
In Germany, human spongiform encephalopathy (except for familial hereditary forms) is subject to notification by name in the event of suspicion, illness and death by the doctor etc. in accordance with Section 6 of the Infection Protection Act (IfSG) . The group of reporting persons is based on § 8 IfSG, what has to be reported according to § 9 IfSG.
Social relevance
In the years 2000 to 2005 reports on TSE appeared on the front pages of newspapers and in the main news on television in Germany and other countries. In Europe, awareness of TSE led to a medium-term change in beef consumption and to a (temporary) significant drop in prices, in the course of which many companies had to change their production at short notice. The legislature of many states and the European Union issued rules which animal products should make "TSE-safe" (e.g. BSE regulation in Germany). These extend not only to meat products for nutrition and as animal feed, but also, for example, to gelatine as a capsule material in medicines and even leather straps for orthopedic purposes in medical products . In Germany, prion research was initiated and funded in 2001.
In 2008 there were nationwide mass riots in South Korea in connection with the easing of meat imports from the USA, which also led to political consequences.
Individual evidence
- ↑ Alphabetical directory for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 719
- ↑ BSE panic: Bundeswehr destroys canned meat worth millions. In: Spiegel Online . January 14, 2001, accessed January 6, 2017 .
- ^ Prion research and TSE coordination office ( Memento of July 3, 2011 in the Internet Archive ).
- ^ BSE panic in South Korea. In: taz.de . June 11, 2008, accessed January 6, 2017 .
literature
- Jean-Philippe Brandel: Transmissible spongiform encephalopathies. Orphanet Encyclopedia, December 2004
