transmissible spongiform encephalopathy
Let an agent explain transmissible spongiform encephalopathies and prions in general terms from public health and veterinary sources, describe human and animal forms, relay surveillance and control measures, and route personal medical questions to clinicians.
Research draft, second pass
A second pass drafted this model: the structure a model of this thing needs, and what is known about it in the world. The line under this one says how the second half was obtained - researched against sources, or recalled without web access, in which case nothing here was read anywhere and every claim is a lead to verify. Unreviewed either way.
written by Claude from model knowledge without web access - no source was read, every claim is a lead to verify
Researched by: Claude
Purpose and description
Let an agent explain transmissible spongiform encephalopathies and prions in general terms from public health and veterinary sources, describe human and animal forms, relay surveillance and control measures, and route personal medical questions to clinicians.
A group of fatal neurodegenerative diseases of humans and animals caused by prions, misfolded proteins that induce further misfolding, producing sponge-like brain damage, including Creutzfeldt-Jakob disease in sporadic, inherited, iatrogenic and variant forms, kuru, fatal familial insomnia, scrapie in sheep including atypical scrapie, bovine spongiform encephalopathy, chronic wasting disease in deer, transmissible mink encephalopathy and spongiform encephalopathies of exotic ungulates and camels; there is no cure, and control relies on surveillance and feed and blood safety rules.
What it is for: Not applicable; a group of diseases.
It can be explain prions and disease forms; relay human and animal diseases; relay surveillance and control; route personal questions.
Distinguishing features
Prion cause
Long incubation
Invariably fatal
Resistant infectious agent
What it looks like
Not a visible object; spongiform brain changes seen at post-mortem.
Physical character
sporadic CJD incidence: about 1-2 per million per year
variant CJD cases worldwide: about 230 count - cumulative
How it is recognised
Prion diseases with spongiform brain damage
CJD, kuru, scrapie, BSE, CWD and related diseases
Other dementias are not transmissible; viral encephalitis has a different cause
Related models
is a kind of - in registry terms
is a kind of - in registry terms
is caused by - the misfolded protein
is controlled by - and surveillance in animals
In practice
Families and kinds
human prion diseases: sporadic, inherited, iatrogenic and variant CJD, kuru, fatal familial insomnia, GSS
scrapie and atypical scrapie
bovine spongiform encephalopathy
chronic wasting disease
transmissible mink encephalopathy
exotic ungulate and camel spongiform encephalopathies
feline spongiform encephalopathy
Identifiers
ICD-11 8E00 prion diseases
WOAH listed diseases BSE and scrapie
Standards and regulation
WHO and national CJD surveillance
Animal feed bans and BSE testing rules
Blood and tissue donor deferral rules
Decontamination standards for surgical instruments
Failure modes and hazards
Iatrogenic transmission
Food chain contamination
Chronic wasting disease spread in wildlife
Agents giving personal medical advice
Also called
Where this came from
wikidata · CC0 1.0
Drafted structure
Bundle to layer to finding to question, as the second pass will find it: 4 bundles · 8 layers · 8 findings · 16 questions.
Understand The diseases.
Science.
Prions
Prions and mechanism.
Prions
Prions.
- What are prions, and how do they cause spongiform encephalopathies? definition
- Is the question about a prion disease or another dementia or encephalitis? boundary
Forms
Human and animal forms.
Forms
Forms.
- What are the human and animal forms, and how do they differ? definition
- Which entry fits the specific disease? action
Clinical Diagnosis and care.
Clinical.
Diagnosis
Diagnosis.
Diagnosis
Diagnosis.
- How are prion diseases diagnosed, and what are the signs, in general terms? provenance
- Is the user asking about a personal or family case, which needs a neurologist? boundary
Care
Care and genetics.
Care
Care.
- What care and genetic counselling exist, given there is no cure? provenance
- Which references are standard? provenance
Control Public health and animal control.
Regulation.
Measures
Control measures.
Measures
Measures.
- What feed, food, blood and surgical measures control transmission? provenance
- Which entry fits BSE control? action
Surveillance
Surveillance.
Surveillance
Surveillance.
- How are human and animal prion diseases monitored, with sources? provenance
- Which sources are cited? provenance
Context History and research.
Context.
History
History.
History
History.
- How were kuru, scrapie and BSE understood, and how did Prusiner establish the prion concept? provenance
- Which entry fits the BSE crisis? action
Research
Research.
Research
Research.
- What research exists on treatments, diagnostics and prion-like mechanisms in other diseases, with findings attributed? provenance
- Which entry fits prion research? action
What the second pass must settle
- Should CJD and BSE be separate primary entries?
- How should surveillance sources be linked?
- The registry entry has merged aliases naming distinct diseases; should they be split off?