In the world of rare and incapacitating illnesses, one disease stands out as especially menacing: Kuru. This devastating malady, also known as the “brain – eating disease,” has a notoriously fearsome reputation because of its near 100% fatality rate. Although it might seem like something straight out of a horror flick, Kuru is an incredibly rare prion disease that was once restricted to certain areas, like New Guinea.
Understanding Prion Diseases
Prion diseases, also referred to as transmissible spongiform encephalopathies (TSEs), are a group of scarce and invariably fatal disorders that affect both humans and animals. Unlike typical infectious diseases brought on by bacteria or viruses, prion diseases are caused by misfolded proteins known as prions.
These prions prompt abnormal folding in normal proteins, triggering a chain reaction of neurological deterioration. Prion diseases are marked by lengthy incubation periods that can span from months to several years, followed by a rapid decline in cognitive and physical capabilities.
Types of Prion Diseases
Aside from the infamous Kuru, there are five other well – known prion diseases:
- Creutzfeldt – Jakob Disease (CJD)
- variant Creutzfeldt – Jakob Disease (vCJD)
- fatal familial insomnia (FFI)
- Gerstmann – Sträussler – Scheinker syndrome (GSS)
- sporadic fatal insomnia (SFI)
Each of these diseases differs in symptoms, onset, and mode of transmission. Creutzfeldt – Jakob Disease (CJD) is the most common, making up at least 85% of all prion disease cases.