Kuru is a very rare, fatal neurodegenerative disorder caused by an infectious protein (prion) found in contaminated human brain tissue. It was primarily found among the Fore people of Papua New Guinea, who practiced a form of ritualistic cannibalism where they consumed the brains of deceased relatives.
Understanding Kuru
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Cause: Kuru is caused by a prion, a misfolded protein that can trigger normal proteins in the brain to misfold as well. This leads to brain damage and the characteristic symptoms of the disease.
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Transmission: The disease was transmitted through the consumption of infected brain tissue during funeral rituals. Women and children were primarily affected because they were the main participants in these rituals.
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Symptoms: Symptoms of kuru include:
- Difficulty walking (ataxia)
- Loss of coordination
- Difficulty swallowing
- Tremors and shaking (kuru means "to shake" in the Fore language)
- Slurred speech
- Mood changes and behavioral disturbances
- Dementia
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Progression: The disease progresses rapidly and is always fatal.
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Decline: Since the cessation of cannibalistic practices in the late 1950s, kuru has become extremely rare.
Prion Diseases
Kuru belongs to a family of diseases known as transmissible spongiform encephalopathies (TSEs), or prion diseases. Other prion diseases include:
- Creutzfeldt-Jakob disease (CJD)
- Variant Creutzfeldt-Jakob disease (vCJD) – often associated with bovine spongiform encephalopathy (BSE), or "mad cow disease."
- Gerstmann-Sträussler-Scheinker syndrome (GSS)
- Fatal familial insomnia (FFI)
These diseases are all characterized by the accumulation of prions in the brain, leading to progressive neurological damage.
Current Status
Thanks to the cessation of cannibalistic practices, kuru has largely disappeared. However, its study has provided valuable insights into prion diseases and the mechanisms of neurodegeneration.
