Kuru is a rare and debilitating neurological disease that primarily affects people in Eastern Highlands Province of Papua New Guinea. The disease is commonly referred to as the ‘disease of laughing death’ due to the characteristic bouts of spontaneous, uncontrollable laughter and changes in behavior displayed by patients.
Kuru is caused by an infectious prion protein that, when ingested, infects and accumulates in the brain tissue, leading to the development of neurological symptoms. The most common cause of transmission is through the consumption of infected human brain tissue, particularly during mortuary rituals and the consumption of dead relatives.
Symptoms of kuru usually appear 10 to 15 years after infection, with the first signs being tremors and muscle weakness affecting the limbs. There may also be difficulty swallowing, slurred speech, and loss of coordination. As the disease progresses, involuntary movements of the limbs and head become more common, along with difficulty walking and eventually leading to confinement to bed.
The incidence of kuru has declined very sharply since the cessation of cannibalistic practices but researchers are still studying the disease in order to understand how it operates. Kuru was first discovered by Australian researchers, and the disease was studied extensively to understand its transmission and pathology. This research was instrumental in the discovery of other prion diseases, such as Creutzfeldt–Jakob disease and bovine spongiform encephalopathy (BSE).
In conclusion, kuru is a rare and devastating disease that was previously endemic to parts of Papua New Guinea. The disease is caused by an infectious prion protein that primarily affects the brain, leading to neurological symptoms such as tremors and muscle weakness. While its incidence has sharply declined since cannibalistic practices have ended, continued research remains essential in understanding how the disease operates and how other prion diseases arise.
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