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Kuru (disease)
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Kuru (disease)
Kuru is a rare, incurable, and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. It is a prion disease which leads to tremors and loss of coordination from neurodegeneration. The term kúru means "trembling" and comes from the Fore word kuria or guria ("to shake"). It is also known as "laughing sickness" due to abnormal bursts of laughter which occur.
It was spread among the Fore people via funerary cannibalism. Deceased family members were traditionally cooked and eaten, which was thought to help free the spirit of the dead. Women and children usually ate the brain, where infectious prions were most concentrated, and therefore were more commonly affected.
The outbreak likely started when a villager developed sporadic Creutzfeldt–Jakob disease and died. When villagers ate the brain, they contracted the disease and then spread it to other villagers who ate their infected brains.
While the Fore people stopped eating human meat in the early 1960s, when this was first speculated as the cause, the disease lingered due to kuru's long incubation period of anywhere from 10 to over 50 years. Cases finally declined after half a century, from 200 deaths per year in 1957 to no deaths from at least 2010 onward, with the last known death in 2005 or 2009.
Kuru, a transmissible spongiform encephalopathy, is a disease of the nervous system that causes physiological and neurological effects which ultimately lead to death. It is characterized by progressive cerebellar ataxia, or loss of coordination and control over muscle movements.
The preclinical or asymptomatic phase, also called the incubation period, averages 10–13 years, but can be as short as five and has been estimated to last as long as 50 years or more after initial exposure.
The clinical stage, which begins at the first onset of symptoms, lasts an average of 12 months. The clinical progression of kuru is divided into three specific stages: the ambulant, sedentary and terminal stages. While there is some variation in these stages from individual to individual, they are highly conserved among the affected population. Before the onset of clinical symptoms, an individual can also present with prodromal symptoms including headache and joint pain in the legs.
In the ambulant stage, the infected individual may exhibit unsteady stance and gait, decreased muscle control, difficulty pronouncing words (dysarthria), and tremors (titubation). This stage is named the ambulant because the individual is still able to walk around despite symptoms.
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Kuru (disease)
Kuru is a rare, incurable, and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. It is a prion disease which leads to tremors and loss of coordination from neurodegeneration. The term kúru means "trembling" and comes from the Fore word kuria or guria ("to shake"). It is also known as "laughing sickness" due to abnormal bursts of laughter which occur.
It was spread among the Fore people via funerary cannibalism. Deceased family members were traditionally cooked and eaten, which was thought to help free the spirit of the dead. Women and children usually ate the brain, where infectious prions were most concentrated, and therefore were more commonly affected.
The outbreak likely started when a villager developed sporadic Creutzfeldt–Jakob disease and died. When villagers ate the brain, they contracted the disease and then spread it to other villagers who ate their infected brains.
While the Fore people stopped eating human meat in the early 1960s, when this was first speculated as the cause, the disease lingered due to kuru's long incubation period of anywhere from 10 to over 50 years. Cases finally declined after half a century, from 200 deaths per year in 1957 to no deaths from at least 2010 onward, with the last known death in 2005 or 2009.
Kuru, a transmissible spongiform encephalopathy, is a disease of the nervous system that causes physiological and neurological effects which ultimately lead to death. It is characterized by progressive cerebellar ataxia, or loss of coordination and control over muscle movements.
The preclinical or asymptomatic phase, also called the incubation period, averages 10–13 years, but can be as short as five and has been estimated to last as long as 50 years or more after initial exposure.
The clinical stage, which begins at the first onset of symptoms, lasts an average of 12 months. The clinical progression of kuru is divided into three specific stages: the ambulant, sedentary and terminal stages. While there is some variation in these stages from individual to individual, they are highly conserved among the affected population. Before the onset of clinical symptoms, an individual can also present with prodromal symptoms including headache and joint pain in the legs.
In the ambulant stage, the infected individual may exhibit unsteady stance and gait, decreased muscle control, difficulty pronouncing words (dysarthria), and tremors (titubation). This stage is named the ambulant because the individual is still able to walk around despite symptoms.
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