Posted by Dr Abhimanyu Kaura, PGYIII (Medicine), Monmouth Medical Center
Kuru disease is one of the five human spongioform encephalopathies caused by prions. This group also includes Creutzfeldt-Jakob disease (CJD), variant CJD, Gerstmann-Straussler Scheinker syndrome and fatal familial insomnia.
Bovine spongiform encephalopathy also known as “mad cow disease”, is another prion disease that affects cattle, and was responsible for bringing more attention to all of these disorders in the 1990s.
Kuru is confined the Fore tribes of Papua, New Guinea.
In the 1950s there were 2100 cases of Kuru in these tribes leading to about 1000 deaths per year.
As per the culture of the Fore tribe, if a person in a family dies his meat is eaten by his family members, especially the wife and children, as a mark of respect to him. 90% of people affected by the disease were women and children.
Between 1996 to 2004 only 11 new cases of Kuru were identified in the region. Currently with the ban of cannibalism in the Farah tribes the disease had become virtually non existent.
Kuru first presents with tremors, then unsteady gait and progresses to leg weakness, ataxia, incoherent speech, sporadic laughter finally. In later stages, affected patients become demented, bed bound and unable to swallow.
Death occurs most commonly from respiratory distress and pneumonia or infection of pressure sores. The disease is fatal within 1-2 years of the onset of symptoms and had no known cure.
Here’s a video introduction
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The causative organism is a prion, a mutated protein which replicates itself like a virus, and is spread by eating the neuronal tissue of infected people.
The precise incubation period of the disease is unknown, but can be up to fifty years.
The pathological hallmark is the presence spongioform encephalopathy with of Prion reactive plaques mostly in the cerebellum:
The detailed studies of theses cases of Kuru has helped us understand other prion diseases such as variant CJD, sporadic CJD whose clinical features and course is similar to this condition.
And maybe another link between Zombies and neurology?
Neurology Update 