Kuru (You get it from eating brains!)

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

Watch the full story here:

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?

Rabies – The curse behind Vampires, Werewolves and Zombies?

Rabies is a viral encephalitis transmitted to humans by the bite of an infected animal, usually a dog, fox or bat.

The virus travels slowly along  peripheral nerves from the bite to the brain, and it can be several months between the animal bite and onset of the encephalitis.

Once encephalitis sets in, the disease is almost invariably fatal without immunization or post-exposure prophylaxis.

Symptoms include headache, fever, confusion and agitation, paranoia, terror, hallucinations, and delirium.  There is increased salivation, but attempts to drink or swallow lead to  excruciatingly painful spasms of the muscles in the throat and larynx leading to “hydrophobia” (fear of water).

The increased salivation, combined with unwillingness to swallow, leads to profuse drooling of saliva infected with virus.  The encephalitis leads to increased aggressiveness, unprovoked attack and biting, and thus facilitates the spread of the virus.

Although the only confirmed cases human to human transmission of rabies have been recipients of infected donor organs, folklore has suggested transmission by sex, nursing and biting, inspiring stories about vampires, werewolves, vampires and zombies.

The similarities between rabies and werewolves needs no further explanation.

In 1998, Juan Gomez-Alonso a Spanish neurologist wrote a paper in Neurology comparing vampirism with human rabies.

The most marked similarity are caused by rabid spasms of the head and throat.

This leads to clenched teeth with retracted lips like and animal, and inability to swallow saliva with frothing at the mouth and vomiting of bloody fluid.

And the same goes for Zombies:

These creatures first made fashionable in the 1954 book I am Legend, and then featured in the more recent movies 28 Days Later and World War Z, are even said to be caused by “infections” transmitted when a human is bitten by a demented zombie.

Click here to find out more similarities between Rabies, Vampires, Werewolves and Zombies.