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Kuru: An Incurable Degenerative Neurological Disorder

Article · February 2013

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Journal of Pharmaceutical and Cosmetic Sciences Vol. 1(1) Pp. 13-14, 13 January, 2013
Available online at http://www.topclassglobaljournals.org

Submitted 17/09/2012 Accepted 05/12/2012

Short Communication

Kuru: An Incurable Degenerative Neurological Disorder

Khalid Hussain Janbaz1, M. Imran Qadir2, Muhammad Siddique2
1
Faculty of Pharmacy, Bahauddin Zakariya University, Multan, Pakistan
2
College of Pharmhacy, G.C. University, Faisalabad, Pakistan

Abstract

Kuru is a very dangerous disease that affects the brain and it is incurable. It is also a degenerative disorder
caused by the prion. W.T. Brown, an Australian scientist, was the first person that noticed the kuru disease
in the people of the Fore tribe of the Eastern Highlands, Province of Papua, New Guinea, in 1957-1959. It is a
very dangerous disease that can lead to ultimately death. It is 8-9 times more prevalent in women and
children than in men.

Key words: Kuru, laughing sickness, Prions

INTRODUCTION

Kuru is a very dangerous disease that affects the brain (Furuoka et al, 2002). It is incurable and also a degenerative
disorder caused by a prion (McKintosh et al, 2003). As a result of this disease, pathological bursts of laughter are
produced in the patient. This is why it is also known as laughing sickness
There are three main stages in the progression of symptoms of the disease. The first stage is called the ambulant
stage; it includes unsteadiness of stance, gait, voice, hands, and eyes; deterioration of speech, tremor, shivering, in-
coordination in lower extremities that moves slowly upward and dysarthria (slurring of speech). The second stage is
known as the sedentary stage. Symptoms of this stage are as follows: the patient can no longer walk without support
and there are more severe tremors and ataxia (loss of coordination of the muscles), shock-like muscle jerks, emotional
instability, outbursts of laughter, depression and mental slowing. (It is important to note that muscle degeneration has
not occurred in this stage, also tendon reflexes are usually still normal).
The third stage is the terminal stage, which is marked by the patient’s inability to sit up without support. At this stage,
there is increase in severe ataxia (loss of muscle coordination), tremor and dysarthria (slurring of speech), urinary and
faecal incontinence. There is usually difficulty in swallowing (dysphagia) and deep ulcerations appear. Cerebellar
dysfunction is the cause of these conditions (García-Alfonso et al, 2010).

HISTORY

W.T. Brown an Australin scientist was the first person that noticed the kuru disease in the people of the Fore tribe of the
Eastern highlands province of papua New Guinea in 1957-1959. Daniel Carleton Gajdusek and Micheal Alpers made a
great research on this disease. Micheal Alpers was a doctor in Australia. In the mid of 1960, a Fore girl named “Kigea”
was reported to have died because she was suffering from kuru disease. Dr. Alper took a sample of the affected brain
tissue when he was performing the post-martem of the said girl. It was reported that the collected sample was taken to
Gajdusek in USA and was transferred into two Chimpanzees.
After two years it was noticed that one of the chimpanzees named “Daisy” had developed the symptoms of kuru
disease. The successful transmission of the affected brain tissue into the chimpanzee demonstrated that this disease

Corresponding author's E-mail: mrimranqadir@hotmail.com

J. Pharm. Cosmet. Sci. 14
Figure 1: Structure of PrPSc
can be transferred from one specie into another. In 1976,
Gajdusek and Baruch S. Blumberg were awarded noble
prizes in physiology having been able to show that kuru is
transmissible to from a human to a chimpanzee (Liberski
et al, 2010).
TRANSMISSION
Early in the year 1961, the Australian doctor, Micheal
Alpers studied the Fore people very closely. He was
assisted by an Authropologist Shirley Lindenbaum. Their
research with the Fore people suggested that kuru might
have started from a single person who lived in a remote
part of the country and then spreads throughout the
whole population. It was reported that women and
children are more vulnerable to kuru disease than the
men due to their endocannibalistic funeral practices.
Women and children are more vulnerable because they
were usually given the duty of bathing corpses who may
have been suffering from cuts and sores.
CAUSES
Kuru is a dangerous disease caused by Prions (Mead et
al, 2009; Salamat et al, 2011). It is related to Creutzfeldt
Jakob disease. Kuru was said to have been spread as an
epidemic in the middle of the twentieth century. kuru is a
transmissible prion disease and it is been proven that the
origin of this disease was due to the consumption of an
individual with sporadic CJO, thus implying a common
pathophysiology (Sakudo et al, 2011). PrPC is the
normal, cellular prion protein, and it is converted into
prion PrPSc which is responsible for the symptoms of
Kuru. The structure of PrPSc is shown in figure 1 (Cohen
and Prusiner, 1998)
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IMMUNITY
As earlier mentioned, Kuru can lead to ultimately death. It
is characterized by truncal ataxia, preceded by
headache, joint pain and shaking of limbs. Kuru is also
bring about shivering throughout the body of a patient. An
English physician Simon Mead and his colleagues found
that the people who had the inheritant factor prion protein
G127V were still alive during the twentieth century
epidemic. (Siddique et al, 2010). So they concluded that
prion protein G127V enhances immunity to a very large
extent.
CONCLUSION
Kuru is an incurable degenerative neurological disorder
caused by prion. It has been very difficult over the years
to detect prion infection, track its transmission and type
the different strains.
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