A rare but dangerous disease that always leads to death is kuru. However, there is no need to worry because, interestingly, kuru is only found in one native tribe of Papua New Guinea.
What is Kuru?
The prion disease kuru is triggered by the ritual eating of the brains of deceased people. Since this ritual was only practiced by the Fore tribe of Papua New Guinea and is now banned, kuru deaths should be very rare these days. See AbbreviationFinder for abbreviations related to Kuru.
Kuru is the name for a brain disease that is one of the so-called transmissible spongiform, i.e. spongy, encephalopathies. Interestingly, the disease erupts only among a specific indigenous people of the Pacific island nation of Papua New Guinea, the Fore.
It is not known when the disease first appeared there, as the native tribe was not visited by scientists from Western civilization until the 1930s and Kuru was first studied more than 20 years later. By then, the disease was epidemic among the Fore, causing large numbers of deaths.
Infection with kuru is not symptomatic. Only with the onset of the disease can specific ailments be identified that suggest kuru. The disease was transmitted because the Fore tribesmen used certain rituals to eat the brains of other Fore, some of which were infected with kuru. However, since such practices were banned by law in Papua New Guinea, the number of cases of kuru has been steadily declining.
The disease manifests itself as a lack of coordination and a typical muscle tremor. Headaches and difficulty swallowing are also common, as is muscle atrophy and speech disorders. Among other things, these lead to an unnatural sounding laugh, which is why the disease is sometimes also referred to as laughing sickness.
Causes
Kuru is triggered by atypical proteins, so-called prions, which are the same pathogens as in the Creutzfeldt-Jakob disease known in Germany. It is assumed that the proteins change due to gene mutations or infections and thus become dangerous variants.
If a protein has changed atypically, it has the ability, among other deviant properties, to convert other proteins into faulty, dangerous forms as well. Thus, after a Fore ritually ate the brain of an infected victim, his body would eventually begin converting healthy protein into the diseased variety as well.
Kuru has a particularly long incubation period. This means that there is a long time between eating an infected brain and the onset of the disease, usually at least three years. So far, it is not exactly certain after how many years kuru can break out in an infected person.
Since there are still isolated new cases today, but the consumption of human brains in Papua New Guinea has long been banned, it is assumed that an outbreak is still possible after 40 or 50 years.
Symptoms, Ailments & Signs
Kuru can manifest itself through a variety of symptoms. The signs of the disease appear years after the infection and then lead to the death of the patient within a few weeks to months. Typical of the disease are coordination disorders and a noticeable tremor, which also occurs with chills. Gait disorders, muscle atrophy and paralysis of the limbs are often added to these symptoms, accompanied by chronic arm and leg pain.
Speech disorders and headaches can also occur. Swallowing disorders usually also develop, which make it difficult to eat and can thus cause weight loss. Strabismus can also develop. The disease can also cause psychological problems. Character changes and irritability, depressive moods and anxiety disorders are possible.
The symptoms of kuru usually have a very long incubation period. They often only appear years or decades after infection. The individual symptoms usually lead to life-threatening complications from which the patient ultimately dies. A severe course can be recognized by the fact that the physical and mental condition deteriorates rapidly. Within six to twelve months, paralysis, organ disorders and other complaints set in, which progress just as quickly and usually lead to death within a year.
Diagnosis & History
Kuru cannot be unequivocally diagnosed in affected individuals because the symptoms are also typical of many other possible diseases and there is no method by which the disease can be unequivocally proven.
For a reliable diagnosis of the disease, tissue must be removed from a patient’s brain in order to examine it for the changes typical of kuru. Therefore, the detection of kuru is only possible after the death of those affected. With such a tissue examination, the optical changes in the brain that are typical of kuru can be immediately detected under the microscope. This becomes increasingly porous and soon resembles a sponge, whose structure is also characterized by many fine holes.
The holes in the brain tissue are caused by a condition called astrogliosis, in which supporting cells in the brain blister, causing them to swell and eventually die. Further investigations can also show a decline in nerve connections or the complete death of nerve cells and the altered proteins.
The longer a patient has been ill with kuru, the more often other typical features such as the deposit of small fibers, so-called amyloids, are found near the blood vessels in the brain during an autopsy. As already mentioned, the disease only breaks out years later and then shows the first typical symptoms. The disease always leads to the death of the infected fore, usually within a year of the onset of the disease.
Complications
Kuru is a serious disease that usually leads to the death of the patentee. However, this disease is extremely rare and occurs in a very isolated area of the world. Those affected suffer from concentration disorders and gait disorders. There is also severe tremors and muscle wasting.
Patients suffer from permanent fatigue and exhaustion. The resilience of those affected also decreases significantly. Kuru also leads to speech disorders and difficulty swallowing. Due to the swallowing difficulties, the patient is usually no longer able to take the usual liquids and food, so that there are severe restrictions in the everyday life of the person concerned.
Headaches and body aches can also occur as a result of kuru and make everyday life even more difficult for those affected. Mental retardation and motor problems also occur. It is not possible to treat the disease. Only the symptoms can still be limited before death. In any case, the disease leads to the death of the patient. However, there are no other complications.
When should you go to the doctor?
If the typical Kuru symptoms occur after a trip to a risk area, you should go to your family doctor immediately. Although the disease is rare, it is always fatal if left untreated. If diagnosed early, the patient’s life can be prolonged with medication and other therapeutic measures. Therefore, unusual gait or coordination disorders, speech disorders, swallowing difficulties and headaches should be examined by a specialist. The trembling all over the body, which is characteristic of the disease, must also be clarified.
Muscle wasting and body aches indicate that the disease is already well advanced, which is why a hospital must be visited immediately with these symptoms. Signs of mental retardation also require a medical examination. If the cause is actually kuru, the patient must be treated in a specialist clinic. Accompanying this, therapeutic help should be turned on, since the disease always represents a considerable psychological burden for the patient and their relatives. The proper doctor to treat kuru is the internist, a neurologist, or a tropical disease specialist.
Treatment & Therapy
If a Fore is infected with kuru but has not yet contracted the disease, there are no symptoms to detect the disease, nor can it be medically proven. Therefore, there is no way to prevent, or at least delay, the onset of kuru through special treatment.
Even after Kuru’s outbreak, there is no way to cure the disease. Only some of the symptoms following the onset of kuru can be relieved with medication, but often only for a relatively short period of time. Ultimately, therefore, the disease always leads to death.
Outlook & Forecast
The prognosis for those affected by kuru is generally poor because it is a communicable disease similar to Kreutz-Jacob disease or mad cow disease. The prion disease kuru is triggered by the ritual eating of the brains of deceased people. Since this ritual was only practiced by the Fore tribe of Papua New Guinea and is now banned, kuru deaths should be very rare these days.
However, the long incubation period of up to 30 years is also problematic here. Due to this period of time, despite the ban on this ritual practice, it can still occasionally happen today that a person dies of kuru. Despite the long incubation period, the actual disease phase is short. As soon as the first kuru symptoms appear, the affected person dies within six months to a year. There is no treatment. At best, some of the symptoms can be alleviated with medication.
Kuru symptoms come on gradually. The gait or coordination disorders are initially not recognized for what they are. At the latest when the patient laughs unnaturally, the diagnosis is certain. However, the prion disease is only confirmed by an autopsy.
From a medical-historical perspective, it is interesting that later gene mutations occurred in the Fore tribe that prevented the outbreak of the disease. Researchers may be able to use this to develop a better prognosis for other degenerative prion diseases.
Prevention
When it was found that kuru was transmitted by eating infected brains, the number of new cases of kuru was drastically reduced by banning ritual brain eating. There are no other ways to prevent the disease. However, due to the long incubation period, despite the preventive measures, there are always new cases when the disease breaks out suddenly after a long time.
Aftercare
In most cases, those affected have very few or even no special aftercare options at Kuru. First and foremost, a doctor must be consulted early on so that further complications can be prevented. In the worst case, kuru can even kill the affected person, so the patient should consult a doctor at the first signs and symptoms of the disease.
In most cases, kuru cannot be completely cured, so the life expectancy of the affected person is significantly reduced by this disease. The onset and progression of the disease can be alleviated and delayed by taking various medications.
In any case, those affected must ensure that they take the medication regularly and continue to pay attention to the correct dosage. Most patients depend on the help of other people in their lives because of kuru. Psychological help can also be necessary, since kuru usually always leads to the death of the person concerned.
You can do that yourself
Since kuru is only found among a certain indigenous tribe in Papua New Guinea, the best self-help measure is not to visit that region. If contact with the members of the Fore tribe is avoided, there is usually no kuru that needs to be treated. Of course, cannibalistic rituals should also be avoided, as should other occasions when unfamiliar foods are eaten. Anyone who has visited the Fore Tribe or the region in question in Papua New Guinea in the past should consult a doctor to be sure.
Due to the very long incubation period of the brain disease, an outbreak can occur years after eating contaminated food. However, kuru is always fatal, which is why no effective treatment options from nature or the field of homeopathy are known. Although the symptoms can be alleviated by medicinal preparations, the course is always fatal. It is therefore important to avoid an infection from the outset and to consult a doctor immediately if you suspect it. Travelers to Papua New Guinea should avoid Fore tribe territories as far as possible and avoid physical contact with locals or refuse food offered.
