A rare but dangerous disease that always leads to death is kuru. Nevertheless, there is no need to worry because, interestingly, kuru occurs only in a native tribe from Papua New Guinea.
What is kuru?
Kuru is the name given to a brain disease that is one of the so-called transmissible spongiform, or spongiform, encephalopathies. Interestingly, the disease breaks out only among a particular native people of the island nation of Papua New Guinea in the Pacific Ocean, the Fore. It is unknown when the disease first appeared there, as the native tribe was not visited by scientists from Western civilization until the 1930s, and Kuru was not first studied until more than 20 years later. Until then, the disease occurred epidemically among the Fore and caused a large number of deaths. Infection with Kuru does not manifest symptomatically. Only with the onset of the disease can specific ailments be identified that suggest kuru. The disease was transmitted because Fore tribal members, in certain rituals, ate the brains of other Fore, some of whom were infected with kuru. However, since such practices were prohibited by law in Papua New Guinea, the number of cases of kuru has steadily declined. The disease is manifested by incoordination and a typical muscle tremor. In addition, there are often headaches and difficulty swallowing, but also muscle atrophy and speech disorders. Among other things, these lead to unnatural-sounding laughter, which is why the disease is sometimes referred to as laughing disease.
Causes
Kuru is caused by atypical proteins called prions, which are the same pathogens used in Creutzfeldt-Jakob disease, a disease known in Germany. It is thought that the proteins change due to genetic mutations or infections, thus becoming the dangerous variants. When a protein has changed atypically, it has, among other deviating properties, the ability to transform other proteins into equally defective, dangerous forms. Therefore, after a Fore ate the brain of an infected victim in the course of a ritual, his body eventually also began to convert healthy proteins into the diseased variant. 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 has long been banned in Papua New Guinea, it is assumed that an outbreak is still possible after 40 or 50 years.
Symptoms, complaints, and signs
Kuru can manifest itself through a number of symptoms. Signs of the disease appear years after infection and then lead to death of the infected person within a few weeks to months. Typical signs of the disease are incoordination and a noticeable tremor, which also occurs with chills. These signs of the disease are often joined by gait disturbances, muscle atrophy and paralysis of the limbs, accompanied by chronic arm and leg pain. Speech disorders and headaches may also develop. In most cases, swallowing disorders also develop, which make it difficult to eat and can cause weight loss. Furthermore, strabismus may develop. The disease can also cause psychological complaints. Possible symptoms are changes of character and irritability, depressive moods and anxiety disorders. The symptoms of Kuru usually have a very long incubation period. They often do not appear until years or decades after infection. The individual symptoms usually lead to life-threatening complications from which the patient eventually 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 dysfunction, and other symptoms set in, progressing just as rapidly and usually leading to death within a year.
Diagnosis and progression
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 proven beyond doubt.To determine the disease with certainty, tissue must be removed from a patient’s brain to examine it for the changes typical of kuru. Therefore, the detection of Kuru is only possible after the death of the affected person. During such a tissue examination, the visual changes in the brain typical of Kuru can be seen immediately under the microscope. The brain becomes increasingly holey and soon resembles a sponge, whose structure is also characterized by many fine holes. The holes in the brain tissue are caused by so-called astrogliosis, in which supporting cells of the brain form blisters, causing them to swell and eventually die. Further examinations can also show a decline in nerve connections or even the complete death of nerve cells, as well as the altered proteins. The longer a patient with Kuru has been ill, the more frequently a postmortem examination also reveals other typical features, such as the deposition of small fibers, so-called amyloids, near the blood vessels in the brain. As already mentioned, the disease breaks out only after years 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 always leads to the death of the patient. However, this disease occurs extremely rarely and in a very isolated area of the world. The affected persons suffer from disturbances of concentration and also gait disturbances. Furthermore, there is also a strong tremor and muscle atrophy. Patients suffer from permanent fatigue and exhaustion. The resilience of those affected also decreases significantly. Kuru also leads to speech disorders and swallowing difficulties. As a result of the swallowing difficulties, it is usually no longer possible for the patient to take in fluids and food in the normal way, so that there are severe restrictions in the daily life of the affected person. Headaches and pain in the limbs can also occur as a result of kuru, further complicating the affected person’s daily life. Furthermore, mental retardation and motor complaints occur. It is not possible to treat the disease. Only the symptoms can be limited before death. However, the disease leads to the death of the patient in any case. However, no other complications occur.
When should one go to the doctor?
If the typical kuru symptoms appear after a trip to a risk area, one should immediately go to the family doctor. Although the disease occurs rarely, it is always fatal if left untreated. If diagnosed early, the patient’s life can be prolonged by medication and other therapeutic measures. Therefore, unusual gait or coordination problems, speech disorders, swallowing difficulties and headaches should definitely be examined by a specialist. The trembling over the entire body that is characteristic of the disease must also be clarified. Muscle atrophy and pain in the limbs indicate that the disease is already far 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 indeed kuru, the patient must be treated in a special clinic. This should be accompanied by therapeutic help, as the disease always places a considerable psychological burden on the sufferer and their relatives. The right doctor to treat kuru is the internist, a neurologist or a specialist in tropical diseases.
Treatment and therapy
If a Fore is infected with kuru but the disease has not yet broken out, the disease cannot be noticed by symptoms and cannot be detected medically. Therefore, there is no way to prevent or at least delay the outbreak of Kuru by specific treatment. Even after the outbreak of Kuru, there is no way to cure the disease. Only some of the symptoms after the outbreak of Kuru can be alleviated with medication, but often only for a relatively short period of time. Ultimately, therefore, the disease always leads to death.
Outlook and prognosis
For those affected by kuru, the prognosis 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 in Papua New Guinea and has since been banned, there should actually be hardly any deaths from kuru nowadays. However, the long incubation period of up to 30 years is also problematic here. Due to this time span, it can still occasionally happen that a person dies from kuru, despite the prohibition of this ritual practice. Despite the long incubation period, however, the actual disease phase is short. As soon as the first Kuru symptoms appear, the affected person is dead within half a year to a year. There is no treatment. At best, some of the symptoms can be alleviated with medication. The onset of Kuru symptoms is gradual. At first, the gait or coordination problems are not recognized for what they are. At the latest when the patients emit an unnatural laugh, the diagnosis is certain. However, the prion disease is only confirmed by an autopsy. From the point of view of medical history, it is interesting that genetic mutations later occurred in the Fore strain that prevented the onset of the disease. It is possible that research can use this to develop a better prognosis for other degenerative prion diseases.
Prevention
When it was discovered that kuru is transmitted by eating infected brains, the number of new cases of kuru was drastically minimized by banning ritual brain consumption. There are no other ways to prevent the disease. However, due to the long incubation period, new cases occur every now and then despite preventive measures when the disease suddenly breaks out after a long time.
Aftercare
Affected persons have very few or even no special options for aftercare in most cases of kuru. In this case, the first priority is to see a doctor early on so that further compplications can be prevented. In the worst case, Kuru can even lead to the death of the affected person, so the patient should see 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 progress of the disease can be alleviated and delayed by taking various medications. In this case, the affected persons must in any case pay attention to a regular intake and furthermore also to a correct dosage of the drugs. Most patients are dependent on the help of other people in their lives due to Kuru. In this case, psychological help may also be necessary, since Kuru usually always leads to the death of the affected person.
This is what you can do yourself
Since kuru only occurs in a particular native tribe in Papua New Guinea, the best self-help measure is to avoid visiting the region in question. If contact with members of the Fore tribe is avoided, there is usually no kuru to treat. Cannibalistic rituals should also be avoided, of course, as should other occasions when unfamiliar foods are consumed. Persons who have visited the Fore tribe or the region in question in Papua New Guinea at some time in the past should consult a doctor to be on the safe side. Because of the very long incubation period of the brain disease, an outbreak can occur years after contaminated food has been eaten. However, kuru is always fatal, which is why there are no known effective treatments from nature or the field of homeopathy. The symptoms can be alleviated by medical preparations, but the course is always fatal. Therefore, it is important to avoid infection in the first place and to consult a doctor immediately in case of suspicion. Travelers to Papua New Guinea should avoid the areas of the Fore tribe as far as possible and should not make physical contact with the locals or refuse any food offered.
