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BBC – The Future – Kefafa: The Mystery of "East African Nervous Disease" t



For the people of Wapogoro there are somewhat worse fates than kifafa .

The tribe lives in the Mahenge Mountains in the deepest Tanzania: a land of intersected mountains, misty forests and rare underground riches. from fossils to brilliant neon pink gems. They have survived in isolation for hundreds of years, quietly dealing with their lives, and largely avoiding the wider world.

Or that was until a Norwegian doctor appeared in 1959. Louise Gilek-Aal was just 28 years old, out of a medical school and determined to create a clinic in the area. But she had not seen patients before she noticed something unusual.

Almost every day, the children of two years old appeared in her office with severe burns. In one case, a little girl was totally unrecognizable; many others died of their wounds. Burns were often embossed with spider webs of pale pink scars that had been accumulated after years of previous burns.

When Jilek-Aall tried to understand what was happening, she met with fear and concealment. Even the relatives of the affected children refused to explain the sinister trend

Indeed, the youngest members of the tribe looked particularly, some would say suspiciously, prone to a wide range of accidents, including regular drowning. When Jilek-Aall tried to understand what was going on, she met with fear and concealment. Even the relatives of the affected children have categorically refused to explain their sinister tendency.

Eventually Jilek-Aall realized that her patients had seizures they called kifafa ̵

1; Swahili for the "Little Death" – and falling down. Sometimes they would land in the open hearths where they prepared their food. Sometimes they would end up in the river.

But the mystery is not over there. The same children also tended to slow growth, intellectual discomfort and typical manners, such as inappropriate gait and grim facial expressions. Most astonishingly, there was the "head"; several times a day they would close their eyes and repeatedly throw their heads in their chest. The disease usually lasted for several years and ends with an early death. Finally, although every Wapogoro member was afraid of kifafa to an almost feverish degree, the disease had not actually been heard. in Tanzania. This is true even for the neighboring tribes.

Nipple syndrome is found in pockets all over East Africa, from South Sudan to northern Uganda, where he has devastated the lives of thousands of people. In every new place it seems to appear from nothing. What can be hidden behind this deadly plague? And how can we stop it?

Even today, nobody knows what causes nodular syndrome. In the past, the disease has been seen as a rare and mysterious form of epilepsy. But now the latest research has suggested that this may be a neurodegenerative disease that occurs as a combination of epilepsy and some symptoms of Parkinson's and Alzheimer's. After all, his victims usually suffer from complications related to seizures, such as brain damage and accidents, or poor mental health and neglect.

The study trail emerged from a study in 2018, which found that the brains of teenagers with illness look remarkably similar to those of adult patients with the last two diseases. "This creates a new line of research because we now know there is some kind of brain damage," says Michael Polanen, a University of Toronto pathologist who runs the study. "You can think of this in the following way: if you can understand the type of brain damage you may be able to work back to the cause."

At least overlapping with other diseases adds an additional sense of urgency to the study. Not only does the nodular syndrome affect some of the most vulnerable people on the planet but it can hold the key to understanding a few disorders that have long since fled scientists.

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Compare nodding syndrome with Alzheimer's disease, it's easy to see why. While one was found in millions of people, with a very different lifestyle, in almost every continent of the planet, the other is found only in small, isolated populations. In such communities, it is easier to determine what a person – say, a child with nodular syndrome – has experienced that others are not. "My intuition says this will be important," says Polanen.

So what do we know so far?

There are many invented theories. In Mahenge, the locals have connected kifafa with a premonitory dream, in which they wash, pouring water over their shoulders, enjoying the feeling. Then they open their eyes and the water turns red with blood.

One thing that is clear is not inherited. "Although it tends to appear in families, it's a disease that comes and goes," says Peter Spencer, a neurologist at the Oregon Institute of Occupational Medicine. "It appeared in 1997 in northern Uganda, reached peaks in the early 2000s and then disappeared. You can not explain it as an inherited genetic disease because it just could not fit into this model. "

A major source of inspiration to understand the nod of the syndrome is another equally confusing disease that is largely based on the island of Guam. This lonely hill of grass and shrubs is buried in a remote corner of the Pacific, small pollen, thousands of kilometers from the nearest continent.

Although Guam is technically American soil, it is home to the Chamorro people who lived. with an enigmatic disease at least since 1904. Scientists call it amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC). For locals it is just known as the lytic-body . The disease was discovered after the death certificates registered that several patients died of muscle loss and peaked in the 1950s when it quickly became the leading killer on the island. There is a lytic which resembles the neurodegenerative disease ALS (motor neurone disease) that the late physicist Stephen Hawking lived for decades. Then there is part bodyguard which is strikingly similar to nodal syndrome; both have elements characteristic of Parkinson's and dementia. Technically speaking, lytico and bodig are two separate diseases but they are collected together because of their overlapping ranges and the fact that they have the same unknown cause. the important thing is that this disease is familial and is supposed to be genetic in the 1950s, "says Spencer. "By the 2000s, he apparently disappears, but the geneticists are still desperately trying to figure out some kind of genetic risk factor. Today, it has disappeared from Guam and this is an obvious environmental illness. "It may sound surprising that the demand for nodular syndrome is so early. But for years scientists have followed a false trail. It began with the outbreak of nodular syndrome in South Sudan in 2002 and the discovery that the patients were more heavily infected with two different types of parasitic worms. One type, Onchocerca volvulus became the focus of intensive efforts to prove the relationship.

Oncoccerosis is a particularly terrible disease that begins when a black fly sinks into the victim's mouth as a drooping mouth, leaving behind a generous dose of parasitic worm larvae. For several months, they mature and ultimately form homes of raised lumps in the skin where they mate and start their own worm family. They will produce up to 1500 babies or microfilaments every day that migrate all over the body – invading almost every organ and tissue.

Unbelievably enough the worms themselves are not a problem. When they die, the body shoots a strong and severely damaging immune response against their bodies. This is the proposed trigger of nodding syndrome.

There are some serious perceptions about the theory, however, including the fact that worms are infected with people in parts of Central and South America – where there is no match syndrome. With that in mind, the worm's accusations look like a little stretching.

But there are other interesting traces.

"The head is the most unusual," says Spencer. "This is only described in another case – a disease after measles, which has recently had fires in Israel and Germany, and probably again in Europe."

Rare disease known as aubacute sclerosing panencephalitis (SSPE) can develop if measles are caught at an early age. Just like a nodular syndrome, the typical symptoms include intellectual disabilities and hanging heads. And, like the nodular syndrome, it tends to come to the surface in childhood. Can the nodding of the syndrome also be related to measles?

In northern Uganda, the idea fits remarkably well. to take control, including the notorious child abductor, the warlord and the self-proclaimed prophet Joseph Connie, who was in charge of his own guerrilla force. More than one million people fled their homes, moving to internal camps filled with diseases such as cholera, measles, and rubella.

The Civil War is over, and Connie has a $ 5 million prize for its head. But for the general public, the consequences can continue. Although cases still occur today, reports of nodding of the syndrome in northern Uganda have reached their peak in the mid-2000s, which is about five years after the bloody phase of the war when people flooded in assaulted camps .

because measles virus has a cruel affinity for babies and very young children, and SSPE needs several years to develop; the affected children in Uganda were typically around seven years old.

Now scientists just have to figure out if the idea makes sense in Tanzania and South Sudan. "If we want to find an answer to this disease, then it will have to be relevant to all three geographic isolates," Spencer says. "And we can say that a common feature of all nodal syndrome isolates is the failure of the usual vaccination practice."

Spencer wishes to emphasize that measles is not the only possible cause. It is also known that rubella causes a similar state of SSPE – and since both viruses cause similar symptoms and are prevented with the same vaccine, it is unclear which one is likely to be behind the Ugandan epidemic. It can not be either; is not likely to be excluded, including some kind of yet undetected virus.

Infections can be blamed for a surprising number of cases of neurodegenerative diseases, including Alzheimer's and Multiple Sclerosis

Nevertheless, any viral trigger slot with the latest findings. It turns out that infections can be guilty of a surprising number of neurodegenerative diseases, from herpes simplex virus, which dramatically increases the chances of developing Alzheimer's disease (along with several other culprits, including bacteria that cause gum disease), to the Epstein-Barr virus, which is found in the majority of patients with multiple sclerosis.

Viruses are accused of entering the brain where they cause generalized inflammation and start producing some key proteins that permanently damage them. the normal functioning of the brain. One such protein is beta-amyloid. It has antimicrobial properties and can play a role in the fight against infections. But when Polanen looked at the brains of Ugandan children who had died from the nodular syndrome, it was strangely absent. Indeed, although the team has found plenty of evidence of neurodegenerative disease – like large tangles of another important protein called tau – there were no signs of infection.

"We do not see inflammation that looks like a virus. , we do not see parasites, we do not see evidence of a bacterial infection, "says Polanen. "We do not see the markers of those types of cell or tissue injuries that we'll see. But whether the lack of this completely excludes the virus? We do not know. "Spencer is equally ambivalent. "Now Michael Polanen will rightly say that there is no evidence of the type of pathology you would expect to see in these diseases after measles, post-rubella, and late inception," he says. "But I think it's fair to say that you do not see evidence of an infection in the brains of patients with Alzheimer's disease … so that's a very complex discussion.

Back to Guam, recent discoveries on lytic -bodig can provide an intriguing alternative: diet

Chamorro people like to eat terrestrial cicada seeds – giant-like giant trees from the time of dinosaurs. They are rich in chemical BMAA, which has been shown to cause the same characteristic brain damage found in patients with this disorder. Can food toxins play a role in nodding the syndrome?

The disease is happening in some of Africa's most deprived communities and as such affected children often have poor diets. During the Ugandan civil war, famine caused families in temporary camps to eat various suspect foods, including moldy maize and seeds covered with pesticides – they were provided by emergency programs and intended for planting. Those who ate these foods are more likely to report having a child with a nodule of the syndrome.

Even in good times, staples like manioc and sorghum can put children at risk. Both contain chemicals that break down into cyanide, a potentially lethal compound loved by Victorian killers. They are believed to protect plants from insect-eating and are already involved in the crippling neurodegenerative condom disease that lurks the generations of the poorest and hungry parts of East and Central Africa.

in carbohydrates, calcium and vitamin C, if not properly processed – cut and soaked with water for several days to reduce the cyanide content – then slowly poisoning one who is desperate enough to get it ate it. Toxin destroys neurons that carry signals from the brain to the muscles, and the end result is irreversible paralysis. Many victims turn into quadriplegies

If nodal syndrome has a dietary cause, its disclosure may have a significant global impact, as the strange twin of the disease has already shown; From the discovery that the BMAA can manage the lytic-body scientists have examined whether it can also participate in the development of other neurodegenerative diseases. It turns out that this may be the case – and a chemical known to protect against such damage is currently in clinical trials. It may soon provide a new way of treating patients with ALS and early-stage dementia.

"I can tell you that I am fully committed to the continuation of this study and will assemble a multidisciplinary team that I think will make a significant contribution," said Polanen. "This work can only be done as a team, and it is obvious that no expert will have the general knowledge needed to solve this problem. So this is the approach we will take. "

For now, the mystery of nodal syndrome continues. Who knows, perhaps one day this vague eastern African disease can provide a vital trace that unlocks the cure of many other diseases around the world. Polanen, however, wishes to highlight the dangers of dealing with human tragedy entirely clinically – almost as an experiment. "Especially in my discipline we focus on the immediate consequences of internal armed conflict. We forget that these acts resonate far longer, "he says. "And what we see with nodular syndrome is part of a humanitarian crisis in the region. There is more than a scientific reason to guarantee the well-being of these children. "

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