Home / Health / BBC – Future – Kifafa: The mystery of "head disease in Africa"

BBC – Future – Kifafa: The mystery of "head disease in Africa"

For the Wapogoro people, there is no worse fate than Kifafa.

The tribe lives in the Mahenge highlands, deep in Tanzania: a land of rugged mountains, mist-covered forests and rare underground treasures, ranging from fossils to sparkling neon-pink gems. They have survived in this isolation for hundreds of years, managing quietly and largely escaping the attention of the world.

Or it was the case, until the arrival of a Norwegian doctor in 1959. Louise Jilek-Aall was only 28 years old, she had just left the hospital. medical school and was determined to create a clinic in the area. But no sooner had she started seeing patients than she had noticed anything special.

Almost every day, children as young as two years old found themselves in his office with severe burns. In one case, a little girl was rendered completely unrecognizable; many others died of their wounds. The burns were often superimposed on pale pink scars, which had accumulated after years of previous burns.

When Jilek-Aall tried to find out what was going on, she met fear and escape. Even the relatives of the affected children have categorically refused to explain the ominous trend

In fact, the youngest members of the tribe appeared to be particularly susceptible, it is said, to a wide range of accidents, including regular drownings. When Jilek-Aall tried to find out what was going on, she met fear and escape. Even the relatives of the affected children have categorically refused to explain the sinister trend.

Jilek-Aall eventually realized that his patients had seizures, which they called Kifafa – Swahili for "the little dying" – and falling. Sometimes they landed in the open fireplaces where they prepared their food. Sometimes they ended up in the river.

But the mystery did not stop there. The same children also tended to have stunted growth, intellectual difficulties and characteristic mannerisms, such as a clumsy approach and impassive facial expressions. The most striking is the "head nodding"; several times a day they closed their eyes and left their heads against their chests several times. The illness usually lasts for several years and ends with an early death.

Finally, even if all members of Wapogoro were afraid of Kifafa to an almost feverish degree, the disease was virtually unknown elsewhere in Tanzania. This was even true of neighboring tribes.

The head syndrome, since discharged, has been discovered in parts of East Africa, from southern Sudan to northern Uganda, where it has devastated the lives of thousands of people. At each new location, it seems to come out of nowhere. What could be behind this deadly wound? And how can we stop it?

To this day, no one knows what causes shame syndrome. In the past, it was generally accepted that the disease was a rare and enigmatic form of epilepsy. But now, the latest research suggests that it could be a neurodegenerative disease manifesting itself in a combination of epilepsy and Parkinson's and Alzheimer's symptoms. Ultimately, victims usually succumb to seizure-related complications, such as brain damage and stroke, or poor mental health and lack of care.

The tempting index appeared in a study in 2018, which found that the brains of teenagers with the disease resembled remarkably that of older patients with the latter two diseases. "This has generated a new line of questioning because we now know that some type of brain damage is occurring," says Michael Pollanen, pathologist at the University of Toronto. "You can think like this: if you can determine the type of brain injury, you may be able to go back to the cause."

At the very least, overlap with other diseases adds an extra sense of urgency to the search. The headache syndrome affects some of the most vulnerable people on the planet, but it can also be the key to understanding many of the issues that have long eluded scientists.

If you like it, you will also enjoy:

If you compare head syndrome to Alzheimer's disease, you will understand why. While one has been found in millions of people, with largely dissimilar lifestyles, on almost every continent of the planet, the other only appears in small isolated populations. In these communities, it is easier to identify what a person – a child with nodding headache, for example – has lived like others. "My intuition says it will be important," says Pollanen.

So, what do we know so far?

There are many imaginative theories. In Mahenge, the inhabitants have linked Kifafa to a disturbing dream, in which they wash themselves, pouring water on their shoulders, savoring the feeling. Then they open their eyes and the water becomes scarlet with blood.

One thing is clear: it's not inherited. "Although this usually happens in families, it's an illness that comes and goes," says Peter Spencer, a neurologist at the Institute of Occupational Health Sciences of the University of Toronto. 39; Oregon. "It appears in 1997 in northern Uganda, peaks in the early 2000s and then disappears. You can not explain that as an inherited genetic disease, because it simply would not fit that model. "

Another equally disconcerting source of inspiration, largely affecting the island of Guam, is a major source of inspiration for understanding the neon syndrome. This solitary hill of grass and scrubland is stuck in a remote corner of the Pacific Ocean, a tiny point, thousands of miles from the nearest continent.

Although technically, Guam is American soil, it houses the Chamorro people, who have lived for at least 190 years with an enigmatic disease. Scientists call it amyotrophic amyotrophic lateral-parkinsonism-dementia complex (ALS-PDC). For locals, it's just about Lytico-Bodig. The disease was discovered after death certificates revealed that several patients had died from muscle loss and had peaked in the 1950s, by which time it had quickly become the leading cause of death. mortality on the island.

The disease can take two forms. There is Lytico, which resembles the neurodegenerative disease SLA (motor neuron disease), experienced by the late physicist Stephen Hawking for decades. Then there is the Bodig part, which is surprisingly similar to the head syndrome both have typical elements of Parkinson's disease and dementia. Technically speaking, Lytico and Bodig There are two distinct diseases, but they are grouped together because of the overlapping of their ranges and the fact that they have the same unknown cause.

"The important thing is that this disease was familial and thought to be genetic in the 1950s," says Spencer. "In the 2000s, it was clearly disappearing, but geneticists were still desperately trying to find a genetic risk factor. Well, today he's gone from Guam and it's clearly an environmental disease. "

False tracks

It may seem surprising that the search for head nodal syndrome is at such an early stage. But for years, scientists have followed a false track. This began with an epidemic of shame syndrome that occurred in South Sudan in 2002, and the discovery that patients tended to be more heavily infested with two different types of parasitic worms. A type, Onchocerca volvulus, has become the focus of intense efforts to prove a connection.

Onchocerciasis is a particularly horrible disease. It begins when a black fly sticks its saw-like mouth into the victim's skin, leaving behind a generous dose of parasitic worm larvae. Over the course of several months, they mature and end up lodged from raised bumps in the skin, where they mate and create their own family of worms. Each day, they will produce up to 1,500 babies, or microfilariae, that will migrate all around the body, invading almost all organs and tissues.

Incredibly, the worms themselves are not a problem. It is when they die that their body triggers a powerful and extremely damaging immune reaction against their carcasses. This was the suggested trigger of head syndrome.

However, this theory presents serious problems, including the fact that worms are known to infect people in parts of Central and South America, where head syndrome does not exist. In this spirit, blaming worms seems a little exaggerated.

But there are other interesting tracks.

"The nods are very unusual," says Spencer. "This has been described in only one other case – a post-measles disorder, which has had recent epidemics in Israel and Germany, and will probably be in Europe again."

The rare disease, known as sclerosing panencephalitis aubacute (SSPE), can develop if measles is detected early. Like the head syndrome, typical symptoms include intellectual difficulties and a drooping head. And just like the head nods syndrome, it tends to surface during childhood. Could the neben syndrome be linked to measles?

In northern Uganda, the idea fits remarkably well.

From the late 1980s to 2006, the region was the epicenter of a fierce civil war. Three factions fought for control, including the infamous kidnapper, warlord, and self-proclaimed prophet Joseph Kony, who was leading his own guerrilla warfare. . More than one million people have fled their homes and settled in internal camps contaminated by diseases such as cholera, measles and rubella.

The civil war is over for a long time and Kony has a premium of $ 5 million (£ 3.8 million) on his head. But for the general public, the consequences could be persistent. Although cases continue to appear to date, cases of nodding head syndrome in northern Uganda peaked in the mid-2000s, about five years after the deadliest phase of the war. when people have infiltrated infested camps.

This is a good time because the measles virus has a cruel affinity for infants and very young children, and the development of SSPE takes several years. In Uganda, affected children were typically around seven years old.

Scientists must now determine whether this idea makes sense in Tanzania and South Sudan. "If we want to find a solution to this disease, it will have to be relevant to all three geographic isolates," says Spencer. "And we can say that a common trait of all shame syndrome isolates is a failure of normal practice of vaccination."

Spencer wants to point out that measles is not the only possible cause. Rubella is also known to cause a condition similar to SSPE – and since both viruses cause similar symptoms and are prevented with the same vaccine, it is not known which of these viruses is likely to cause the Ugandan outbreak. It could be neither one nor the other; no possibility is excluded, including some kind of virus not yet discovered.

Infections can cause a surprising number of cases of neurodegenerative disease, including Alzheimer's disease and multiple sclerosis.

Anyway, any viral trigger would integrate well with recent discoveries. It turns out that infections can be at the origin of a surprising number of cases of neurodegenerative disease, due to the herpes simplex virus, which greatly increases the chances of developing the disease Alzheimer's (as well as several other culprits, including the bacterium that causes gum disease), the Epstein-Barr virus, present in the vast majority of patients with multiple sclerosis.

Viruses are accused of entering the brain, where they cause widespread inflammation and relaunch the production of some key proteins, which permanently alter the normal functioning of the brain.

One of these proteins is beta-amyloid. It has antimicrobial properties and can play a role in the fight against infections. But when Pollanen examined the brains of Ugandan children who died as a result of shame syndrome, he was oddly absent. In fact, although the team found abundant evidence of neurodegenerative disease – such as large entanglements of another important protein called tau – there was no evidence of infection .

"We do not see inflammation that looks like a virus, we do not see parasites, we do not see any evidence of a bacterial infection," says Pollanen. "We do not see the markers of these types of cell or tissue damage that we would see. But does the absence of that totally exclude a virus? We do not know. "

Spencer is also ambivalent. "Now, Michael Pollanen will correctly say that there is no evidence of the type of pathology that one would expect to see in these post-measles, post-rubella, post-rubella diseases. Late appearance, "he says. "But I think it's also fair to say that you do not see evidence of infection in the brains of patients with Alzheimer's disease … so it's a very complex discussion. "

Back in Guam, the latest discoveries on Lytico-Bodig can provide an intriguing alternative: diet.

Chamorro people like to eat crushed cycad seeds, giant fern-like trees that have existed since the reign of the dinosaurs. They are rich in chemical BMAA, which has been shown to cause the same brain damage as in patients with the disease. Could food toxins also play a role in head syndrome?

The disease occurs in some of the most disadvantaged communities in Africa and, as such, affected children often have poor diets. During the Ugandan civil war, hunger drove families in temporary camps to eat various dubious foods, including moldy maize and pesticide-coated seeds, provided by emergency relief and planting programs. Those who ate it were also more likely to report having a child with head syndrome.

Even in times of prosperity, commodities such as cassava and sorghum can put children at risk. Both contain chemicals that break down into cyanide, a potentially deadly compound well-liked by murderers of the Victorian era. It is thought that they protect plants against the consumption of insects and are already involved in the disabling neurodegenerative disease, konzo, which has been tracking for generations the poorest and most hungry regions of the world. Eastern and Central Africa.

Although cassava root is rich in carbohydrates, calcium and vitamin C, if it is not processed properly – cut into pieces and soaked in water for several days, to reduce the cyanide content – it will slowly poison those who need it most. The toxin destroys the neurons that transmit signals from the brain to the muscles and results in irreversible paralysis. Many victims end up becoming quadriplegic.

If the syndrome of the head has a food cause, then discovering it could have a significant global impact, as has already been shown by the strange twin of the disease; since the discovery that BMAA could drive Lytico-BodigScientists have checked to see if it could also be involved in the development of other neurodegenerative diseases. It turns out that this could be the case – and a chemical known to protect against such damage is currently undergoing clinical trials. It may soon offer a new way to treat patients with ALS and early dementia.

"I can tell you that I am totally committed to continuing this research and that I will be training a multidisciplinary team that I think will make a significant contribution," said Pollanen. "This work can only be done as a team and it is obvious that no expert will have all the necessary knowledge to solve this problem. It is therefore the approach we are going to adopt. "

For the moment, the mystery of the head nods syndrome persists. Who knows, maybe one day this obscure disease of East Africa could provide a vital clue to finding treatments for many other diseases in the world. But Pollanen stresses the danger of viewing the human tragedy in a purely clinical way – almost as if it were an experience. "In my discipline in particular, we tend to focus on the immediate consequences of an internal armed conflict. We forget that these acts resonate much longer, "he says. "And what we see with head-pushing head syndrome is part of a humanitarian crisis in the region. There is more than one scientific reason for ensuring the well-being of these children. "

Join more than 900,000 Future fans by loving us on Facebookor follow us on Twitter or Instagram.

If you liked this story, sign up for the weekly newsletter on the features of bbc.comcalled "If you only read 6 things this week". A selection of BBC Future stories, Culture, Capital and Travel, handpicked, delivered to your inbox every Friday.

Source link