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Sudan Mystery Disease (Nodding Disease; Nodding Syndrome)

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Mysterious 'Nodding Disease' Hits Sudan

Ten-year-old Susannah Jackson is dying of what may be the world's newest and oddest disease - an illness so rare and mysterious that science has not yet come up with an official name for it.

Sitting outside her family's mud hut, near the small town of Lui, Susannah is gripped by a series of brain seizures which force her neck to arch forwards, down, and then up again.

No wonder people in this isolated corner of southern Sudan call it "nodding disease."

"We have no clue as what is causing this. It's like a detective novel and a murder mystery, because it's fatal," says Dr Mickey Richer, a tropical disease specialist from Unicef.

So far, almost 300 children are known to have caught the disease - all in one small region of the country.

Bizarrely, the seizures normally occur when the sufferers start to eat, or when it is particularly cold.

When Dr Richer asks for a bowl of sorghum to be placed in front of Susannah, the "nodding" begins almost immediately, and stop when she has finished eating.

Curiously, Susannah does not react if she eats unfamiliar food - a chocolate bar for instance.

Susannah's eight-year-old brother, Jacob, is now in Lui's crowded hospital with the same disease - at a more developed stage.

He shows signs of being mentally retarded and physically stunted.

During a particularly fierce seizure he threw himself into a fire, and is now being treated for severe burns to his leg.

Two other children in the same ward have almost identical stories.

An hour's drive from Lui along a rutted track lies the village of Amadi - a silent cluster of huts trapped in a forest of thick green grass.

Amadi is now considered to be the epicentre of the disease - 12% of children here are affected.

Last year, experts from the World Health Organisation did neurological scans on some of the children, enabling them to confirm that this is a specific and unique condition.

Twelve-year-old Ruben Nicholas stands in the middle of the village, gripped by a sudden seizure.

It looks as though someone is forcing his chin down onto his chest.

"He is nodding three times a day," says his father, Nicholas Lado.

"The disease stops him from growing. His brother died from the same disease, in August."

Dr Richer gets out her stethoscope and starts examining a dozen children. A small boy called Maika Philip stares dully into the distance. He looks about 11, but his mother confirms that he is 18.

So what could be causing this horrific outbreak? If this had happened in western Europe, it seems likely there would be at least some answers by now.

Here, in poverty-stricken southern Sudan, families still do not know if they should be trying to quarantine their children.

"If one child has nodding, we separate them from the other children, because maybe it's through air we don't know," says Reverend Sosthen Amen Lati, whose son is affected.

Some villagers say the disease is a curse, others blame the country's long civil war and suspect that government forces have been dropping chemical weapons on Lui and other rebel-held areas.

A toxicology report commissioned last year by the United Nations comes close to ruling out chemical warfare, and also plays down the likelihood of any link to local diets or food production.

At the same time it cautiously raises a few intriguing theories, including the possibility of infected monkey flesh.

International relief aid is also mentioned. Some locals have admitted to eating donated seeds, meant for planting not consumption, which are coated with toxic substances.

But Dr Richer's hunch is more straightforward. She notes that the victims are all concentrated near the Yei River.

She also points out that 93% of those surveyed are infected with a parasitic worm which causes Onchocerciasis (also known as river blindness).

The level of infection among children without "nodding" is 63%. The worm is carried by black flies which breed near fast-flowing rivers like the Yei.

Could it turn out to be the killer in Dr Richer's mystery?

http://news.bbc.co.uk/2/hi/africa/3133440.stm
 
so have any other adults been affected then?
 
It's extremely likely the worm is involved, although why are the 63% of unaffected children with the worm showing no symptoms. (Doesn't discount it as the cause, but knowing why people with the worm don't show symptoms will help explain what is causing it.)

The symptoms sound eerily reminiscent of chiru (or scrapie, or BSE for that matter). So it could be a prion disease, or it could just attack the brain in a similar way.
 
At a place I worked in a couple of years back there was this lad who used do have violent involuntary nodding fits. It appeared that he was unaware of them when the minor ones happened, but only too aware when the major ones happened and he'd do stuff like nearly whack the computer monitor. In fact, I recall that they had to call an amublance for him on one occassion after he'd headbutted the wall in the smoke wall and knocked himself out cold.

I never found out what was up with him.

Perhaps the children mention in this report are some kind of human equivalent to myelin puppies except with a gradual degrading of the neuron structure as opposed to a congenital one. Brought on by worms.
 
anome said:
The symptoms sound eerily reminiscent of chiru (or scrapie, or BSE for that matter). So it could be a prion disease, or it could just attack the brain in a similar way.

Yes, it made me think of a prion disease as well. If they eat monkey meat, it could well be. BSE came as a bit of a surprise as the prion had to make a species jump to get from cows to us. Kuru came from people eating the brains of their ancestors. If the kids are eating monkey meat, there is not so much of a species jump to make, so it is very possible.
 
Mystery Disease Terrifies Sudan Officials
Wed Jan 28,11:18 AM ET Add Health - AP to My Yahoo!


By EMMA ROSS, AP Medical Writer

KACNGUAN, Sudan - Martha Halim is stricken with mysterious seizures that frighten her from eating. Her parents have tried everything to diagnose the ailment. She's been to a hospital, she's seen a Western doctor and she's taken anti-epileptic drugs.

Halim lives in fear. She is terrified of the moon's phases, afraid of eating and fearful of fires, rivers and ponds.

The 13-year-old has been to witch doctors and followed the advice of one, crawling through a termite mound while her parents slit the throat of a goat.

She gives a grim description of what it's like when her disease overpowers her.

"When it comes, it looks like a black cloud but in the shape of a human," said Martha. "That's all I know. At the end, I find myself on the floor."

Martha suffers from a strange affliction called "nodding syndrome," apparently unique to southern Sudan. Its young victims tend to nod vigorously at the sight of food. The condition often progresses to severe seizures, mental retardation and death.

Martha fell into a fire last year when she had a seizure while cooking. Her right leg is disfigured by a severe burn from knee to foot; she protected it with a soiled beige rag.

Her father, Neen Majak, says he has nearly given up hope. Anti-epileptic drugs haven't helped and neither have the remedies of witch doctors.

The affliction, which has been found in about 300 children so far, baffles experts. The World Health Organization began investigating it about two years ago, around a year after Martha's symptom first appeared.

Peter Spencer, an American neurotoxicologist who has investigated the condition for WHO, encountered another 13-year-old girl with a bizarre variation of the illness.

"I was able to demonstrate with her that she was a regular nodder with local food and by contrast she did not nod when eating a variety of American food — candy bars or whatever. It was absolutely staggering," he said.

As she sits on a sisal mat with her parents under the shade of a tree beside their mud huts, Martha says no treatment has helped her.

One traditional healer claimed that Martha's aunt, who was killed by lightning a few years ago, had bewitched the girl. Following his advice, the family held a ceremony attended by the entire village and sacrificed a sheep.

Recently, another traditional healer told the family to take the girl into the forest to wash the bad spirit away.

"He told us to bring a black goat and a red hen and make a tunnel in a termite hill," Majak said. "She crawled through the hole back and forth three times. Then we had to kill the goat and the hen in sacrifice."

"You can consider her a dead person, because she is not going to marry and she is going to die of this disease," her father says. "If this treatment doesn't work, then all I can do is wait to let the child die."

Experts say a few children recover. Doctors with WHO think the disease may be related to a disorder seen in Uganda called Nakalanga syndrome, which also has symptoms of convulsions, stunted growth and sometimes nodding.

Spencer's investigation has found no obvious environmental causes. He wouldn't rule out a food connection, but said it is unlikely.

"What was striking is that the majority of the population that is affected by this disease in southern Sudan has a different lifestyle from the itinerant Dinka people, who are sort of herders. They are not affected by this disease," he said.

Spencer said one theory that cannot be ruled out, although it is not a leading suspicion, is the disease could have come from eating monkeys. Ebola can be spread to humans by chimpanzees. AIDS also made its way from primates to humans.

Spencer and other investigators believe nodding syndrome could be connected to river blindness, a disease transmitted by the blackfly, which is particularly widespread in southern Sudan. Martha and several other members of her family are afflicted.

Unraveling the mystery of nodding syndrome is a question of money and time, Spencer said.

"If we're smart, we will unravel it. We won't let it burn on like we did HIV," he said. "You just cannot imagine a greater disaster for a community than their children being hit in this way."


http://story.news.yahoo.com/news?tm.../20040128/ap_on_he_me/sudan_exotic_diseases_1
 
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"... she was a regular nodder with local food and by contrast she did not nod when eating a variety of American food — candy bars or whatever...

I find that rather odd. Maybe it's the packaging, but then what if this is an elaborate and wide-spread hoax by the 300 children to get candy bars. I mean, after all, kids love candy bars.

But then that's just me assuming again. :cross eye
 
I'm guessing that the major source of food in the region would be locally-grown crops... if there was a pathogen in the local crops then the poisoned children may well recover, at least partially, when moved away from the area and/or given different food.

Jane.
 
Sudan: Hell on Earth?

February 1, 2004

World's worst diseases come together in southern Sudan

By Emma Ross
The Associated Press




RUMBEK, Sudan - It sounds like a place stricken by a biblical plague - disease after unimaginable disease, all come to rest in one pitiful region of a vast African country.

Worms oozing out of people's feet; two kinds of flies whose bites cause death in bizarre ways; a baffling syndrome that throws children into seizures and retards their development before it ultimately kills them.

While some of these devastating diseases can be found scattered around the world's poorest places, it is only in southern Sudan that they are all seen together in one country at the same time.

``This really is the forgotten front line when it comes to health,'' said Francois Decaillet, a public health specialist at the World Bank who has 20 years of experience in Africa.

Southern Sudan is one of the poorest and most neglected areas on Earth, with possibly the worst health situation in the world.

There is, in essence, no health care system; humanitarian groups provide nearly all the doctors and medicine. There are a total of three surgeons serving southern Sudan, which covers 80,000 square miles - 1 1/2 times the size of Iraq. There are three proper hospitals, and in some areas there is just one doctor for about 500,000 people. Experts estimate 6 million to 8 million people live in the region.

Situated in northeast Africa, just south of Egypt, Sudan is the largest country on the continent. It has been in and out of civil war since 1955, and while northern Sudan is about as developed as its neighbors, the south has been ignored and, even by African standards, is unusually underdeveloped.

Peace talks between the government in the north and rebels in the south continue, but large parts of southern Sudan are still inaccessible to aid groups. International health workers are hopeful of greater access if peace is achieved, but for now the situation remains uncertain.

Operation Lifeline Sudan, the United Nations-led humanitarian effort, has about 700 workers on the ground at any one time, covering everything from food distribution, education, human rights and health. Because of the war, aid workers have frequently had to evacuate their posts, making it difficult to establish any long-term programs.

Malaria, diarrhea, malnutrition and respiratory infections are the biggest killers here - as in most of Africa.

But what's unique is that southern Sudan has the double burden of those illnesses as well as a number of ghastly tropical diseases that have been stamped out in most of the world.

The area is a hotbed of exotic diseases, from the feared Ebola virus, which was first identified here and in Zaire, to nodding syndrome, a newly discovered life-threatening condition that attacks children and appears to be unique to southern Sudan.

The geography and environment - with a succession of floods and droughts - are hostile enough. Their combination with long-running war, primitive isolation, poverty and exotic disease is rarely seen in one place at one time elsewhere in the world.

Such conditions have enabled the full range of nasty infectious diseases - many of which are fading out or become nonexistent elsewhere - to flourish, says Dr. Nevio Zagaria, an expert in neglected diseases at the World Health Organization.

But even if doctors could reach the people of southern Sudan, many of their diseases are untreatable. There isn't much of a market for drugs to treat obscure illnesses in a poor country, so medicine makers have little to offer, Zagaria says.

One of the most stubborn afflictions in the south is Guinea worm disease, something all but defeated almost everywhere else. Last year, 80 percent of the world's cases were reported in southern Sudan.

http://www.registerguard.com/news/2004/02/01/a13.int.sudan.0201.html
 
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