July 29, 2017 by
It starts with the nodding — otherwise normal children begin to nod their heads, pathologically. Then come the seizures. The children stop growing and stop talking. Ultimately, the disease wrecks the children, physically and mentally. The strange and deadly illness known as nodding syndrome affects only children, and only in a small pocket of East Africa. It has affected more than 3,000 children since the late 1990s, when it first appeared in what was then southern Sudan. And for more than three years, the cause of nodding syndrome has eluded epidemiologists around the globe. Investigators at the Centers for Disease Control in Prevention in Atlanta have made the syndrome among their top priorities on the short list of new, unexplained outbreaks. But as they, and others, continue to investigate the syndrome — figuring out its origin and how it spreads — much of the work trying to treat affected children has been left to Uganda. "Because it's not really a ward," Nyeko says. "It's just somewhere where they have dumped these children." One of five that the Ministry of Health opened last summer in northern Uganda, the ward in Pader is a squat, one-story cement building. It's not spacious. It couldn't handle much more than the 11 mattresses, which sit directly on the ground, that run the length of the walls. Lamona Santiana sits on one of these mattresses. She came here a week ago with her grandson, Denis. Santiana says that for Denis, as with most children affected, it began with the pathological nodding of the head nearly a decade ago. He's 16 now, she says, but you can only get a glimpse of his age in his face – he looks half the size you'd expect him to be. He rarely moves. He hardly speaks. If he wants water, she says, he'll take a cup nearby and tap the cement floor with it. Most everything else is nonsense, hallucinations. "He's just in his own world," Nyeko says. This happens with children with nodding syndrome: They fall apart cognitively. And so what Nyeko and others hired by the Ministry of Health are trying to do is bring children like Denis back to some sort of normalcy, which is difficult to do here. Health care in Uganda isn't good. Less than one-third of the major health centers in the country are fully operational. Still, the Ugandan Ministry of Health carved out $1.4 million in its budget last year to mount a response to nodding syndrome. Medicine Treats The Symptoms The ministry hired and trained physicians and nurses, like Nyeko. It bought big white vans, emblazoned with "Nodding Syndrome Response Team" in blue lettering. It also bought more than a million tablets of a generic anti-convulsant called sodium valproate, a drug that came on recommendation from the Centers for Disease Control and Prevention and the World Health Organization. The idea is that if you can begin to control the nodding — in other words, the seizure — then maybe you can start to return a child to normal. Workers get the drug to nearly all of the 3,000 affected Ugandan children by covering thousands of square miles of dirt roads. As of now, there aren't numbers, or at least nothing peer reviewed, to back up how well the drug is working, so instead you get anecdotes like Geoffrey Tabo. In a small village in Pader, Geoffrey sits cross-legged next to his father in a patch of grass, away from the crowd of parents and children who were still awaiting treatment. He says he doesn't remember much from when he was sick. His father fills in the blanks, describing how the nodding began three years ago. Over that time, he watched his son withdraw from friends and eventually stop talking all together. But, after six months on sodium valproate, Geoffrey can hold a conversation. He has returned to school. He's in the third grade and says he likes it. There are other stories like Geoffrey's. "Some of the worst affected ones couldn't feed themselves and now they're feeding themselves," says Robert Downing, a microbiologist at the CDC's campus in Entebbe, Uganda. "One of the ones I saw couldn't walk and now is walking." The CDC in Atlanta has spent hundreds of thousands of dollars and countless hours assisting the Ugandan government in investigating nodding syndrome. Among the possibilities explored is whether nodding syndrome could be caused by war-related chemical exposure, a response to a local parasite, or a vitamin deficiency or genetics. So far, investigators have hit dead ends. So, short of a cause, they're trying to treat a symptom. Sodium valproate has proved effective in treating seizures similar to nodding syndrome, which is why the CDC and the World Health Organization recommended the drug to the Ugandan government as a potential treatment. Parents Do The Best They Can But Downing says that any anti-convulsant treatment is only one aspect of a response plan the CDC helped draft with the Ugandan government last year. There were also plans for re-education, nutrition, rehabilitation and more. But those programs were reliant on outside donors and have either withered up, gone unfunded or unimplemented. And nongovernmental organizations that champion nodding syndrome as a cause are few and far between, leaving families with children who might not nod as much, but are nowhere near normal. "So they do need help," Downing says. "And ..." He lets out a heavy sigh. "There's so little there." The outreach team in the district of Pader makes 27 house visits every month, going to see children one by one in some villages. At one home members visited recently, the parents were gone. Morris Oyo, lets us in. There, his brother, Robert, lies on the dirt floor, back against the clay wall, slowly eating rice from a bowl. A linked chain is wrapped around his ankle. It runs up to the roof, tied to a wooden beam. Morris tells me his brother has been nodding for 10 years. When it got bad, his parents took to tying him up in the hut so they could continue to work the fields. Most parents fear if their child is left alone, they might wander off, drown in a nearby well or fall into a fire. Morris says his parents can't afford to do anything else. After we walked out of the hut, a few from the response team told me they will often forcibly remove children from their homes if they think the child is neglected. I ask them if this constitutes neglect. They tell me no. This is just the reality of the situation. Matthew Kielty is an NPR Above the Fray fellow, sponsored by the John Alexander Project, dedicated to supporting young journalists and finding untold stories. Seeking A Return To Normalcy The nodding syndrome ward in the northern district of Pader, in Uganda, is a bleak place. Even a nurse here, Nicole Nyeko, will admit as much.

July 29, 2017 by
Between 1990 and 2013, thousands of children in war-torn South Sudan and northern Uganda suddenly developed a severe and puzzling form of epilepsy. When exposed to food or cold temperatures, affected children nodded their heads uncontrollably. Over time the seizures often worsened, leaving the children severely disabled. Many died of malnutrition, accidents, or secondary infections. A boy receives ivermectin to prevent river blindness. Some think the drug may also prevent nodding syndrome. In some communities, roughly half of families had at least one child with the condition, called nodding syndrome; by 2013, an estimated 1600 children in Uganda were affected. But the cause of the devastation was a mystery. Now, a study finds that a parasitic worm often found in the children might trigger the body’s own defenses to attack neurons. The study doesn’t prove the worm is the culprit, but it “is the first to show that a cause-effect relationship is plausible,” says Hermann Feldmeier, a parasitologist at the Charité University Hospital in Berlin, who was not involved in the study. The rash of cases in Uganda and South Sudan triggered an intense hunt for the cause, but searches for viruses, bacteria, environmental toxins, genetic factors, and nutritional deficits all came up empty. One key clue: Areas with nodding syndrome also had high rates of onchocerciasis, an infection with the parasitic worm Onchocerca volvulus. Spread through the bites of black flies, which breed in swift-flowing streams, the worms can invade the eye, and the infection is commonly known as river blindness. The World Health Organization estimates that at least 18 million people, most in sub-Saharan Africa, are infected. Researchers had suggested as early as the 1960s that high rates of epilepsy in Tanzania, with similar nodding symptoms, might be related to onchocerciasis. Others have noted that children with nodding syndrome are more likely to be infected than their healthy peers. But there’s no evidence that the worm invades the brain or directly causes seizures. Some researchers suggested that the worm instead causes an autoimmune reaction that damages the nervous system. Searches for antibodies that might play an autoimmune role had come up empty. But neuroimmunologists Avindra Nath and Tory Johnson of the National Institutes of Health in Bethesda, Maryland, decided to use an improved protein chip to screen for antibodies to thousands of proteins at once. The new tool proved its worth. Blood from nodding syndrome patients reacted strongly to four proteins; in the case of one protein, called leiomodin-1, patient sera reacted 33,000 times more strongly than did sera from unaffected controls. The researchers then looked for the antibodies causing the reaction. As they report this week in Science Translational Medicine, antibodies to leiomodin-1 turned up in 29 of 55 nodding syndrome patients but only 17 of 55 controls. Patients also carried much higher antibody levels than controls. Leiomodin-1, which likely plays a role in cell shape, is found in smooth muscle and thyroid cells. Johnson’s team showed that it is expressed in the nervous system and brain, too. They also found a clue to what might trigger an autoimmune reaction to the protein: Several O. volvulus proteins resemble it. After the immune system gears up to fight the worm, similarities between an O. volvulus protein and leiomodin-1 may cause the antibodies to mistakenly attack neurons. The study gives little hope to children already affected, Nath says. Although antiseizure drugs can help, if the immune system has attacked neurons, the damage is likely permanent. However, the work could suggest a straightforward way to eliminate the disease, says infectious disease specialist Robert Colebunders of the University of Antwerp in Belgium, because the drug ivermectin kills the worm. Existing campaigns to eliminate river blindness by giving the drug could have a collateral benefit: After the Ugandan government stepped up ivermectin treatment, new cases of nodding syndrome plunged to nearly zero, Colebunders says. “If you eliminate the onchocerciasis, the epilepsy really disappears.” Yet the link between the worm and nodding syndrome doesn’t explain why the illness suddenly appeared in a region where onchocerciasis has likely been common for centuries, or why nodding syndrome only affects children and youth. Johnson, now at Johns Hopkins University in Baltimore, Maryland, says malnutrition, exposure to other diseases, or genetic variation in how the body makes antibodies may also play a role. Other researchers have suggested that measles infection followed by malnutrition could trigger the disease. Neurologist Erich Schmutzhard of the Medical University of Innsbruck in Austria has other doubts. He says that the leiomodin-1 antibodies could be a result of epilepsy, not its cause. The protein seems to be on the inside of neurons, not the outside, he notes. Seizures kill neurons, and he speculates that dying neurons could spill the protein into the blood stream, triggering the antibodies. The onchocerciasis connection is intriguing but far from definitive, says neurologist Andrea Winkler of the Technical University of Munich in Germany. She, too, thinks the syndrome is likely caused by multiple factors, such as malnutrition, parasites, and viruses like measles. “There are still lots of links missing.” Original Article written by Gretchen Vogel from sciencemag.org

August 11, 2017 by
In parts of Eastern Africa children in some communities have been afflicted by a mysterious condition. When given food or experiencing bouts of cold weather, they are struck by a strange form of epilepsy that makes the children uncontrollably nod their heads. Known as “nodding syndrome”, the condition eventually gets worse, with the children suffering more severe seizures leading to disability and occasionally death. Over a 23 year period from 1990 to 2013, thousands of children suddenly came down with the condition in South Sudan and Uganda, leading a team of researchers to start investigating the cause of the syndrome. It turns out that this strange form of epilepsy may be down to a parasitic worm common in the region, and the cause of another prevalent disease. Known scientifically as Onchocerca volvulus, the nematode worm is usually associated with the neglected tropical disease, river blindness. Found mainly in sub-Saharan Africa, it is thought that up to 120 million people are at risk from the disease, which is spread by the bite of the black fly in which the worm spends half its life cycle. It tends to cause severe itching of the skin, development of bumps under the skin, and can eventually lead to a thickening of the cornea that causes blindness. Now, in a new paper published in Science Translational Medicine, researchers suggest that it could also be responsible for nodding syndrome, by over-stimulating the immune system that then attacks the neurons. They are careful to note that they have not proven that this is the case, but they found evidence that those with the syndrome were much more likely to have a particular antibody that targets a protein called leiomodin-1. This protein is found in smooth muscle and thyroid cells, but it is also present in brain tissue and the nervous system. But more importantly, it also closely resembles other proteins typically found on the surface of O. volvulus nematodes. The researchers suspect that the immune system may have originally been launching an attack against the worm, but then got confused and mistook the proteins on the body’s own neurons for the parasite. The cases closely resemble another situation seen in Tanzania, where in the 1960s doctors suggested that children experiencing epileptic seizures and nodding symptoms may well have been related to the presence of river blindness. This means that the mysterious nodding illness could, in theory, be easily treated by ridding the children of the nematodes, which should also stop the seizures. There has been a push for the treatment of river blindness to be expanded, which is achieved using the drug ivermectin, something that the Carter Center is now working towards. Article retrieved from: http://www.iflscience.com/health-and-medicine/cause-of-mysterious-nodding-syndrome-may-have-been-discovered/

December 16, 2017 by
Omoro- Omoro District leaders, Acholi Region of Northern Uganda, have asked government to take over the Nodding Syndrome Care Facility in Odek Sub-county, citing financial constraints. The care centre has since 2012 been supported by Hope for Humans, a non-governmental organisation. Hope for Humans was providing medical and personal care, special schooling, and nutritious meals to children with nodding syndrome. The leaders say the facility currently lacks funds to adequately take care of children and carry out outreach programmes in homes of those recovering from nodding syndrome. A total of 29 are currently at the centre while 237, who were rehabilitated, have been reintegrated within communities. The district chairperson, Mr Douglas Peter Okello, told Daily Monitor in an interview recently that managing the care centre has been complex since Hope for Humans cut their support. The decision The organisation’s chief executive officer, Ms Suzanne Gazda, in a July email seen by Daily Monitor, noted that board of directors had made a ‘difficult decision’ to dissolve the organisation and transition the children into the care of Ugandan government. According to Mr Okello, the lives of the children are at a risk since they are not receiving adequate food ration and special care. “We have many children who had shown signs of recovery but they are now relapsing because of lack specialised care and treatment,” Mr Okello said. He revealed that many parents of the affected children are poor peasant farmers. However, Mr Okello also suggested that government should expand the centre into a special needs school to cater for special needs children. The district health officer, Mr Ben Ongom, said although the government through Office of the Prime Minister [OPM] recently gave some food donations, the high operational cost of the facility still remain a big challenge. He said the district health department cannot afford to support the budget and activities of the facility since they too are cash strapped. “Our proposal to government is that they should extend more support to the centre so that it operates normally. We are unsure of how the centre will operate next year without adequate funding,” Mr Ongom said. Mr Caesar Okot, the programme manager Hope for Humans, acknowledged the financial cut. “Our donor had initially intended to stop funding the centre by August but after a series of engagements, the donor accepted to extend support to December 31. What we are receiving currently is a minimum support,” Mr Okot said. He noted that they have made proposals in consultation with the district leaders seeking government’s intervention before they completely run out of funding. Ms Grace Kwiyocwiny, the State Minister for Northern Uganda, however, said government through the Ministry of Health has been supporting the centre for the past years. “Every quarter, the government has been giving financial support to the centre. The problem could be arising from how the district leadership are drawing their budget to use the money; government is supporting the centre…..” Ms Kwiyocwiny said in a telephone interview on Monday. She, however, did not specify how much the centre has been receiving quarterly from the government. But information this newspaper obtained contradicts the minister’s statement that government has been supporting the care centre. According to a source at the district, government only released Shs10 million in the last financial year when Omoro had just gained a district status to enhance outreach programmes. An estimated 3,000 children mainly in Kitgum, Gulu, Pader and Lamwo districts in Acholi Sub-region were affected by the strange since 2009 when its outbreak was first reported. By JULIUS OCUNGI