The teenager in the photo can't stand alone. He was lifted up with his feet in front of the thatched roof hut in northern Uganda. His face was obscured for privacy, but it was clear how his leg muscles had disappeared. He looks a bit like an old man.
Like thousands of other Ugandan children from the Acholi people, he is a victim of the nodding syndrome, a mysterious epidemic named after his initial symptoms of a strange stereotypical nod from the head, which has spread throughout northern Uganda along with brutal armed rebellions.
Some imagine viruses are playing, like measles. Others think there are some poisons in food, air, or water. There seems to be a strong connection with parasitic worms that cause river blindness, which is transmitted to humans through the bite of a fly. But none of these theories produce convincing evidence.
Notice the obvious paradox
Although rare and mysterious, Nodding Syndrome can also be a clue to other human diseases that have more universal risks and concerns. Thanks to the pioneering research of the head of Ontario forensic pathologist Michael Pollanen, a man better known for his testimony in the courtroom about physical trauma to victims of Canadian murder, this largely neglected tropical suffering began to highlight the age-related nature of dementia and Alzheimer's. .
"Pay attention to the obvious paradox. Teenagers. In an epidemic. In northern Uganda. Dying from a neurodegenerative disease, "Pollanen said at a small hearing at the University of Toronto's Health Sciences Building. "This is truly extraordinary."
That is, they are dying of illness which, in other places, is exclusive to parents. There are rare exceptions, such as in congenital genetic diseases. But if that happens here, parents will also get sick. But adults don't get Nodding Syndrome.
"Why aren't parents affected?" Pollanen said. The answer, he believes, is that some convergence of genetic and environmental factors cannot be explained. This is the third outbreak like this in Africa, after one in Tanzania in the 1960s and one in South Sudan in 1998.
Nodding Syndrome is a confusing epidemic, strangely oriented in space and time. It is currently only in very poor and unsafe environments in villages and subsistence farming camps for displaced people in Uganda's northern border region near South Sudan. It only affects children between five and 15, peaking around the age of 11.
The beginning coincided precisely with the social turmoil that occurred during the 1990s and early 2000s when warlord Joseph Kony and Lord's Resistance Army raged in this troubled part of Central Africa, kidnapping children as slaves or sex soldiers , displacing millions of people, and committing crimes against humanity. which led to the accusation of Kony in absentia by the International Criminal Court.
The epidemic was first discovered in 2003, at the height of the LRA rebellion, in a camp for internally displaced people in the Kitgum district. Children will first display a classical nod, which is considered a seizure attack, or general muscle loss. From there, the progress of the disease is cruel and fast, through severe intellectual disruption, in many cases, great grand mal seizures. It seems almost always fatal.
At the next stage, the victim is mute, some are paralyzed. Some have more symptoms like Parkinson's. Some have reddish frontal lobe syndrome which causes strange behaviors such as praying loudly without stopping or constant genital touch, Pollanen said.
Secondary injuries increase the number of victims of the disease in a dangerous camp environment for internally displaced persons. Victims accidentally caught fire by cooking charcoal, sank in open water, injured themselves, or were subjected to helpless sexual violence, Pollanen said.
The first and most famous theory is built on strong epidemiological relationships with onchocerciasis, a common parasitic infection of worms that can cause skin problems, blindness, and possibly affect the brain.
This theory has two versions. Whether the worm actually enters the brain, or there are some indirect mechanisms used by the human body in what Pollanen calls the "molecular mimicry" of a substance from the worm, creating something like an autoimmune disease.
Nodding Syndrome is a confusing epidemic, strangely oriented in space and time
Another theory is that Nodding Syndrome is a lingering effect of infection by the measles virus, which leads to neurodegenerative disease. Maybe this epidemic is like the Awakenings movie plot, where infection with influenza viruses caused years later a disease known as post-encephalitis Parkinsonism.
Pollanen said the U.S. Disease Control Center The US, which has a keen eye on emerging diseases, investigated the Nodding Syndrome and was "clear" to him that they did not find everything that could be found in the brains of dissected victims.
Pollanen has studied the brains of five fatal cases, all of whom died in 2014, including some of the same studied by the CDC. One typical case, a 14-year-old girl, said to be neglected, malnourished, wasted, dehydrated, with various healing wounds, which eventually died from dehydration and malnutrition.
The main finding is there is no evidence of worm infection, and there are no signs of viral infection.
A common factor, as explained by Pollanen and colleagues in a new paper, is the neurofibrillary tangles, the same typical brain lesions that play a central role in Alzheimer's.
He asked doctors and epidemiologists about how these abnormalities exist in the frontal cortex, and the answer came back, "memory and cognitive impairment." Likewise, what if they are in the brain stem? It will be displayed as Parkinsonism. The symptoms reflect the tangled distribution in the brain.
This is scientific excitement, that Nodding Syndrome somehow "recapitulates" Alzheimer's events, but in children.
Pollanen said there might be some clues here about the causal relationship between age-related plaques in brain gray matter and tangles in neurons. He described many questions left. It is not clear, for example, why children born later in the birth order are far less likely to be affected.
He plans to return to Uganda to investigate and observe later stages, aged 15 to 22 years, funded by Raymond Chang's foundation, because his work is too speculative for traditional funding institutions.
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