The young boy in the picture can not stand alone. He is detained on his feet in front of a roof hut in northern Uganda. His face is hidden for privacy, but it is clear how his leg muscles have been wasted. He looked a bit like an old man.
Like thousands of other Ugandan children of Acholi's men, he is the victim of a nodding syndrome, a mysterious epidemic called an early symptom of a strange stereotypical nod of the head that spread all over northern Uganda in a brutal armed attack.
Some of them visualized a virus that is playing, like measles. Others think there is a toxin in food, air or water. There seems to be a strong link to the parasitic worm that causes blindness of the river, which is transmitted to humans through fly bites. But none of these theories yielded convincing proof.
Notice the obvious paradox
As rare and mysterious as it is, Nodding syndrome may also be a clue to another human disease of universal risk and concern. Thanks to the pioneering study of Ontario's chief forensic pathologist, Michael Polanan, a better-known person in his testimony to the court about the physical trauma of Canadian murder victims, this neglected tropical jolt begins to shed light on the nature of age-related dementia and Alzheimer's.
"Pay attention to the obvious paradox, teenagers, in the epidemic, in northern Uganda, dying from neurodegenerative disease," Pollanan told a small audience at the University of Toronto Health Sciences Building. "It's absolutely amazing."
He meant that they were dying of diseases that everywhere else were exclusive to old people. There are rare exceptions, such as hereditary genetic diseases. But if that's what happens here, even a parent will be ill. But adults do not get nodding syndrome.
"Why are not parents affected?" Said Pollannen. The answer, in his opinion, is an unexplained convergence of genetic and environmental factors. This is the third such outbreak in Africa, after one in Tanzania in the 1960s and one in southern Sudan in 1998.
Nodeding syndrome is an embarrassing epidemic, strangely directed in space and time. It exists today only in the missing and insecure environment of agricultural villages and camps for displaced persons within the northern border regions of Uganda near Southern Sudan. It affects only children between 5 and 15, peaking around the age of 11.
The beginning coincided with the social turmoil of the 1990s and early 2000s, when Connie and his God's Resistance Army raged on this troubled part of Central Africa, kidnapping children as slaves or soldiers, sterilizing millions and committing crimes against humanity that led The indictment of Connie in the absence by the International Criminal Court.
The epidemic was first discovered in 2003, at the height of the LRA uprising, in the camp to the DP camp in Kitgum district. Children were the first to introduce the classic nod, thought to have an atonic seizures, or a general short-term loss of muscle. From there, progression of the disease is cruel and rapid, through severe intellectual impairment, in most cases, seizures are fully filled. It seems almost always fatal.
In later stages, the victims are mute, some paralyzed. There are symptoms more similar to Parkinson's. Some have a frontal lobe syndrome that causes strange behaviors such as incessant prayer or constant contact with the genitals, Poulanan said.
Secondary injuries double the price of the disease in the dangerous environment of the DP camps. Victims are accidentally burned by charcoal fires, drowned in open water, hurt themselves falling, or helpless targets of sexual violence, said Polanan.
The first and most well-known theory was built on the strong epidemiological relationship with Oncissa and caryasis, a common parasite infection of a worm that can cause skin problems, blindness, and possibly affect the brain somehow.
This theory had two versions. Or the worms actually entered the brain, or there was some indirect mechanism in which the human body is involved in what is called a "molecular mimicry" of a substance from the worm, creating something like an autoimmune disease.
Nodeding syndrome is an embarrassing epidemic, strangely directed in space and time
Another theory was that the nodding syndrome is the ongoing effect of measles virus infection, leading to degenerative disease. Perhaps this epidemic was like the plot of the film "Awakening," in which infection with the flu virus led many years later to a disease known as post-encephalitic Parkinson's disease.
According to Polanan, the US Centers for Disease Control, which have a keen eye on developing diseases, have studied the "nodding syndrome" and "seen" that they have not found everything that can be found in the victims' detached minds.
Pollanen examined the brain of five fatal cases, all died in 2014, including some of those investigated by the CDC. One typical case, a 14-year-old girl, was said to be criminal, malnourished, wasted, dehydrated, with multiple healing injuries, which eventually died of dehydration and malnutrition.
A key finding was no evidence of worms, and no signs of viral infection were found.
The common factor, as Fallonen and colleagues describe it in a new article, is a neurofibrillary complication, the same unique lesions in the brain that play the central role in Alzheimer's.
He asked his doctors and epidemiologists what it would look like if these tangles were in the frontal cortex, and the answer was back, "Memory and cognitive difficulties." Also, what if they were in the brain stem? It would look like Parkinson's. Symptoms reflect the distribution of complications in the brain.
This is the scientific excitement that Nording syndrome somehow "sums up" the events of Alzheimer's, but in children.
Poulanan said that there might be some clue to the causal link between age-related plaque in the gray matter of the brain and the tangle of neurons. He described the many questions left. It is not clear, for example, why children born later in the birth order are much less affected.
He intends to return to Uganda to investigate and examine cases at a later stage, aged 15-22, funded by the late Raymond C. Chang Foundation, as the work is too speculative for traditional funding agencies.