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Mysterious, deadly epidemic in children in Uganda could provide the key to Alzheimer's disease

The young teenager in the photo can not stand alone. He is kept on his feet in front of a thatched-roof hut in northern Uganda. His face is hidden for solitude, but it is obvious how the muscles of his legs disappeared. He looks a bit like an old man.

Like thousands of other Uchandan children of the Acoli people, he is a victim of Kivania syndrome, a mysterious epidemic named after its early symptom of a strange stereotypical nodding of the head that spread throughout northern Uganda at the threshold of a violent armed insurrection.

Some suggest that the virus plays a role, such as measles. Others think there are toxins in food, air or water. Apparently, there is a strong connection with the parasitic worm, which causes river blindness, which is transmitted to humans through the bite of flies. But none of these theories gave convincing evidence.

Note the obvious paradox.

No matter how rare and mysterious it may be, Nodding's syndrome can also be the key to another human disease, which represents a more universal risk and anxiety. Thanks to the pioneering research of Ontario’s Chief Forensic Pathologist, Michael Pollanen, a man better known for his testimony in the courtroom about the physical trauma of Canadian homicide victims, this largely forgotten tropical disease begins to shed light on the nature of dementia associated with age and Alzheimer's disease,

“Pay attention to the obvious paradox. Teenagers. In the epidemic. In northern Uganda. Dying from a neurodegenerative disease, ”Pollanen told a small audience at the University of Toronto Health Science Building. "This is absolutely wonderful."

He meant that they were dying from diseases that are spreading everywhere on the elderly. There are rare exceptions, as with hereditary genetic diseases. But if this is what is happening here, the parent will also be sick. But in adults there is no nodding syndrome.

“Why are parents not affected?” Said Pollanen. The answer, in his opinion, lies in some inexplicable convergence of genetic and environmental factors. This is the third outbreak, like this one in Africa, after one in Tanzania in the 1960s and one in South Sudan in 1998.

Nodding syndrome is a mysterious epidemic, strangely oriented in space and time. Today it exists only in extremely poor and unsafe conditions in villages and camps for subsistence farmers in the northern border areas of Uganda near South Sudan. This applies only to children between the ages of five and 15 years old, who have reached a peak at the age of about 11 years.

Its beginning coincided exactly with the social upheavals that arose in the 1990s and early 2000s, when commander Joseph Kony and his Lord’s Resistance Army raged in this troubled part of Central Africa, kidnapping children as a sex slave or soldier, moving millions and committing crimes against humanity. this led to Kony’s absentee accusations by the International Criminal Court.

A 14-year-old Ugandan girl suffering with a nod.

Stephen Wandera / AP, File

The epidemic was first discovered in 2003, at the height of the LRA insurgent movement, in an internally displaced persons camp in Kitgum. At first, the children showed a classic nod, which was considered an atonic attack, or a general short-term loss of muscle tone. From there, the progression of the disease is brutal and rapid, due to a serious mental disorder, in most cases, due to full-blown major attacks. It seems almost always deadly.

In the later stages, the victims are mute, some are paralyzed. For some, symptoms are more like parkinsonism. According to Pollanen, some people have the syndrome of a sluggish frontal lobe, which causes strange behavior, such as unceasing prayer or constant touching of the genitals.

Secondary injuries increase the number of diseases in the dangerous conditions of camps for internally displaced persons. According to Pollanen, victims are accidentally burned as a result of a coal fire, drown in open water, are injured when they fall, or become helpless objects of sexual violence.

The first and most well-known theory was built on a strong epidemiological connection with onchocerciasis, a common parasitic worm infection that can cause skin problems, blindness, and perhaps somehow affect the brain.

This theory had two versions. Either the worms actually penetrated the brain, or there was some indirect mechanism by which the human body is involved in what Pollanen called the substance from the worm as "molecular mimicry", creating something like an autoimmune disease.

Nodding syndrome is a mysterious epidemic, strangely oriented in space and time

Another theory was that Nodding's syndrome was a lasting effect of measles virus infection, leading to a neurodegenerative disease. Perhaps this epidemic was similar to the plot of the film "Awakening", in which infection with the influenza virus after many years led to a disease known as post-encephalitis parkinsonism.

Pollanen said that the US Centers for Disease Control, which are closely monitoring the emerging diseases, investigated Nodding's syndrome, and it was "obvious" for him that they did not find everything that could be found in the dissected brain of the victims.

Pollanen studied the brain of five deaths, all of which died in 2014, including some of those that were studied by the CDC. One typical case is a 14-year-old girl, who was said to be unkempt, undernourished, exhausted, dehydrated, with multiple healing traumas, who eventually died of dehydration and malnutrition.

The key finding was no signs of worm infection or signs of a viral infection.

A common factor, as Pollanen and his colleagues describe in the new article, are the neurofibrillary tangles, the very distinctive brain lesions that play a central role in Alzheimer's disease.

Dr. Michael Pollanen, Chief Forensic Pathologist, Ontario

Jeff Robins / Postmedia / File

He asked his audience of doctors and epidemiologists how it would look if these tangles were in the frontal cortex, and the answer came back: "memory and cognitive impairment." Similarly, what if they were in the brain stem? It would show as parkinsonism. Symptoms reflect the distribution of tangles in the brain.

It is a scientific disturbance that Nodding's syndrome somehow “repeats” the events of Alzheimer's disease, but in children.

Pollanen said that there could be some clue about the causal relationship between age-related plaques in the gray matter of the brain and tangled neurons. He described many of the remaining questions. For example, it is unclear why children born later in birth order are subject to a much lower probability.

He plans to return to Uganda to investigate and observe cases at a late stage, between the ages of 15 and 22, funded by the late Raymond Chung, because the work is too speculative for traditional funding agencies.

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