Neurodegenerative Disease: Youth/Elderly

"Notice the obvious paradox. Teenagers. In an epidemic. In norther Uganda. Dying of a neurodegenerative disease."
"It's totally remarkable. Nodding syndrome is a tauopathy. Our hypothesis is that nodding syndrome is a neurodegenerative disease, like Alzheimer's."
Michael Pollanen, chief forensic pathologist, Ontario

A 2012 image from Uganda shows an 11-year-old boy suffering from nodding syndrome.

JAMES AKENA/REUTERS

Nodding Syndrome is recognized as rare and as a disease whose origins are still unknown, mysterious. Understanding it, however, may give medical science a clue to origins and patterns recognized in Alzheimer's Disease. And Ontario's chief forensic pathologist, Michael Pollanen has produced some pioneering research that has been published recently in the journal Acta Neuropathologica. His insight may lead to a new understanding of the nature of age-related dementia and Alzheimer's; one of the oddities here is that Nodding Disease is a neurodegenerative disease that victimizes children, quite the opposite age spectrum that Alzheimer's strikes.

What Dr. Pollanen found remarkable, is that children were dying of an affliction that everywhere else in the world has symptoms that exclusively strike the elderly, with rare exceptions. What is also noted is that while children are being struck down by this degenerative attack on their neural system, their parents remain unaffected by Nodding Syndrome. "Why are the parents not affected?", asks Dr. Pollanen and the answer to that may very well answer a great deal more, with respect to the much broader worldwide issue of Alzheimer's Disease.

He has a scientist's educated hypothesis based on what he already is aware of that a convergence of genetic and environmental factors may be found to be at play. One such epidemic of Nodding Syndrome broke out in the 1960s in Tanzania, and in South Sudan in 1998. Now it has become an epidemic in Northern Uganda; all African nations, all countries that have been roiled by brutal conflicts, by issues of malnutrition and desperate existential fear.

Where it strikes is in impoverished and insecure environments, where subsistence-farming villages have become susceptible to its mysterious onset, along with those languishing in camps for internally displaced people. Only children between around five and fifteen years of age are affected; the disease peaks around the age of eleven. Societal turmoil appears to unleash its onset; in central Africa when the Lord's Resistance Army abducted children to be trained as soldiers or act as sex slaves.

The epidemic first surfaced in a camp for internally displaced persons in 2004 when children would initially display classical nodding, mistaken for atonic seizures; a generalized brief loss of muscle tone that degenerated to severe intellectual impairment, grand mal seizures and death. Victims become mute, some paralyzed in its later stages -- while others yet exhibit Parkinsonism-like symptoms.

These physical and mentally vulnerable children then become victim in a perilous environment of camps to being accidentlally burned in charcoal cooking fires, drowning in open water, injured when falling from heights, or they become the helpless victims of sexual violence. Strong epidemiological association with a common parasitic infection of a water-borne worm that can cause skin problems, blindness and affects the brain has been theorized.

As another theory would have it, Nodding Syndrome represents a lingering effect of the measles virus infection, leading to a neurodegenerative disease.

According to Dr. Pollanen, the U.S. Centers for Disease Control investigated Nodding Syndrome but it appears found no significant answers. It appeared that the agency found nothing in the dissected brains of the victims, leaving room for more intensive investigation. The brains of five fatal cases were studied by Dr. Pollanen; a typical 14-year-old girl had been malnourished, wasted, dehydrated, had multiple healing injuries and died of dehydration and malnutrition.

In their newly published paper, Dr. Pollanen and his colleagues describe a neurofibrillary tangle; distinctive brain lesions that are seen as playing a central role in Alzheimer's. In addressing an audience of doctors and epidemiologists how it would appear if these tangles were in the frontal cortex, the response was: "memory and cognitive impairments". And if the tangles were in the brain stem? Parkinsonism.

The general consensus reached is that Nodding Syndrome "recapitulates" in a manner not yet understood let alone proven -- in children, not adults, the type of neurodegenerative disease typically found in adults worldwide. Tangles in the neurons, age-related plaques in the brain's grey matter, all somehow appear to figure into the equation. What is also unclear is why it is that children born later in birth order are less likely to be affected.