THE STRANGE AND LITTLE KNOWN DISEASE WHOSE CAUSE NO ONE KNOWS

Okot Nyormoi is Associate Editor of Nile Journal 
and is the author of the novel Burden of Failure

The year was 1960, it was the first ever report of a strange and mysterious disease characterized by constant nodding of the head in affected individuals, all of whom were children. Adults appeared to be immune to the new disease! The report located the disease in a remote part of southern Tanzania, a country known at the time as Tanganyika.

Years later the disease showed up in war ravaged South Sudan. And then in the 1990s the disease arrived in northern Uganda then in the throes of a war that uprooted millions and put them in concentration camps under appalling conditions. In 2010 the nodding disease showed up in Democratic Republic of the Congo, a country that for a long time has been constantly in a state of war.

Tanzania had not and has not experienced war. Nevertheless, all three locations where the disease occured including the one in Tanzania, shared one thing in common, abject poverty. In Northern Uganda, South Sudan, and the DRC, war had brought severe poverty.     

When nodding disease syndrome (NS) as it became known, appeared in South Sudan and later in northern Uganda, the chase began to find the cause. Doctors looked for factors such as nutritional deficiencies, poison from contaminated food, chemical contamination from munitions, virus infection, but to no avail. The observation that the areas where NS occurred overlaps with that of the parasite Onchocerca volvulus, the parasite that causes river blindness, led to speculation that, the parasite causes Nodding Syndrome.

However, studies showed that only 54% of children with Nodding Syndrome, tested positive for Onchocerca volvulus infection. Moreover, children in other parts of Uganda and other places where there were no NS, also tested positive for parasite Onchocerca volvulus. Northern Uganda, in the past, a prosperous region, never had cases of Nodding Syndrome till the war and made people poor.

Samples from Nodding Syndrome patients were sent to the US for analysis. Results were not conclusive. Crystal deposits and tau protein associated with Alzheimer’s disease were found in the brain of NS patients. However, these observations are not compelling enough to establish a causal link between the parasite and Nodding Syndrome.

Next researchers examined the process by which a person’s own immunity attacks own. A promising lead was found in a brain protein (leiomodin-1) similar to proteins found in Onchocerca volvulus. This led to the speculation that NS is an autoimmune disease. Because it could not explain why only children and not adults got NS, the idea was soon dropped.

Most recently, attention turned to the bacterium, Wolbachia, which mutually co-exists Onchocerca volvulus, as they need each other to reproduce. It was thought Wolbachia induces autoimmune attacks in the brains of NS victims. Again, this idea was dropped because adults as well as certain children affected with the parasites do not develop NS. Nevertheless, the suspicion is being tested by treating NS patients with doxycycline, an antibiotic that kills the bacterium Wolbachia. If the bacterium is the cause of NS, treating patients with the antibiotic should cure patients of the disease. So far, no results have been published. While we wait for the outcome of the treatment of patients with doxycycline, I predict that the results will be the same as the other outcomes that in the end came to nothing.

The elusive cause of NS may lie in one common denominator, abject poverty which forces people to do whatever possible to overcome the scarcity of food, medicine, beverages and physical safety. They often end up eating and drinking whatever they can to survive. In the case of South Sudan and northern Uganda, pregnant women must have been exposed to something that affected the development of their fetuses at the specific point in time. Although the babies were born normal, whatever affected them did not manifest itself until they were 11-18 years old.

This possibility was dismissed because nobody found traces of poison in wild or cultivated plants consumed as food or contaminated food distributed by humanitarian organizations in the specific locations. Unfortunately, it is impossible to discover such a substance if the specific batch of the offending food, medicine or munition, is no longer available for analysis.

Recently 262 people in Karamoja in north eastern Uganda fell sick and a three died from eating porridge made from contaminated flour distributed by the World Food Program. The similarity here with NS is food insecurity requiring distribution of imported food. The difference is that the symptoms appeared almost immediately, allowing for food and water samples to be tested. Even so, to protect the integrity of the food suppliers, nobody may admit responsibility. To make matters worse, results of many investigations in failed states are rarely released to the public in the name of protecting the good name of the country or organization. Consequently, we may never know the true cause of NS. No results have been released yet.

There has been at least one situation in history where a drug, thalidomide, affected fetal development and the effect remained unknown till birth. The wonder drug was developed in the 1950s and was fashionably used, among other things, to treat morning sickness in pregnant women. To the horrors of users, it was revealed in 1961 that women who took it during pregnancy were giving births to babies with various malformations depending on when in pregnancy the fetus was exposed to thalidomide. Fortunately, the drug affected wealthy and influential people who forced the German pharmaceutical company, Grunenthal, to admit its responsibility for the horrific side effects.

Could NS be another thalidomide-like substance that selectively affects developing human embryos? Anecdotal evidence suggests that the possibility should not be discounted. If so, the culprit is most likely not a natural substance found in the area. Otherwise, its effect would have been known long before the 1960s in Tanzania and the 1990s in South Sudan and northern Uganda. Thus, it appears that the substance was introduced in the community at a time of the horrendous living conditions during wars. No new cases have been reported since the shooting war ended in 2008. Although some people attribute the decline of NS cases in northern Uganda to the bi-annual use of insecticide to control black flies that transmit the river blindness-causing parasite, Onchocerca volvulus, it can equally be attributed to the significant improvement in the living conditions.