The Chilling Case of Nodding Syndrome

An outbreak of a creepy, mysterious illness is quietly spreading among children and adolescents in Eastern Africa – leaving scientists stumped. Previously healthy children are falling prey to a debilitating disease that deprives them of their childhood, and is devastating hundreds of families – a condition now known as nodding syndrome.

As the name suggests, the syndrome’s hallmark symptom is a peculiar seizure-like repetitive nodding of the head towards the chest triggered by the sight of food or cold weather. The sight of food becomes unbearable and eating can become a harrowing task. Nodding episodes, which last several minutes, occur from few times a week to several times a day, depending on the severity of the disease; during the episodes, children become unaware of their surroundings and lost in their own world.

Eventually, the disease leaves them mentally and physically disabled. Their mental capacities slowly decline, they stop going to school, their growth is stunted, they become malnourished, their arms and legs wither, and they may lose their ability to communicate; ultimately, they die – either through malnutrition since they cannot eat or by accidents such as drowning, burning, or falling due to the seizures.

Two boys of the same age, but the shorter one has nodding syndrome.  Image: (Tumwine et al., 2012)

Two boys of the same age, but the shorter one has nodding syndrome.
Image: Tumwine et al., 2012

But this disease isn’t new; cases were reported as far back as the 1960s in Tanzania, but it wasn’t until the 1990s that it gained attention among local authorities, and until the turn of the century when the World Health Organization stepped in. Since 2009, Ugandan officials sought help from the CDC in USA to determine the cause. Scientists formulated standardized case definitions only in 2012, which also include videotaped recordings of the nodding episodes.

Using the newly defined cases, hundreds of children are probably affected, but thousands of cases were reported by the local media in Uganda alone. Nearly all cases are centered in rural areas of three countries: southern Tanzania, northern Uganda, and South Sudan. The bad news is that cases are rising in Uganda and South Sudan over the past few years. Curiously, most cases are among internally displaced people – who relied on refugee camps for food and shelter, escaping the wrath of the Lord’s Resistance Army – and poor tribes, which suffered from lack of food prior to the outbreak of nodding syndrome.

Notably, this little-known disease has affected more people than the widely-reported outbreaks of Ebola did.

Despite numerous studies exploring a multitude of possible risk factors, from exposure to environmental toxins and war munitions to consumption of baboon brains and red sorghum, the causes are still a mystery. Even modern brain imaging and electrical monitoring techniques failed to provide clues.

But there is a common link: Most patients live near a river where a disease known as river blindness (also called onchocerciasis) is prevalent. This disease is caused by infestation of a parasitic worm Onchocerca volvulus, which is carried by black flies and transmitted by their bites. Several studies, mostly in Uganda, have reported a large proportion of nodding syndrome children are infested by the parasitic worm compared with unaffected children using skin snip specimens. A recent study by Foltz and colleagues in Uganda’s Kitgum district has confirmed this association using serological studies for the first time; they found that a higher proportion of nodding syndrome sufferers have antibodies against the parasitic worm. So, the worm does appear to play a role, but scientists are baffled as river blindness is also present in areas where nodding syndrome is not. Also, most of these studies involved fewer than 50 patients.

The green areas show countries where river blindness was eradicated.  The beige areas are countries where programs to combat river blindness are ongoing.  The red circles delineate areas where nodding syndrome is reported.  Image Source: (Dowell et al, 2013)

The green areas show countries where river blindness was eradicated.
The beige areas are countries where programs to combat river blindness are ongoing.
The red circles delineate areas where nodding syndrome is reported.
Image: Dowell et al, 2013

Another possible link is vitamin B6 deficiency. Initial studies had associated vitamin B6 deficiency among nodding syndrome children. Foltz and colleagues also noticed that more nodding syndrome children had this deficiency, though it was also present in large numbers of unaffected children. In Uganda, clinical trials will take place to see if high doses of vitamin B6 may help these children.

Foltz’s team also found an interesting association: Consumption of crushed roots for traditional medicine among nodding syndrome children was more common than in unaffected children. They suspected cyanide toxicity from cassava roots, commonly consumed in the region, but were unable to find evidence upon further testing.

One strange observation, noted by researchers is that the sight of non-familiar foods such as a western chocolate bar, for example, did not trigger head nodding.

Sadly, no cure exists, and children are treated with anti-epileptic drugs, but their effects are limited.

Caring for children with this dreadful disease places a huge burden on parents who are already afflicted by abject poverty. They often have to leave their jobs in the fields as these children require constant care and without working they sink deeper into poverty. In one case, a heart-breaking video and report reveals how a father reluctantly resorts to tying his 12-year old daughter, Nancy Lamwaka, to a tree for 13 hours daily to prevent her from wandering off or from falling due to violent nodding episodes. Another report sheds light on the plight of two teenage siblings, one epileptic and the other suffering from nodding syndrome – and is unable to sit and talk – that were abandoned by their parents.

Hens-try-to-poke-her-rotting-wounds

12-year old Lamwaka is tied to a tree and is unable to eat her meal. She leaves her plate on the side, which the chickens are eyeing for lunch.
Image: The Observer

What’s worse, these children have to face stigma from society. Even children that have somewhat improved in government health centers are ostracized when they return to their villages and school. Upon returning, a 15-year-old sensed that his friends and teachers are afraid of being around him and classmates call him “demon-possessed”.

Lamwaka is left all alone; her siblings stay away from her, afraid they might catch the disease.
Image: The Observer

On the positive side, a charity, Hope for Humans, initiated by two American doctors is helping nodding syndrome children by running a rehabilitation center – the only one in Gulu district in northern Uganda – that gives them specialized education, daily balanced nutritious meals, proper hygiene, psycho-social support, along with anti-epileptic drugs. This approach is showing promising results: The children are able to talk and play – feats they were unable to accomplish before. They still have nodding episodes, but less frequently. Addressing these basic needs is the best strategy to alleviate their symptoms and provide them with a better, more meaningful life by integrating them into society until effective treatment emerges. But some parents simply cannot afford the transportation to send their children to these centers.

Delving deeper into the underlying causes of the syndrome requires large-scale studies. Perhaps new cases in previously unknown locations may give some clues. But challenges lie ahead in the crusade against nodding syndrome. Corruption among officials is not only hampering research efforts but also delaying treatment and food for the children, and training of adequate staff to treat the children. The National Coordinator of the Nodding Syndrome response in Uganda, Dr. Bernard Opar Toliva claims that the money allocated to assess the prevalence of the disease never even reached his office. A recent report claims government health centers in Udanga’s districts are urging for more funding as they are running out of food and drugs to feed and treat the children.

There is an urgent need to educate the public on nodding syndrome to dispel their fears so that children suffering from the disease are not further tormented by stigmatization. Psychological counseling also needs to be provided when treating the children to ensure they can smoothly integrate back into society.

While effective treatment is still a far cry, the well-being of these neglected children lies in fulfilling their basic needs through nutritious meals, an education, and social support. These may be the only hope in bringing back their long-lost smiles.

References

Dowell SF, Sejvar JJ, Riek L, Vandemaele KAH, Lamunu M, Kuesel AC, et al. Nodding syndrome. Emerg Infect Dis [Internet]. 2013 Sep. DOI: 10.3201/eid1909.130401

Foltz JL, Makumbi I, Sejvar JJ, Malimbo M, Ndyomugyenyi R, et al. (2013) An Epidemiologic Investigation of Potential Risk Factors for Nodding Syndrome in Kitgum District, Uganda. PLoS ONE 8(6): e66419. doi:10.1371/journal.pone.0066419

Tumwine JK, Vandemaele K, Chungong S, Richer M, Anker M, Ayana Y, et al. Clinical and epidemiologic characteristics of nodding syndrome in Mundri County, southern SudanAfr Health Sci. 2012 September; 12(3): 242–248.
Video – IRIN Humanitarian News and Analysis: Nodding Syndrome 
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