Nodding sickness

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The nodding disease (also: nodding syndrome , English nodding disease or nodding syndrome ) is a so far little researched disease that was first described in the 1960s in Sudan . It is a fatal, neurologically progressive disease that occurs almost exclusively in children and causes mental and physical disabilities . Most of those affected are between five and 15 years old, and adults are occasionally affected. Currently, the spread of the disease is limited to small regions in South Sudan , Tanzania and northern Uganda . Even before it appeared in South Sudan, the disease had been described in remote mountain regions of Tanzania in 1962, and only later were both clinical pictures seen as expressions of one and the same disease.

Symptoms

The symptoms of Nick's disease are very characteristic. When a child becomes ill with it, growth and development come to a standstill and, over time, mental retardation . The name of the disease can be traced back to the characteristic "nodding attacks" of the sick. These often begin when the child is eating or seeing traditional food, and rarely are they caused by the cold. However, the seizures do not occur with unusual foods such as chocolate or non-traditional foods. The seizures are often very strong, but only brief and end as soon as the child stops eating, turns their eyes or is warm again. The neurotoxicologist Peter Spencer, who investigated the disease, found that as soon as the affected person is offered food, "one or two children begin to nod very quickly and pendulously [...], some are cold". Particularly strong seizures can lead to collapse , which leads to further damage. Subclinical seizures were identified in electroencephalograms, MRI examinations showed severe atrophy of the brain and damage to the hippocampus and glial cells .

The seizures often cause the children to fall and seriously injure themselves. They also fall into open fires and on pointed objects. They also become disoriented and get lost in the forest, where further dangers lurk. At some point, many legal guardians begin to tie the children to trees and furniture.

Diagnosis and treatment

The diagnosis is mainly based on the characteristic "nodding attacks" as well as growth and mental retardation. In the future, neurological examinations could also be used for diagnosis. There is no known effective therapy. Anticonvulsants are used. In addition, drugs against malaria were used, the effects of which have not yet been precisely documented.

forecast

The disease is associated with physical and psychological weakness. In 2004, Peter Spencer stated: "It has been reported to be a progressive and fatal dysfunction, possibly lasting three or more years." Although some children are reported to have recovered from the illness, most die from it.

According to the Ugandan Daily Monitor , 165 out of 532 children enrolled in Aromo Wanglobo's primary school in 2011 had the disease. 12 died and 73 had to drop out of school.

Possible triggers

The cause of the disease is still unknown. Based on the epidemiological characteristics, it is assumed that it is an infectious disease or a disease with a parainfectious cause (e.g. an autoimmune process triggered by an infection ). Another possible explanation would be chronic poisoning. There are hypotheses that the parasitic nematode Onchocerca volvulus , which is common in all regions where the disease occurs, plays a role. O. volvulus is transmitted by the black fly and is the cause of onchocerciasis (river blindness). In 2004, most children with Nick Disease lived near the Yei River, a hotbed of river blindness, and 93% of them carried the nematode - a much higher percentage than children without Nick Disease. Earlier it was suggested that there is a connection between river blindness and normal epileptic seizures or growth retardation, but this has not yet been proven. About the link between O. vovulus and Nick's disease, Scott Dowell, investigator for the US Center for Disease Control and Prevention (CDC), said, "We know [Onchocerca volvulus] is involved in some way, but it is a bit puzzling that [the worm] is also fairly common in regions unaffected by Nick's disease. " Andrea Winkler, the first author of a 2008 Tanzanian study, said of the link: "We couldn't find any evidence that O. volvulus actually penetrates the brain, but we cannot rule out that there is an autoimmune mechanism that is present during the Disease expires. " In the worst affected region of Uganda, the infection rate of children with epilepsy or Nick's disease with microfilariae fluctuates between 70 and 100%. The CDC is investigating a possible link to wartime chemical exposure. The team is also investigating whether a deficiency in vitamin B 6 ( pyridoxine ) could be a trigger for the disease, as there is a widespread deficiency of this vitamin among patients and parallels to pyridoxine-dependent epilepsy.

Distribution and Epidemiology

Distribution area of ​​Nick's disease in South Sudan on the border with Uganda:
  • Spread in 2001
  • additional distribution in 2011
  • sporadic occurrence in 2011

    • Although the disease first appeared in mountainous regions, the disease is now spreading in densely populated river plains. According to a WHO study published in 2001 and 2002 only in 2012, the city of Lui (Mundri County, Western Equatoria State in the Republic of South Sudan ) had a prevalence of 2.3% and the village of Amadi a prevalence of 6.7%. The settlements are close to the Yei River; it flows past two kilometers from Lui and less than a kilometer from Amadi.

    Currently, Nick's disease is mainly prevalent in South Sudan, with around 300 cases in 2003 in Mundri County alone . By 2008, it spread across the border to Uganda , where, according to the Ugandan ministry, at least 2000 children fell ill in 2011. At the end of 2011, Nick's disease occurred mainly in Kitgum , Pader and Gulu - over 1,000 cases were reported in the second half of 2011 alone. In early 2012, there were further outbreaks in Tanzania, South Sudan and Uganda. The spread of the disease is likely to get worse in the future due to poor hygiene .

    literature

    • Gretchen Vogel: Mystery Disease Haunts Region. In: Science. April 13, 2012, pp. 144-146.

    Web links

    Individual evidence

    1. ↑ Nick Syndrome: The Curse of the Blackfly in Northern Uganda. In: derstandard.at . Retrieved December 14, 2012 .
    2. ↑ M. Lacey: Nodding disease: mystery of southern Sudan. In: The Lancet Neurology . 2003; 2 (12), p. 714. doi : 10.1016 / S1474-4422 (03) 00599-4 . PMID 14649236 .
    3. ↑ a b c d APA: Mystery Deadly Nick Disease. on: derStandard.at , August 7, 2012.
    4. ↑ UGANDA: Nodding disease or "river epilepsy"? IRIN Africa, August 10, 2009, accessed October 19, 2010 .
    5. ↑ a b "Nodding Disease in East Africa". CNN, June 5, 2011, accessed April 5, 2012 .
    6. ↑ a b c d e Meredith Wadman: African outbreak stumps experts. In: Nature. 2011; 475 (7355), pp. 148-149. doi: 10.1038 / 475148a . PMID 21753824
    7. ↑ a b Andrew Harding: 'Nodding disease' hits Sudan. In: BBC News. September 23, 2003, accessed April 5, 2012 .
    8. ^ World Health Organization joins other partners to support Nodding Disease investigations in Southern Sudan. World Health Organization, 2012, accessed April 5, 2012 .
    9. ↑ a b c d Emma Ross: Sudan A Hotbed Of Exotic Diseases. In: CBS News (Rumbek, Sudan). February 3, 2004, archived from the original on February 18, 2004 ; accessed on April 5, 2012 .
    10. ↑ Emma Ross: Bizarre Illness Terrifies Sudanese. CBS News, February 11, 2009, accessed April 5, 2012 .
    11. ↑ Richard Idro, Hanifa Namusoke, Catherine Abbo, and others. a .: Patients with nodding syndrome in Uganda improve with symptomatic treatment: a cross-sectional study. In: BMJ Open. No. 4, 2014, e006476, doi : 10.1136 / bmjopen-2014-006476 ( full text online ).
    12. ↑ a b c Abraham, Curtis (December 23, 2011): Mysterious nodding syndrome spreading through Uganda. In: New Scientist. Retrieved December 25, 2011.
    13. ↑ Lekshmi Santhosh: When Nodding Means Dying: A baffling new epidemic is sweeping Sudan. In: The Yale Journal of Public Health. 2004, archived from the original on May 19, 2006 ; accessed on April 5, 2012 .
    14. ↑ M. Druet-Cabanac, M. Boussinesq, L. Dongmo, G. Farnarien, B. Bouteille, PM Preux: Review of epidemiological studies searching for a relationship between onchocerciasis and epilepsy. In: Neuroepidemiology. 2004; 23 (3), pp. 144-149. doi: 10.1159 / 000075958 . PMID 15084784 .
    15. ↑ E. Ovuga, W. Kipp, M. Mungherera, S. Kasoro: Epilepsy and retarded growth in a hyperendemic focus of onchocerciasis in rural western Uganda. In: East African medical journal. 1992; 69 (10), pp. 554-556. PMID 1473507 .
    16. ↑ B. Marin, M. Boussinesq, M. Druet-Cabanac, J. Kamgno, B. Bouteille, PM Preux: Onchocerciasis-related epilepsy? Prospects at a time of uncertainty. In: Trends Parasitol. 2006; 22 (1), pp. 17-20. doi: 10.1016 / j.pt.2005.11.006 . PMID 16307906 .
    17. ↑ a b J. K. Tumwine, K. Vandemaele, S. Chungong, M. Richer, M. Anker, Y. Ayana, ML Opoka, DN Klaucke, A. Quarello, PS Spencer: Clinical and epidemiologic characteristics of nodding syndrome in Mundri County, southern Sudan. In: African Health Sciences. Vol. 12, No. 3 (Sept.), 2012, pp. 242-248.
    18. ^ East African Mystery Disease: Nodding Syndrome. In: Daily Kos. March 14, 2012, accessed March 14, 2012 . or as pdf Meredith Wadman: African outbreak stumps experts. In: Nature. Vol. 475, July 14, 2011 (PDF; 924 kB)