Volume 93, Issue 1
  • ISSN: 0002-9637
  • E-ISSN: 1476-1645



Nodding syndrome (NS) is a poorly understood condition, which was delineated in 2008 as a new epilepsy syndrome. So far, confirmed cases of NS have been observed in three circumscribed African areas: southern Tanzania, southern Sudan, and northern Uganda. Case–control studies have provided evidence of an association between NS and infection with , but the causation of NS is still not fully clarified. We report a case of a 15-year old boy with head nodding seizures and other characteristic features of NS from an onchocerciasis endemic area in western Uganda, with no contiguity to the hitherto known areas. We suggest that the existence of NS should be systematically investigated in other areas.


Article metrics loading...

The graphs shown below represent data from March 2017
Loading full text...

Full text loading...



  1. Aall-Jilek LM, , 1965. Epilepsy in the Wapagoro tribe in Tanganyika. Acta Psychiatr Scand 41: 5786.[Crossref] [Google Scholar]
  2. Winkler AS, Friedrich K, König R, Meindl M, Helbok R, Unterberger I, Gotwald T, Dharsee J, Velicheti S, Kidunda A, Jilek-Aall L, Matuja W, Schmutzhard E, , 2008. The head nodding syndrome—clinical classification and possible causes. Epilepsia 49: 20082015.[Crossref] [Google Scholar]
  3. Lacey M, , 2003. Nodding disease: mystery of southern Sudan. Lancet Neurol 2: 714.[Crossref] [Google Scholar]
  4. Tumwine JK, Vandemaele K, Chungong S, Richer M, Anker M, Ayana Y, Opoka ML, Klaucke DN, Quarello A, Spencer PS, , 2012. Clinical and epidemiological characteristics of nodding syndrome in Mundry County, southern Sudan. Afr Health Sci 12: 242248. [Google Scholar]
  5. Sejvar JJ, Kakooza AM, Foltz JL, Makumbi I, Atai-Omoruta AD, Malimbo M, Ndyomugyenyi R, Alexander LN, Abang B, Downing RG, Ehrenberg A, Guilliams K, Helmers S, Melstrom P, Olara D, Perlman S, Ratto J, Trevathan E, Winkler AS, Dowell SF, Lwamafa DKW, , 2013. Clinical, neurological and electro-physiological features of nodding syndrome, Uganda: an observational case series. Lancet Neurol 12: 166174.[Crossref] [Google Scholar]
  6. Foltz JL, Makumbi I, Sejvar JJ, Malimbo M, Ndyomugyenyi R, Atai-Omoruto AD, Alexander LN, Abang B, Melstrom P, Kakooza AM, Olara D, Downing RG, Nutman TB, Dowell SF, Lwamafa DKW, , 2013. An epidemiologic investigation of potential risk factors for nodding syndrome in Kitgum District, Uganda. PLoS ONE 8: e66419.[Crossref] [Google Scholar]
  7. Idro R, Opoka RO, Aanyu HT, Kakooza-Mwesige A, Piloya-Were T, Namusoke H, Musoke SB, Nalugya J, Bangirana P, Mwaka AD, White S, Chong K, Atai-Omoruto AD, Mworozi E, Nankunda J, Kiguli S, Aceng JR, Tumwine JK, , 2013. Nodding syndrome in Ugandan children—clinical features, brain imaging and complications: a case series. BMJ Open 3: e002540.[Crossref] [Google Scholar]
  8. Van Bemmel K, Derluyn I, Stroeken K, , 2014. Nodding syndrome or disease? On the conceptualization of an illness-in-the-making. Ethn Health 19: 100118.[Crossref] [Google Scholar]
  9. Dowell SF, Sejvar JJ, Riek L, Vandemaele KAH, Lamunu M, Kuesel AC, Schmutzhard E, Matuja W, Bunga S, Foltz J, Nutman TB, Winkler AS, Mbonye AK, , 2013. Nodding syndrome. Emerg Infect Dis 19: 13741384.[Crossref] [Google Scholar]
  10. World Health Organization, 2012. International Scientific Meeting on Nodding Syndrome, Kampala, Uganda, 30 July–1 August 2012. Available at: http://www.who.int/neglected_diseases/Nodding_syndrom_Kampala_Report_2012.pdf. Accessed November 8, 2014. [Google Scholar]
  11. Kaiser C, Kipp W, Asaba G, Mugisa C, Kabagambe G, Rating D, Leichsenring L, , 1996. The prevalence of epilepsy follows the distribution of onchocerciasis in a west Ugandan focus. Bull World Health Organ 74: 361367. [Google Scholar]
  12. Kaiser C, Asaba G, Leichsenring M, Kabagambe G, , 1998. High incidence of epilepsy related to onchocerciasis in west Uganda. Epilepsy Res 30: 247251.[Crossref] [Google Scholar]
  13. Kaiser C, Rubaale T, Tukesiga E, Kipp W, Kabagambe G, Okech Ojony J, Asaba G, , 2011. Association between onchocerciasis and epilepsy in the Itwara hyperendemic focus, west Uganda: controlling for time and intensity of exposure. Am J Trop Med Hyg 85: 225228.[Crossref] [Google Scholar]
  14. Kaiser C, Benninger C, Asaba G, Mugisa C, Kabagambe G, Kipp W, Rating D, , 2000. Clinical and electro-clinical classification of epileptic seizures in west Uganda. Bull Soc Pathol Exot 93: 255259. [Google Scholar]
  15. Garms R, Lakwo TL, Ndyomugyenyi R, Kipp W, Rubaale T, Tukesiga E, Katamanywa J, Post RJ, Amazigo UV, , 2009. The elimination of the vector Simulium neavei from the Itwara onchocerciasis focus in Uganda by ground larviciding. Acta Trop 111: 203210.[Crossref] [Google Scholar]
  16. Kaiser C, Asaba G, Mugisa C, Kipp W, Kasoro S, Rubaale T, Kabagambe G, , 1998. Antiepileptic drug treatment in rural Africa: involving the community. Trop Doct 28: 7377.[Crossref] [Google Scholar]
  17. Kaiser C, , 1998. Introducing Anti-Epileptic Treatment into Primary Health Care: A Case Study in an Area of High Epilepsy Prevalence in Kabarole District, West Uganda. Thesis, Master of Science in Community Health and Health Management in Developing Countries. Heidelberg, Germany: Institute of Tropical Hygiene and Public Health, University of Heidelberg. [Google Scholar]
  18. Kaiser C, Asaba G, Kasoro S, Rubaale T, Kabagambe G, Mbabazi M, , 2007. Mortality from epilepsy in an onchocerciasis-endemic area in west Uganda. Trans R Soc Trop Med Hyg 101: 4855.[Crossref] [Google Scholar]
  19. National Center for Health Statistics, 1977. NCHS Growth Curves for Children: Birth–18 Years. DHEW publication no. (PHS) 78-1650. Rockville, MD: U.S. Department of Health Education and Welfare. Available at: http://www.cdc.gov/nchs/data/series/sr_11/sr11_165.pdf. Accessed November 8, 2014. [Google Scholar]
  20. Van der Waals FW, Goudsmit J, Gajdusek DC, , 1983. See-ee: clinical characteristics of highly prevalent seizure disorders in the Gbawein and Wroughbarh clan region of Grand Bassa County, Liberia. Neuroepidemiology 2: 3544.[Crossref] [Google Scholar]
  21. Gerrits C, , 1984. Contextual Meaning of “Sii,” epilepsy, in a part of Grand Bassa County, Liberia. Liberian Stud J 10: 5566. [Google Scholar]
  22. Prischich F, De Rinaldis M, Bruno F, Egeo G, Santori C, Zappaterreno A, Fattouch J, Di Bonaventura C, Bada J, Russo G, Pizzuti A, Cardona F, Sa'a Vullo V, Giallonardo AT, D'Erasmo E, Pellicia A, Vanacore N, , 2008. High prevalence of epilepsy in a village in the Littoral Province of Cameroon. Epilepsy Res 82: 200210.[Crossref] [Google Scholar]
  23. Rwiza HT, Kilonzo GP, Haule J, Matuja WBP, Mteza I, Mbena P, Kilima PM, Mwaluko G, Mwang'ombola R, Mwaijande F, Rweyemamu G, Matowo A, Jillek-Aall LM, , 1992. Prevalence and incidence of epilepsy in Ulanga, a rural Tanzanian district: a community-based study. Epilepsia 33: 10511056.[Crossref] [Google Scholar]
  24. Winkler AS, Wallner B, Friedrich K, Pfausler B, Unterberger I, Matuja W, Jilek-Aall L, Schmutzhard E, , 2014. A longitudinal study on nodding syndrome—a new African epilepsy disorder. Epilepsia 55: 8693.[Crossref] [Google Scholar]
  25. Winkler AS, Friedrich K, Meindl M, Kidunda A, Nassri A, Jillek-Aall L, Matuja W, Schmutzhard E, , 2010. Clinical characteristics of people with head nodding in southern Tanzania. Trop Doct 40: 173175.[Crossref] [Google Scholar]
  26. Idro R, Musubire KA, Byamah Mutamba B, Namusoke H, Muron J, Abbo C, Oriyabuzu R, Ssekyewa J, Okor C, Mwaka D, Ssebadukka P, Makumbi I, Opar B, Aceng JR, Mbonye AK, , 2013. Proposed guidelines for the management of nodding syndrome. Afr Health Sci 13: 219232. [Google Scholar]
  27. Idro R, Namusoke H, Abbo C, Mutamba BB, Kakooza-Mwesige A, Opoka RO, Musubire AK, Mwaka AD, Opar BT, , 2014. Patients with nodding syndrome in Uganda improve with symptomatic treatment: a cross-sectional study. BMJ Open 4: e006476.[Crossref] [Google Scholar]
  28. Scheffner D, König S, Rauterberg-Ruland I, Kochen W, Hofmann WJ, Unkelbach S, , 1988. Fatal liver failure in 16 children with valproate therapy. Epilepsia 29: 530542.[Crossref] [Google Scholar]
  29. Meador KJ, Baker GA, Browning N, Cohen MJ, Bromley RL, Clayton-Smith J, Kalayjian LA, Kanner A, Liporace JD, Pennell PB, Privitera M, Loring DW, NEAD Study Group; , 2013. Fetal antiepileptic drug exposure and cognitive outcomes at age 6 years (NEAD study): a prospective observational study. Lancet Neurol 12: 244252.[Crossref] [Google Scholar]
  30. Star K, Edwards IR, Choonara I, , 2014. Valproic acid and fatalities in children: a review of individual case safety reports in VigiBase. PLoS ONE 9: e108970.[Crossref] [Google Scholar]
  31. Zhang LL, Zeng LN, Li YP, , 2011. Side effects of phenobarbital in epilepsy: a systematic review. Epileptic Disord 13: 349365. [Google Scholar]
  32. Yasiry Z, Shorvon SD, , 2012. How phenobarbital revolutionized epilepsy therapy: the story of phenobarbital in epilepsy in the last 100 years. Epilepsia 53 (Suppl 8): 2639.[Crossref] [Google Scholar]
  33. Spencer PS, Vandermaele K, Richer M, Palmer VS, Chungong S, Anker M, Ayana Y, Opoka ML, Klaucke BN, Quarello A, Tumwine JK, , 2013. Nodding syndrome in Mundri county, South Sudan: environmental, nutritional and infectious factors. Afr Health Sci 13: 183204. [Google Scholar]
  34. Centers for Disease Control and Prevention, 2012. Nodding syndrome—South Sudan, 2011. MMWR 61: 5253. [Google Scholar]
  35. Pion SDS, Kaiser C, Boutros-Toni F, Cournil A, Taylor MM, Meredith SEO, Stufe A, Bertocchi I, Kipp W, Preux P-M, Boussinesq M, , 2009. Epilepsy in onchocerciasis endemic areas: systematic review and meta-analysis of population-based surveys. PLoS Negl Trop Dis 3: e461.[Crossref] [Google Scholar]
  36. Kaiser C, Pion SDS, Boussinesq M, , 2013. Case-control studies on the relationship between onchocerciasis and epilepsy: systematic review and meta-analysis. PLoS Negl Trop Dis 7: e2147.[Crossref] [Google Scholar]
  37. König R, Nassri A, Meindl M, Matuja W, Kidunda AR, Siegmund V, Bretzel G, Löscher T, Jillek-Aall L, Schmutzhard E, Winkler AS, , 2010. The role of Onchocerca volvulus in the development of epilepsy in a rural area of Tanzania. Parasitology 137: 15591568.[Crossref] [Google Scholar]
  38. Duke BO, Vincelette J, Moore PJ, , 1976. Microfilaria in the cerebral fluid, and neurological complications, during treatment of onchocerciasis with diethylcarbamazine. Tropenmed Parasitol 27: 123132. [Google Scholar]
  39. Kaiser C, Pion S, Boussinesq M, , 2009. Head nodding syndrome and river blindness: a parasitological perspective. Epilepsia 50: 23252326.[Crossref] [Google Scholar]
  40. Nabbout R, , 2012. Autoimmune and inflammatory epilepsies. Epilepsia 53 (Suppl 4): 5862.[Crossref] [Google Scholar]
  41. Johnson T, Tyagi R, Lee PR, Lee M, Johnson KR, Kowalak J, Medynets M, Hategan A, Nutman TB, Sejvar J, Makumbi I, Aceng JR, Dowell Nath A, , 2014. Detection of auto-antibodies to leiomodin-1 in patients with nodding syndrome. J Neuroimmunol 275: 103.[Crossref] [Google Scholar]
  42. Marchi N, Granata T, Ghosh C, Janigro D, , 2012. Blood-brain barrier dysfunction and epilepsy: pathophysiologic role and therapeutic approaches. Epilepsia 53: 18771886.[Crossref] [Google Scholar]
  43. Colebunders R, Hendy A, Nanyunja M, Wamala JF, Van Oijen M, , 2014. Nodding syndrome—a new hypothesis and new direction for research. Int J Infect Dis 27: 7477.[Crossref] [Google Scholar]
  44. Colebunders R, Post R, O'Neill S, Haesaert G, Opar B, Lakwo T, Laudisoit A, Hendy A, , 2015. Nodding syndrome since 2012: recent progress, challenges and recommendations for future research. Trop Med Int Health 20: 194200.[Crossref] [Google Scholar]
  45. Buchmann K, , 2014. ‘These nodding people’: experiences of having a child with nodding syndrome in postconflict northern Uganda. Epilepsy Behav 42: 7177.[Crossref] [Google Scholar]
  46. Coffeng LE, Stolk WA, Zoure HG, Veerman JL, Agblewonu KB, Murdoch ME, Noma M, Fobi G, Richardus JH, Bundy DA, Habbema D, de Vlas SJ, Amazigo UV, , 2013. African Programme for Onchocerciasis Control 1995–2015: model-estimated health impact and cost. PLoS Negl Trop Dis 7: e2032.[Crossref] [Google Scholar]

Data & Media loading...

  • Received : 26 Dec 2014
  • Accepted : 15 Mar 2015
  • Published online : 08 Jul 2015

Most Cited This Month

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error