Skip directly to site content Skip directly to page options Skip directly to A-Z link Skip directly to A-Z link Skip directly to A-Z link
Volume 19, Number 9—September 2013
Synopsis

Nodding Syndrome

Scott F. DowellComments to Author , James J. Sejvar, Lul Riek, Katelijn A.H. Vandemaele, Margaret Lamunu, Annette C. Kuesel, Erich Schmutzhard, William Matuja, Sudhir Bunga, Jennifer Foltz, Thomas B. Nutman, Andrea S. Winkler, and Anthony K. Mbonye
Author affiliations: Centers for Disease Control and Prevention, Atlanta, Georgia, USA (S.F. Dowell, J.J. Sejvar, S. Bunga, J. Foltz); Ministry of Health, Juba, South Sudan (L. Riek); World Health Organization, Geneva, Switzerland (K.A.H. Vandemaele, M. Lamunu, A.C. Kuesel); University of Innsbruck, Innsbruck, Austria (E. Schmutzhard); Muhimbili University, Dar es Salaam, Tanzania (W. Matuja); National Institutes of Health, Bethesda, Maryland, USA (T.B. Nutman); Technical University of Munich, Munich, Germany (A.S. Winkler); Ministry of Health, Kampala, Uganda (A.K. Mbonye); Makerere University, Kampala (A.K. Mbonye)

Main Article

Table 4

Selected risk factors for nodding syndrome from 4 case–control studies*

Risk factor Study (reference)
Sudan, 2002; 13 case-patients, 19 controls (2) Uganda, 2009; 49 case-patients, 49 controls (6) Uganda, 2010; 73 case-patients, 73 controls† South Sudan, 2011; 38 case-patients, 38 controls (7,8)
Infectious
History of measles Less common (15% cases vs. 58% controls; p = 0.03) No difference (24% case-patients vs. 6% controls; p = 0.02‡) NA NA
History of malaria NA ND NA NA
History of meningitis No difference (0% vs. 6%; p = 1.0) NA NA NA
Prior treatment for onchocerciasis (ivermectin) No difference 62% cases vs. 37% controls) No difference (33% case-patients vs. 25% controls) NA NA
Consumption of animal brain (risk for prion disease)
 Baboon brain consumption (46% vs. 22%; p = 0.25)
 No association with brain consumption
 NA
 NA
Toxic/environmental
Exposure to munitions NA More common in case-patients (71% vs. 51%; p = 0.06) Exposure to gun raids more common in case-patients (54% vs. 27%) ND
Exposure to pesticides (on seeds) No difference ND NA NA
Consumption of crushed roots NA More common in case-patients (39% vs. 16%; p = 0.02) No differences in 17 root types before onset of nodding syndrome; root consumption more common after onset of nodding syndrome (22% vs. 0%) ND
Consumption of cassava (thiocyanate toxicity)
 Widely consumed, no acute toxicity reported
 ND (100% consumption)
 Specifically asked about bitter cassava: no difference
 NA
Nutritional
Early malnutrition NA NA NA Hunger more common for case-patients 2 y of age (24% vs. 8%; p = 0.03)
Current wasting Weight-for-age Z scores lower in case-patients (−1.6 vs. −1.0, p = 0.09) Low BMI for age (42% vs. 13%; p<0.01) Low BMI for age (42% vs. 26%; p = 0.03) Low BMI for age (16% vs. 3%; p = 0.06)
Current stunting Height-for-age Z scores lower in case-patients (−1.5 vs. −0.8, p = 0.29) Low height for age (60% vs. 29%; p<0.01) Low height for age (35% vs. 20%; p = 0.05) Low height for age (24% vs. 3%; p = 0.03)
Consumption of red sorghum 54% case-patients vs. 16% controls; p = 0.05 ND ND ND
Consumption of spoiled relief foods NA ND NA NA
Consumption of river fish NA ND NA ND
Consumption of rodent brain NA ND NA NA
Consumption of river water NA ND NA NA

*Selected risk factors, all positive associations and selected negative findings (see original reference for full listings). NA, not assessed; ND, no difference; BMI body mass index.
†Unpub. data.
‡Not significant after age adjustment.

Main Article

References
  1. Lacey  M. Nodding disease: mystery of southern Sudan. Lancet Neurol. 2003;2:714 and. DOIPubMedGoogle Scholar
  2. Tumwine  JK, Vandemaele  K, Chungong  S, Richer  M, Anker  M, Ayana  Y, Clinical and epidemiologic characteristics of nodding syndrome in Mundri County, South Sudan. Afr Health Sci. 2012;12:242–8 .PubMedGoogle Scholar
  3. van der Waals  F, Goudsmit  J, Gajdusek  DC. See-ee: clinical characteristics of highly prevalent seizure disorders in the Gbawein and Wroughbarh clan region of Grand Bassa County, Liberia. Neuroepidemiology. 1983;2:35–44. DOIGoogle Scholar
  4. Aall-Jilek  LM. Epilepsy in the Wapogoro tribe in Tanganyika. Acta Psychiatr Scand. 1965;41:57–86. DOIGoogle Scholar
  5. Kaiser  C, Benninger  C, Asaba  G, Mugisa  C, Kabagambe  G, Kipp  W, Clinical and electro-clinical classification of epileptic seizure in west Uganda. Bull Soc Pathol Exot. 2000;93:255–9 .PubMedGoogle Scholar
  6. Foltz  J, Makumbi  I, Sejvar  JJ, Malimbo  M, Ndyomugyenyi  R, Atai-Omoruto  AD, An epidemiologic investigation of potential risk factors for nodding syndrome in Kitgum District, Uganda. PLoS ONE. 2013;8. In press. DOIPubMedGoogle Scholar
  7. Sejvar  JJ, Kakooza  AM, Foltz  JL, Makumbi  I, Atai-Omoruto  AD, Malimbo  M, Clinical, neurologic, and electrophysiologic features of nodding syndrome, Kitgum, Uganda: an observational case series. Lancet Neurol. 2013;12:166–74 . DOIPubMedGoogle Scholar
  8. Centers for Disease Control and Prevention. Nodding syndrome—South Sudan, 2011. MMWR Morb Mortal Wkly Rep. 2012;61:52–4 .PubMedGoogle Scholar
  9. Winkler  AS, Friedrich  K, Konig  R, Meindl  M, Helbrok  R, Unterberger  I, The head nodding syndrome: clinical classification and possible causes. Epilepsia. 2008;49:2008–15 . DOIPubMedGoogle Scholar
  10. Winkler  AS, Friedrich  K, Meindl  M, Kidunda  A, Nassri  A, Jilek-Aall  L, Clinical characteristics of people with head nodding in southern Tanzania. Trop Doct. 2010;40:173–5. DOIPubMedGoogle Scholar
  11. Nyungura  JL, Lako  A, Gordon  A, Lejeng  L, William  G. Investigation into the nodding syndrome in Witto Payam, Western Equatoria State, 2010. Southern Sudan Medical Journal. 2011;4:3–6.
  12. Donnelly  J. CDC planning trial for mysterious nodding syndrome. Lancet. 2012;379:299. DOIPubMedGoogle Scholar
  13. Williams  SC. Nodding syndrome leaves baffled scientists shaking their heads. Nat Med. 2012;18:334 . DOIPubMedGoogle Scholar
  14. Wasswa  H. Ugandan authorities deal with a mysterious ailment that leaves people nodding continuously. BMJ. 2012;344:e349. DOIPubMedGoogle Scholar
  15. Fallon  A. Hundreds of new nodding disease cases reported. iUganda 2012;Section. Uganda local news story [cited 2013 Jun 4]. http://iuganda.ug/news/local/11793-hundreds-of-new-nodding-cases-reported.html
  16. World Health Organization. Report of an international conference on nodding syndrome. Bull World Health Organ. 2013;91. In press.
  17. Sillanpää  M, Shinnar  S. Long-term mortality in childhood-onset epilepsy. N Engl J Med. 2010;363:2522–9. DOIPubMedGoogle Scholar
  18. Lin  FC, Wei  LJ, Shih  PY. Effect of levetiracetam on truncal tic in neuroacanthocytosis. Acta Neurol Taiwan. 2006;15:38–42 .PubMedGoogle Scholar
  19. Noma  M, Nwoke  BE, Nutall  I, Tambala  PA, Enyong  P, Namsenmo  A, Rapid epidemiological mapping of onchocerciasis (REMO): its application by the African Programme for Onchocerciasis Control (APOC). Ann Trop Med Parasitol. 2002;96(Suppl 1):S29–39. DOIPubMedGoogle Scholar
  20. World Health Organization Economic and Social Council. Onchocerciasis and its control. Geneva: The Organization; 1995.
  21. Sauerbrey  M. The Onchocerciasis Elimination Program for the Americas (OEPA). Ann Trop Med Parasitol. 2008;102(Suppl 1):25–9 . DOIPubMedGoogle Scholar
  22. Jilek-Aall  L, Jilek  W, Miller  JR. Clinical and genetic aspects of seizure disorders prevalent in an isolated African population. Epilepsia. 1979;20:613–22. DOIPubMedGoogle Scholar
  23. Demirkaya  E, Ozen  S, Bilginer  Y, Ayaz  NA, Makay  BB, Unsal  E, The distribution of juvenile idiopathic arthritis in the eastern Mediterranean: results from the registry of the Turkish Paediatric Rheumatology Association. Clin Exp Rheumatol. 2011;29:111–6 .PubMedGoogle Scholar
  24. Demiroren  K, Yavuz  H, Cam  L, Oran  B, Karaaslan  S, Demiroren  S. Sydenham’s chorea: a clinical follow-up of 65 patients. J Child Neurol. 2007;22:550–4. DOIPubMedGoogle Scholar
  25. Bonmarin  I, Nunga  M, Perea  WA. Konzo outbreak, in the south-west of the Democratic Republic of Congo, 1996. J Trop Pediatr. 2002;48:234–8. DOIPubMedGoogle Scholar
  26. Getahun  H, Mekonnen  A. TekleHaimanot R, Lambein F. Epidemic of neurolathyrism in Ethiopia. Lancet. 1999;354:306–7. DOIPubMedGoogle Scholar
  27. Mutonga  DM, Pimentel  G, Muindi  J, Nzioka  C, Mutiso  J, Klena  JD, Epidemiology and risk factors for serogroup X meningococcal meningitis during an outbreak in western Kenya, 2005–2006. Am J Trop Med Hyg. 2009;80:619–24 .PubMedGoogle Scholar
  28. Morenikej  OA, Idowu  BA. Studies on the prevalence of urinary schistosomiasis in Ogun State, south-western Nigeria. West Afr J Med. 2011;30:62–5 .PubMedGoogle Scholar
  29. Barskey  AE, Glasser  JW, LeBaron  CW. Mumps resurgences in the United States: a historical perspective on unexpected elements. Vaccine. 2009;27:6186–95. DOIPubMedGoogle Scholar
  30. Collie  DA, Summers  DM, Sellar  RJ, Ironside  JW, Cooper  S, Zeidler  M, Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol. 2003;24:1560–9 .PubMedGoogle Scholar
  31. Geschwind  MD, Shu  H, Haman  A, Sejvar  JJ, Miller  BL. Rapidly progressive dementia. Ann Neurol. 2008;64:97–108 . DOIPubMedGoogle Scholar
  32. Basáñez  MG, Pion  SD, Boakes  E, Filipe  JA, Churcher  TS, Boussinesq  M. Effect of single-dose ivermectin on Onchocerca volvulus: a systematic review and meta-analysis. Lancet Infect Dis. 2008;8:310–22 . DOIPubMedGoogle Scholar
  33. Pion  SD, Kaiser  C, Boutros-Toni  F, Cournil  A, Taylor  MM, Meredith  SE, Epilepsy in onchocerciasis endemic areas: systematic review and meta-analysis of population-based surveys. PLoS Negl Trop Dis. 2009;3:e461 . DOIPubMedGoogle Scholar
  34. Kaiser  C, Rubaale  T, Tukesiga  E, Kipp  W, Kabasambe  G, Ojony  JO, Association between onchocerciasis and epilepsy in the Itwara hyperendemic focus, West Uganda: controlling for time and intensity of exposure. Am J Trop Med Hyg. 2011;85:225–8. DOIPubMedGoogle Scholar
  35. Katabarwa  M, Lakwo  T, Habumogisha  P, Richards  F, Eberhard  M. Could neurocysticercosis be the cause of “onchocerciasis-associated” epileptic seizures? Am J Trop Med Hyg. 2008;78:400–1 .PubMedGoogle Scholar
  36. König  R, Nassri  A, Meindl  M, Matuja  W, Kidunda  AR, Siegmund  V, The role of Onchocerca volvulus in the development of epilepsy in a rural area of Tanzania. Parasitology. 2010;137:1559–68. DOIPubMedGoogle Scholar
  37. Stockler  S, Plecko  B, Gospe  SM Jr, Coulter-Mackie  M, Connolly  M, van Karnebeek  C, Pyridoxine dependent epilepsy and antiquitin deficiency: clinical and molecular characteristics and recommendations for diagnosis, treatment and follow-up. Mol Genet Metab. 2011;104:48–60. DOIPubMedGoogle Scholar
  38. Gospe  SM Jr. Neonatal vitamin-responsive epileptic encephalopathies. Chang Gung Med J. 2010;33:1–12 .PubMedGoogle Scholar
  39. Kirvan  CA, Swedo  SE, Kurahara  D, Cunningham  MW. Streptococcal mimicry and antibody-mediated cell signaling in the pathogenesis of Sydenham's chorea. Autoimmunity. 2006;39:21–9. DOIPubMedGoogle Scholar
  40. Quek  AM, Britton  JW, McKeon  A, So  SE, Lennon  VA, Shin  C, Autoimmune epilepsy: clinical characteristics and response to immunotherapy. Arch Neurol. 2012;69:582–93. DOIPubMedGoogle Scholar

Main Article

Page created: August 20, 2013
Page updated: August 20, 2013
Page reviewed: August 20, 2013
The conclusions, findings, and opinions expressed by authors contributing to this journal do not necessarily reflect the official position of the U.S. Department of Health and Human Services, the Public Health Service, the Centers for Disease Control and Prevention, or the authors' affiliated institutions. Use of trade names is for identification only and does not imply endorsement by any of the groups named above.
file_external