Volume 19, Number 9—September 2013
Synopsis
Nodding Syndrome
Table 3
Clinical and neurodiagnostic findings of case studies of nodding syndrome*
Location, author, date, (reference) | No. patients, case definition | Clinical findings | EEG findings | CSF findings | Neuroimaging findings |
---|---|---|---|---|---|
Sudan, Tumwine et al., 2001 (2) | 39 with episodes of repetitive head nodding several times a day | Neurologic examination results largely normal for 32 patients, with exception of mental retardation | 39 cases evaluated; seizures recorded in 6; disease progression correlated with diffuse slowing and delta–theta activity | 16 CSF specimens negative for Onchocerca spp. by PCR | ND |
Tanzania, Winkler etal., 2008 (9) | 62 with a repetitive short loss of neck muscle tone resulting in nodding of the head | Neurologic examination results for 12 patients largely unremarkable; 2 with upper motor neuron findings | 6 of 10 with abnormal EEG results, including intermittent generalized slowing and sharp wave activity | 48 CSF specimens; 3 with increased lymphocyte counts; protein and glucose levels within reference range for all | 8 of 12 brain MRIs showing abnormalities, including hippocampal abnormalities (3), gliotic lesions (3), or both (2) |
Uganda, Sejvar et al., 2010 (7) | 23 with head nodding in previously normal child, with ≥1 other neurodevelopmental abnormality | Neurologic examination results for 23 patients largely unremarkable; 2 with focal findings | 10 of 12 with abnormal EEG results, including generalized slowing and runs of spike activity; 2 nodding episodes recorded, demonstrating atonic seizure | 16 CSF specimens; all grossly clear, with glucose and protein levels within reference ranges | 4 of 5 brain MRIs showing varying degrees of cortical and cerebellar atrophy disproportionate to age; no focal/white matter lesions |
South Sudan, Bunga, 2011 (unpub. data) | 25 with head nodding in previously normal child, with ≥1 other neurodevelopmental abnormality | Neurologic examination for 25 nonfocal patients | ND | ND | ND |
*EEG, electroencephalography; CSF, cerebrospinal fluid; ND, not done; MRI, magnetic resonance image.
References
- Lacey M. Nodding disease: mystery of southern Sudan. Lancet Neurol. 2003;2:714 and. DOIPubMedGoogle Scholar
- 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
- 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
- Aall-Jilek LM. Epilepsy in the Wapogoro tribe in Tanganyika. Acta Psychiatr Scand. 1965;41:57–86. DOIGoogle Scholar
- 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
- 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
- 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
- Centers for Disease Control and Prevention. Nodding syndrome—South Sudan, 2011. MMWR Morb Mortal Wkly Rep. 2012;61:52–4 .PubMedGoogle Scholar
- 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
- 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
- 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.
- Donnelly J. CDC planning trial for mysterious nodding syndrome. Lancet. 2012;379:299. DOIPubMedGoogle Scholar
- Williams SC. Nodding syndrome leaves baffled scientists shaking their heads. Nat Med. 2012;18:334 . DOIPubMedGoogle Scholar
- Wasswa H. Ugandan authorities deal with a mysterious ailment that leaves people nodding continuously. BMJ. 2012;344:e349. DOIPubMedGoogle Scholar
- 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
- World Health Organization. Report of an international conference on nodding syndrome. Bull World Health Organ. 2013;91. In press.
- Sillanpää M, Shinnar S. Long-term mortality in childhood-onset epilepsy. N Engl J Med. 2010;363:2522–9. DOIPubMedGoogle Scholar
- Lin FC, Wei LJ, Shih PY. Effect of levetiracetam on truncal tic in neuroacanthocytosis. Acta Neurol Taiwan. 2006;15:38–42 .PubMedGoogle Scholar
- 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
- World Health Organization Economic and Social Council. Onchocerciasis and its control. Geneva: The Organization; 1995.
- Sauerbrey M. The Onchocerciasis Elimination Program for the Americas (OEPA). Ann Trop Med Parasitol. 2008;102(Suppl 1):25–9 . DOIPubMedGoogle Scholar
- 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
- 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
- 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
- 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
- Getahun H, Mekonnen A. TekleHaimanot R, Lambein F. Epidemic of neurolathyrism in Ethiopia. Lancet. 1999;354:306–7. DOIPubMedGoogle Scholar
- 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
- 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
- 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
- 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
- Geschwind MD, Shu H, Haman A, Sejvar JJ, Miller BL. Rapidly progressive dementia. Ann Neurol. 2008;64:97–108 . DOIPubMedGoogle Scholar
- 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
- 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
- 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
- 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
- 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
- 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
- Gospe SM Jr. Neonatal vitamin-responsive epileptic encephalopathies. Chang Gung Med J. 2010;33:1–12 .PubMedGoogle Scholar
- 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
- 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