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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)

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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.

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