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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 6

Possible causes of nodding syndrome, by toxic, nutritional, and genetic factors*

Possible cause by category Investigation (reference) Negative findings Positive findings
Toxic encephalopathy
Mercury Foltz et al., Uganda (6) Urine (12 patients) None
Bunga. , Sudan (8) Urine (20 patients) None
Homocysteine Foltz et al., Uganda (6) Urines (23 patients) None
Thiocyanates (cassava toxicity) Foltz et al., Uganda (6) Urinary thiocyanate levels not increased (7% patients vs. 7% controls; p = NS) None
Bunga, Sudan (8) Urinary thiocyanate levels not increased (20% patients vs. 20% controls; p = NS) None
Copper Foltz et al., Uganda (6) No increases in serum levels (17 patients tested) None
Lead Foltz et al., Uganda, 2010 No difference (all within reference ranges)
Arsenic
Bunga, Sudan (8)
Urine (20 patients)
None
Nutritional neuropathology
Cobalamin (vitamin B12) Foltz et al., Uganda (6) Normal (92% patients vs. 92% controls; p = NS) None
Bunga, Sudan (8) Normal (97% patients vs. 100% controls; p = NS) None
Folate Foltz et al., Uganda (6) Normal (91% patients vs. 100% controls; p = NS) None
Pyridoxine (vitamin B6) Foltz et al., Uganda (6) None Deficient (73% patients vs. 64% controls; p = NS)
Bunga, Sudan (8) None Deficient (79% patients vs. 59% controls; p = 0.06)
Retinol (vitamin A) Foltz et al., Uganda (6) Normal (60% patients vs. 67% controls; p = NS) None
Zinc Foltz et al., Uganda (6) Normal (53% patients vs. 33% controls; p = NS) None
Selenium
Foltz et al., Uganda (6)
None
Deficient (all cases and controls)
Genetic epilepsy
Deep exome sequencing Sejvar (Washington University, St. Louis, MO, USA., unpub data) Uganda, South Sudan No specific epilepsy genes or consistent rare variant genes (1 gene from an affected child in Sudan and 1 gene from an affected child in Uganda sequenced) None

*NS, not significant.

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