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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 5

Possible causes of nodding syndrome, by infectious and postinfectious findings*

Possible cause by category Investigation (reference) Negative findings Positive findings
Infectious encephalitis
Malaria Foltz et al., Uganda (6) Blood smear (98% case-patients vs. 95% controls) None
Trypanosomiasis Foltz et al., Uganda (6) Seronegative (36 patients tested) None
Tumwine et al., Sudan (2) Seronegative (69 patients tested) None
Cysticercosis Foltz et al., Uganda (6) Seronegative (36 patients tested); cysts absent by MRI (5 patients tested) None
Prion disease Sejvar et al., Uganda (7) EEG and MRI results and clinical course not compatible None
Winkler et al., Tanzania (9) EEG and MRI results and clinical course not compatible None
Onchocerciasis Tumwine et al., Sudan, 2001 investigation (2) None Skin snip specimens for 93% of patients vs. 63% in controls; p<0.001
Tumwine et al., Sudan, 2002 investigation (2) None Skin snip specimens for 93% of patients vs. 44% of controls; p<0.008
Winkler et al., Tanzania (9) 48 CSF samples PCR negative for Onchocerca volvulus microfilariae Microfilariae in skin correlated with lesions by MRI; p = 0.02
Foltz et al., Uganda (6) None Antibody in 95% of patients vs. 49% of controls; p<0.001
Riek et al., Sudan (8) None Skin snip in 76% of patients vs. 47% of controls; p = 0.02
Other microfilarial disease Tumwine et al., Sudan, 2001 investigation (2) None Mansonella perstans nematodes in 52% of patients vs. 31% of controls; p = 0.005
Tumwine et al,, Sudan, 2001 investigation (2) Blood sample for Loa loa microfilariae (69 patients tested) None
Tumwine et al., Sudan, 2001 investigation (2) Lymphatic filariasis by ICT ( 26 patients tested) None
Unpub. data, Uganda None 2 skin snip DNA sequences similar to those of Mansonella spp. nematodes
Unknown pathogens
Unpub. data, Uganda
42 serum samples and 16 CSF specimens by broadly reactive PCRs for 19 virus families
None
Para/postinfectious encephalopathy
Measles (SSPE like) Foltz et al., Uganda (6) No epidemiologic association for 16 CSF samples by PCR None
Acute disseminated encephalomyelitis Sejvar et al., Uganda (7) Brain MRI (5 patients) None
Winkler et al., Tanzania (9) Brain MRI (12 patients) None
Poststreptococcal (Sydenham chorea-like) Sejvar et al., Uganda (7) No movement disorders None
Neuronal antibodies Unpub. data (Mayo Clinic, Rochester, MN, USA), Uganda 12 CSF samples for known neuronal antibodies None
Unpub. data (Emory University, Atlanta, GA, USA), Uganda 3 CSF samples for neuronal antibodies by in situ hybridization with rat brain slices and human brain homogenate None
Hepatitis E Foltz et al., Uganda (6) Seronegative (52% case-patients vs. 58% controls) None

*EEG, electroencephalography; MRI, magnetic resonance imaging CSF, cerebrospinal fluid; ICT, immunochromatographic test; SSPE, subacute sclerosing panencephalitis.

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