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1

Dowell, Scott F., James J. Sejvar, Lul Riek, et al. "Nodding Syndrome." Emerging Infectious Diseases 19, no. 9 (2013): 1374–73. http://dx.doi.org/10.3201/eid1909.130401.

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2

Abd-Elfarag, Gasim, and Michaël Boele van Hensbroek. "Nodding Syndrome." Pediatric Infectious Disease Journal 38 (June 2019): S67—S71. http://dx.doi.org/10.1097/inf.0000000000002327.

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3

Bhwana, Dan, Bruno Mmbando, An Hotterbeekx, and Robert Colebunders. "Nodding Syndrome." Pediatric Infectious Disease Journal 38, no. 11 (2019): e313. http://dx.doi.org/10.1097/inf.0000000000002442.

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4

Imma, Ferraiuolo. "Nodding syndrome." Journal of the Neurological Sciences 455 (December 2023): 121272. http://dx.doi.org/10.1016/j.jns.2023.121272.

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5

Spencer, P. S., R. Mazumder, V. S. Palmer, and M. S. Pollanen. "Nodding syndrome phenotypes." Revue Neurologique 175, no. 10 (2019): 679–85. http://dx.doi.org/10.1016/j.neurol.2019.09.005.

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6

Johnson, Tory P., James Sejvar, Thomas B. Nutman, and Avindra Nath. "The Pathogenesis of Nodding Syndrome." Annual Review of Pathology: Mechanisms of Disease 15, no. 1 (2020): 395–417. http://dx.doi.org/10.1146/annurev-pathmechdis-012419-032748.

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Nodding syndrome is a rare, enigmatic form of pediatric epilepsy that has occurred in an epidemic fashion beginning in the early 2000s in geographically distinct regions of Africa. Despite extensive investigation, the etiology of nodding syndrome remains unclear, although much progress has been made in understanding the pathogenesis of the disease, as well as in treatment and prevention. Nodding syndrome is recognized as a defined disease entity, but it is likely one manifestation along a continuum of Onchocerca volvulus–associated neurological complications. This review examines the epidemiol
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7

Vieri, Melissa Krizia, Adam Hendy, John L. Mokili, and Robert Colebunders. "Nodding syndrome research revisited." International Journal of Infectious Diseases 104 (March 2021): 739–41. http://dx.doi.org/10.1016/j.ijid.2021.02.006.

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8

Arndts, Kathrin, Josua Kegele, Alain S. Massarani, et al. "Epilepsy and nodding syndrome in association with an Onchocerca volvulus infection drive distinct immune profile patterns." PLOS Neglected Tropical Diseases 17, no. 8 (2023): e0011503. http://dx.doi.org/10.1371/journal.pntd.0011503.

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Previous studies have described the association of onchocerciasis (caused by Onchocerca volvulus) with epilepsy, including nodding syndrome, although a clear etiological link is still missing. Cases are found in different African countries (Tanzania, South Sudan, Uganda, Democratic Republic of the Congo, Central African Republic and Cameroon). In our study we investigated immunological parameters (cytokine, chemokine, immunoglobulin levels) in individuals from the Mahenge area, Tanzania, presenting with either epilepsy or nodding syndrome with or without O. volvulus infection and compared them
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9

Kaiser, Christoph, Walter Kipp, Ephraim Tukesiga, George Asaba, and Tom Rubaale. "Nodding Syndrome, Western Uganda, 1994." American Journal of Tropical Medicine and Hygiene 93, no. 1 (2015): 198–202. http://dx.doi.org/10.4269/ajtmh.14-0838.

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10

Abd-Elfarag, Gasim Omer Elkhalifa, Arthur Wouter Dante Edridge, René Spijker, Mohamed Boy Sebit, and Michaël B. van Hensbroek. "Nodding Syndrome: A Scoping Review." Tropical Medicine and Infectious Disease 6, no. 4 (2021): 211. http://dx.doi.org/10.3390/tropicalmed6040211.

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Nodding syndrome (NS) is a debilitating yet often neglected neurological disease affecting thousands of children in several sub-Saharan African countries. The cause of NS remains unknown, and effective treatment options are lacking. Moreover, knowledge regarding NS is scarce and is based on a limited number of publications, with no comprehensive overview published to date. Therefore, the aim of this scoping review was to summarise the current evidence and identify existing knowledge gaps in order to help clinicians, scientists, and policymakers develop guidelines for prioritising this severe c
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11

Sehgal, Rachna, Neha Agarwal, and Rani Gera. "Nodding Syndrome - an Indian case." African Health Sciences 18, no. 3 (2018): 837. http://dx.doi.org/10.4314/ahs.v18i3.43.

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12

Colebunders, Robert, and Maarten J. Titulaer. "Nodding syndrome: Preventable and treatable." Science Translational Medicine 9, no. 377 (2017): eaam8532. http://dx.doi.org/10.1126/scitranslmed.aam8532.

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13

Edridge, Arthur W. D., Gasim Abd-Elfarag, Martin Deijs, Maarten F. Jebbink, Michael Boele van Hensbroek, and Lia van der Hoek. "Divergent Rhabdovirus Discovered in a Patient with New-Onset Nodding Syndrome." Viruses 14, no. 2 (2022): 210. http://dx.doi.org/10.3390/v14020210.

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A divergent rhabdovirus was discovered in the bloodstream of a 15-year-old girl with Nodding syndrome from Mundri West County in South Sudan. Nodding syndrome is a progressive degenerative neuropathy of unknown cause affecting thousands of individuals in Sub-Saharan Africa. The index case was previously healthy until she developed head-nodding seizures four months prior to presentation. Virus discovery by VIDISCA-NGS on the patient’s plasma detected multiple sequence reads belonging to a divergent rhabdovirus. The viral load was 3.85 × 103 copies/mL in the patient’s plasma and undetectable in
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14

van Bemmel, Karin. "Conceptualizing Illness: Nodding Syndrome in Northern Uganda." Afrika Focus 33, no. 1 (2020): 143–52. http://dx.doi.org/10.1163/2031356x-03301010.

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This paper presents an ethnographic study of conceptualizations of nodding syndrome (NS) in Uganda. NS is a poorly understood condition characterized by repetitive nodding of the head, mental retardation and stunted growth, which affects thousands of children in northern Uganda, South Sudan and Tanzania. Although extensive research for causative agents has been conducted, no convincing single cause has been reported. This study establishes an understanding of different representations of NS and argues that the episodes of head nodding are related to the socio-political body in which they are m
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15

Idro, Richard, Rodney Ogwang, Edward Kayongo, et al. "The natural history of nodding syndrome." Epileptic Disorders 20, no. 6 (2018): 508–16. http://dx.doi.org/10.1684/epd.2018.1012.

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16

Colebunders, R., A. Hendy, and M. van Oijen. "Nodding Syndrome in Onchocerciasis Endemic Areas." Trends in Parasitology 32, no. 8 (2016): 581–83. http://dx.doi.org/10.1016/j.pt.2016.05.013.

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17

Friedrich, M. J. "Potential Cause of Nodding Syndrome Identified." JAMA 317, no. 15 (2017): 1517. http://dx.doi.org/10.1001/jama.2017.3089.

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18

Colebunders, Robert, Amber Hadermann, and Joseph Nelson Siewe Fodjo. "The onchocerciasis hypothesis of nodding syndrome." PLOS Neglected Tropical Diseases 17, no. 8 (2023): e0011523. http://dx.doi.org/10.1371/journal.pntd.0011523.

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Nodding syndrome (NS) is a phenotypic presentation of onchocerciasis-associated epilepsy (OAE). OAE is an important public health problem in areas with high ongoing Onchocerca volvulus transmission. OAE, including NS, is preventable by strengthening onchocerciasis elimination programs. The presence of tau in OAE postmortem brains could be the consequence of neuroinflammation directly or indirectly induced by O. volvulus. Omics research is needed to investigate whether O. volvulus worms contain a neurotropic virus.
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19

Benedek, Gil, Mahmoud Abed El Latif, Keren Miller, et al. "Macrophage migration inhibitory factor in Nodding syndrome." PLOS Neglected Tropical Diseases 15, no. 10 (2021): e0009821. http://dx.doi.org/10.1371/journal.pntd.0009821.

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Nodding syndrome (NS) is a catastrophic and enigmatic childhood epilepsy, accompanied by multiple neurological impairments and neuroinflammation. Of all the infectious, environmental and psychological factors associated with NS, the major culprit is Onchocerca Volvulus (Ov)–a parasitic worm transmitted to human by blackflies. NS seems to be an ’Autoimmune Epilepsy’ in light of the recent findings of deleterious autoimmune antibodies to Glutamate receptors and to Leiomodin-I in NS patients. Moreover, we recently found immunogenetic fingerprints in HLA peptide-binding grooves associate with prot
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20

Vogel, Gretchen. "Parasitic worm may trigger mystery nodding syndrome." Science 355, no. 6326 (2017): 678. http://dx.doi.org/10.1126/science.355.6326.678.

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21

Spinney, Laura. "Link to Alzheimer's seen in nodding syndrome." Science 362, no. 6421 (2018): 1341. http://dx.doi.org/10.1126/science.362.6421.1341.

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22

Ridler, Charlotte. "Nodding syndrome discovered to be a tauopathy." Nature Reviews Neurology 14, no. 11 (2018): 632. http://dx.doi.org/10.1038/s41582-018-0086-8.

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23

Hotterbeekx, A., S. Van Hees, J. N. Siewe Fodjo, and R. Colebunders. "From nodding syndrome to onchocerciasis-associated epilepsy." Revue Neurologique 176, no. 5 (2020): 405–6. http://dx.doi.org/10.1016/j.neurol.2019.12.011.

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24

Burton, Adrian. "Uganda: how goes the nodding syndrome war?" Lancet Neurology 15, no. 1 (2016): 30–31. http://dx.doi.org/10.1016/s1474-4422(15)00350-6.

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25

Donnelly, John. "CDC planning trial for mysterious nodding syndrome." Lancet 379, no. 9813 (2012): 299. http://dx.doi.org/10.1016/s0140-6736(12)60126-3.

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26

Pollanen, Michael S., Sylvester Onzivua, Janice Robertson, et al. "Nodding syndrome in Uganda is a tauopathy." Acta Neuropathologica 136, no. 5 (2018): 691–97. http://dx.doi.org/10.1007/s00401-018-1909-9.

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27

Colebunders, Robert, Julia Irani, and Rory Post. "Nodding syndrome—we can now prevent it." International Journal of Infectious Diseases 44 (March 2016): 61–63. http://dx.doi.org/10.1016/j.ijid.2016.01.016.

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28

Buchmann, Kristine. "‘These nodding people’: Experiences of having a child with nodding syndrome in postconflict Northern Uganda." Epilepsy & Behavior 42 (January 2015): 71–77. http://dx.doi.org/10.1016/j.yebeh.2014.10.027.

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29

Williams, Sarah C. P. "Nodding syndrome leaves baffled scientists shaking their heads." Nature Medicine 18, no. 3 (2012): 334. http://dx.doi.org/10.1038/nm0312-334.

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30

Cross, J. Helen. "Nodding syndrome—a challenge for African public health." Lancet Neurology 12, no. 2 (2013): 125–26. http://dx.doi.org/10.1016/s1474-4422(12)70326-5.

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31

Winkler, Andrea, Valerie Palmer, Erich Schmutzhard, and Peter Spencer. "Le nodding syndrome : une nouvelle forme d’épilepsie tropicale." Revue Neurologique 174 (April 2018): S186—S187. http://dx.doi.org/10.1016/j.neurol.2018.02.073.

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32

Idro, R. "The nodding syndrome: A new form of epilepsy?" Revue Neurologique 169 (April 2013): A195—A196. http://dx.doi.org/10.1016/j.neurol.2013.01.466.

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33

Spencer, P. "Nodding Syndrome: an epileptic disorder restricted to Africa?" Journal of the Neurological Sciences 357 (October 2015): e509. http://dx.doi.org/10.1016/j.jns.2015.09.332.

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34

Idro, Richard. "Is nodding syndrome an onchocerca volvulus associated epilepsy?" IBRO Reports 7 (December 2019): 53–54. http://dx.doi.org/10.1016/j.ibror.2019.09.007.

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35

Pollanen, Michael S., and Sylvester Onzivua. "The tau hypothesis of nodding syndrome in Africa." PLOS Neglected Tropical Diseases 17, no. 8 (2023): e0011526. http://dx.doi.org/10.1371/journal.pntd.0011526.

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36

Ogwang, Rodney, Ronald Anguzu, Pamela Akun, et al. "Asymptomatic malaria parasitaemia and seizure control in children with nodding syndrome; a cross-sectional study." BMJ Open 8, no. 10 (2018): e023624. http://dx.doi.org/10.1136/bmjopen-2018-023624.

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ObjectivePlasmodium falciparumis epileptogenic and in malaria endemic areas, is a leading cause of acute seizures. In these areas, asymptomatic infections are common but considered benign and so, are not treated. The effects of such infections on seizures in patients with epilepsy is unknown. This study examined the relationship betweenP. falciparuminfection and seizure control in children with a unique epilepsy type, the nodding syndrome.DesignThis cross-sectional study was nested in an ongoing trial ‘Doxycycline for the treatment of nodding syndrome (NCT02850913)’. We hypothesised that, in p
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37

Mwaka, Amos, Jerome Roy Semakula, Catherine Abbo, and Richard Idro. "Nodding syndrome: recent insights into etiology, pathophysiology, and treatment." Research and Reports in Tropical Medicine Volume 9 (May 2018): 89–93. http://dx.doi.org/10.2147/rrtm.s145209.

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38

Spencer, P. S., C. Okot, V. S. Palmer, R. Valdes Angues, and R. Mazumder. "Nodding syndrome: A key role for sources of nutrition?" eNeurologicalSci 27 (June 2022): 100401. http://dx.doi.org/10.1016/j.ensci.2022.100401.

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39

De Castro, Ana Cristina, and Ivan Ortega-Deballon. "Nodding syndrome: bridging the gap—a scoping review protocol." BMJ Open 10, no. 10 (2020): e035269. http://dx.doi.org/10.1136/bmjopen-2019-035269.

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IntroductionNodding syndrome (NS) is an encephalopathy of unknown origin that affects children aged between 3 and 15 years old. Cases have been reported since the 1950 in Tanzania and South Sudan, the most heavily affected population is the Acholi community in Uganda. In response to the high incidence of the disease, the Ugandan Government has developed a management algorithm, but access to such measures in affected communities is limited. There is little funding for research on the disease, consequently, few studies have been conducted to date. Nevertheless, the number of scientific publicati
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40

Johnson, Tory, Thomas Nutman, Scott Dowell, and Avindra Nath. "Closing the Loop between Nodding Syndrome and Onchocerca Infection." Trends in Parasitology 33, no. 7 (2017): 490. http://dx.doi.org/10.1016/j.pt.2017.05.002.

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41

Mitchell, Katrina B., Julie Kornfeld, Joseph Adiama, et al. "Nodding syndrome in northern Uganda: Overview and community perspectives." Epilepsy & Behavior 26, no. 1 (2013): 22–24. http://dx.doi.org/10.1016/j.yebeh.2012.10.030.

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42

Spencer, P. S., D. L. Kitara, S. K. Gazda, and A. S. Winkler. "Nodding syndrome: 2015 International Conference Report and Gulu Accord." eNeurologicalSci 3 (June 2016): 80–83. http://dx.doi.org/10.1016/j.ensci.2015.11.001.

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43

Spencer, Peter S., Rajarshi Mazumder, and Valerie S. Palmer. "Response to R. Colebunders: Helminth infections in Nodding syndrome." Journal of the Neurological Sciences 372 (January 2017): 441. http://dx.doi.org/10.1016/j.jns.2016.10.010.

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44

Winkler, Andrea S., Katrin Friedrich, Rebekka König, et al. "The head nodding syndrome-Clinical classification and possible causes." Epilepsia 49, no. 12 (2008): 2008–15. http://dx.doi.org/10.1111/j.1528-1167.2008.01671.x.

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45

Kaiser, Christoph, Sébastien Pion, and Michel Boussinesq. "Head nodding syndrome and river blindness: A parasitologic perspective." Epilepsia 50, no. 10 (2009): 2325–26. http://dx.doi.org/10.1111/j.1528-1167.2009.02280.x.

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46

Abd-Elfarag, Gasim O. E., Jake D. Mathewson, Lukudu Emmanuel, et al. "Nodding Syndrome: Clinical Characteristics, Risks Factors, Access to Treatment, and Perceptions in the Greater Mundri Area, South Sudan." Pathogens 12, no. 2 (2023): 190. http://dx.doi.org/10.3390/pathogens12020190.

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We conducted a house-to-house survey in the Mundri, Western Equatoria state of South Sudan to investigate the clinical characteristics, risk factors, access to treatment and perceptions about nodding syndrome (NS). In total, 224 NS cases with median age of seizure onset of 10 years were identified. Head nodding only was reported in 50 (22.3%) cases, and head nodding plus other types of seizures in 174 (77.7%) cases. Wasting, stunted growth, delayed sexual development and speech and behavioral abnormalities were observed in 17 (23.6%), 16 (22.2%), 9 (17.3%), 14 (19.4%) and 4 (5.6%) cases, respe
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47

Metanmo, Salvatore, Farid Boumédiène, Pierre-Marie Preux, et al. "First description of Nodding Syndrome in the Central African Republic." PLOS Neglected Tropical Diseases 15, no. 6 (2021): e0009430. http://dx.doi.org/10.1371/journal.pntd.0009430.

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Background The term Nodding Syndrome (NS) refers to an atypical and severe form of childhood epilepsy characterized by a repetitive head nodding (HN). The disease has been for a long time limited to East Africa, and the cause is still unknown. The objective of this study was to confirm the existence of NS cases in Central African Republic (CAR). Methodology/Principal findings This was a cross-sectional descriptive study in the general population. The identification of NS cases was conducted through a door-to-door survey in a village near Bangui along the Ubangui River. Based on Winkler’s 2008
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48

Kitara, David, Suzanne Gazda, Eger Ambrose, et al. "Nodding Episodes and High Anion Gap in a 13 Year Old Child with Nodding Syndrome: A Case Report." British Journal of Medicine and Medical Research 6, no. 8 (2015): 851–58. http://dx.doi.org/10.9734/bjmmr/2015/14595.

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49

Pollanen, MS, and S. Onzivua. "Nodding syndrome, an epidemic young-onset epilepsy-dementia complex in Uganda." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 48, s1 (2021): S5. http://dx.doi.org/10.1017/cjn.2021.96.

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Nodding syndrome (NS) is an enigmatic recurrent epidemic neurological disease that affects children in East Africa. The illness begins with nodding of the head and grand mal seizures that may lead to death after several years. The most recent outbreaks of NS occurred in northern Uganda and South Sudan. We describe the clinicopathologic spectrum of NS in Uganda. Ten children or young adults with NS were studied at autopsy and the neuropathological findings correlated with the onset, duration and progression of their neurological illness. All cases had epilepsy with grand mal seizures. Three cas
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50

Ongaya, Kizito, Augustus Aturinde, Mahdi Farnaghi, et al. "Spatiotemporal Analysis of Nodding Syndrome in Northern Uganda 1990-2014." Health 12, no. 02 (2020): 180–93. http://dx.doi.org/10.4236/health.2020.122015.

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