{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T13:00:24Z","timestamp":1772283624250,"version":"3.50.1"},"reference-count":18,"publisher":"IMR Press","issue":"2","license":[{"start":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T00:00:00Z","timestamp":1772064000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rev. Neurol."],"abstract":"<jats:p>Background: Nodding syndrome is a childhood-onset epileptic encephalopathy described in onchocerciasis-endemic regions of sub-Saharan Africa. Although characterized by recurrent atonic seizures with repetitive head-nodding movements, increasing evidence suggests that the condition extends beyond a purely motor epilepsy and involves progressive cognitive, behavioral, and emotional impairment. Methods: This study was designed as a narrative review with a specific neuropsychological focus. A literature search was conducted in PubMed, Scopus, and Google Scholar for publications between 2013 and 2025. Studies describing cognitive, behavioral, emotional, psychiatric, neuroimaging, or neuropathological findings in patients with nodding syndrome were included. Due to heterogeneity in study design and assessment methods, findings were synthesized narratively. Results: Across studies, nodding syndrome is consistently associated with progressive cognitive decline affecting attention, processing speed, executive functions, memory, and global intellectual functioning. Behavioral disturbances such as irritability, aggression, and emotional lability are frequently reported, alongside depressive symptoms and social withdrawal. Neuroimaging findings commonly demonstrate cortical and cerebellar atrophy, while neuropathological studies report tau-protein deposition and neuronal loss, supporting diffuse cerebral involvement with possible neurodegenerative features. Conclusions: Nodding syndrome represents a complex epileptic encephalopathy characterized not only by seizures but also by significant cognitive, behavioral, and emotional impairment. A clearer neuropsychological characterization may inform clinical assessment and guide future research aimed at improving supportive and rehabilitative interventions.<\/jats:p>","DOI":"10.31083\/rn47379","type":"journal-article","created":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T12:11:13Z","timestamp":1772280673000},"source":"Crossref","is-referenced-by-count":0,"title":["Cognitive, Behavioral, and Emotional Manifestations in Nodding Syndrome: A Neuropsychological Narrative Review"],"prefix":"10.31083","volume":"81","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5924-0100","authenticated-orcid":true,"given":"Ana Cristina","family":"de Castro","sequence":"first","affiliation":[{"name":"Department of Nursing, Faculty of Medicine and Health Sciences, Universidad de Alcal\u00e1, 28801 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7275-6742","authenticated-orcid":true,"given":"Mar\u00eda J.","family":"Garc\u00eda-Rubio","sequence":"additional","affiliation":[{"name":"Global Neuroscience and Social Change, Universidad Internacional de Valencia, 46002 Valencia, Spain"},{"name":"Cognition, Affect and Resilience (CARE) Research Group, Health Sciences Faculty, Universidad Internacional de Valencia, 46002 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"16242","published-online":{"date-parts":[[2026,2,26]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","unstructured":"Winkler AS, Friedrich K, K\u00f6nig R, Meindl M, Helbok R, Unterberger I, et al. The head nodding syndrome\u2014clinical classification and possible causes. Epilepsia. 2008; 49: 2008\u20132015.","DOI":"10.1111\/j.1528-1167.2008.01671.x"},{"key":"ref2","doi-asserted-by":"publisher","unstructured":"Idro R, Opar B, Wamala J, Abbo C, Onzivua S, Mwaka AD, et al. Nodding syndrome in Ugandan children\u2014clinical features, brain imaging, and complications: a case series. BMJ Open. 2016; 6: e009171.","DOI":"10.1136\/bmjopen-2012-002540"},{"key":"ref3","doi-asserted-by":"publisher","unstructured":"Ridler C. Nodding syndrome discovered to be a tauopathy. Nature Reviews. Neurology. 2018; 14: 632.","DOI":"10.1038\/s41582-018-0086-8"},{"key":"ref4","doi-asserted-by":"publisher","unstructured":"Idro R, Musubire KA, Byamah Mutamba B, Namusoke H, Muron J, Abbo C, et al. Proposed guidelines for the management of nodding syndrome. African Health Sciences. 2013; 13: 219\u2013232.","DOI":"10.4314\/ahs.v13i2.4"},{"key":"ref5","doi-asserted-by":"publisher","unstructured":"De Castro AC, Ortega-Deballon I. Nodding syndrome: bridging the gap-a scoping review protocol. BMJ Open. 2020; 10: e035269.","DOI":"10.1136\/bmjopen-2019-035269"},{"key":"ref6","doi-asserted-by":"publisher","unstructured":"Dowell SF, Sejvar JJ, Riek L, Vandemaele KAH, Lamunu M, Kuesel AC, et al. Nodding syndrome. Emerging Infectious Diseases. 2013; 19: 1374\u20131384.","DOI":"10.3201\/eid1909.130401"},{"key":"ref7","doi-asserted-by":"publisher","unstructured":"Idro R, Namusoke H, Abbo C, Mutamba BB, Kakooza-Mwesige A, Opoka RO, et al. Patients with nodding syndrome in Uganda improve with symptomatic treatment: a cross-sectional study. BMJ Open. 2014; 4: e006476.","DOI":"10.1136\/bmjopen-2014-006476"},{"key":"ref8","doi-asserted-by":"publisher","unstructured":"Abd-Elfarag GOE, Edridge AWD, Spijker R, Sebit MB, van Hensbroek MB. Nodding Syndrome: A Scoping Review. Tropical Medicine and Infectious Disease. 2021; 6: 211.","DOI":"10.3390\/tropicalmed6040211"},{"key":"ref9","doi-asserted-by":"publisher","unstructured":"Kegele J, Wagner T, Kowenski T, Wiesmayr M, Gatterer C, Alber M, et al. Long-term clinical course and treatment outcomes of individuals with Nodding Syndrome. Journal of the Neurological Sciences. 2024; 457: 122893.","DOI":"10.1016\/j.jns.2024.122893"},{"key":"ref10","doi-asserted-by":"publisher","unstructured":"Morin A, Guillaume M, Ngarka L, Tatah GY, Siewe Fodjo JN, Wyart G, et al. Epilepsy in the Sanaga-Mbam valley, an onchocerciasis-endemic region in Cameroon: electroclinical and neuropsychological findings. Epilepsia Open. 2021; 6: 513\u2013527.","DOI":"10.1002\/epi4.12510"},{"key":"ref11","doi-asserted-by":"publisher","unstructured":"Stacey HJ, Woodhouse L, Welburn SC, Jones JD. Aetiologies and therapies of nodding syndrome: a systematic review and meta-analysis. Journal of Global Health Reports. 2019; 3: e2019066.","DOI":"10.29392\/joghr.3.e2019066"},{"key":"ref12","doi-asserted-by":"publisher","unstructured":"Olum S, Scolding P, Hardy C, Obol J, Scolding NJ. Nodding syndrome: a concise review. Brain Communications. 2020; 2: fcaa037.","DOI":"10.1093\/braincomms\/fcaa037"},{"key":"ref13","doi-asserted-by":"publisher","unstructured":"Abd-Elfarag GOE, Mathewson JD, Emmanuel L, Edridge AWD, van Beers S, Sebit MB, et al. Nodding Syndrome: Clinical Characteristics, Risks Factors, Access to Treatment, and Perceptions in the Greater Mundri Area, South Sudan. Pathogens (Basel, Switzerland). 2023; 12: 190.","DOI":"10.3390\/pathogens12020190"},{"key":"ref14","doi-asserted-by":"publisher","unstructured":"Mazumder R, Lubowa SK, Salamon N, Jackson NJ, Kawooya M, Akun PR, et al. Comparison of Structural Changes in Nodding Syndrome and Other Epilepsies Associated With Onchocerca volvulus. Neurology(R) Neuroimmunology & Neuroinflammation. 2022; 10: e200074.","DOI":"10.1212\/NXI.0000000000200074"},{"key":"ref15","doi-asserted-by":"publisher","unstructured":"de Polo G, Romaniello R, Otim A, Benjamin K, Bonanni P, Borgatti R. Neurophysiological and clinical findings on Nodding Syndrome in 21 South Sudanese children and a review of the literature. Seizure. 2015; 31: 64\u201371.","DOI":"10.1016\/j.seizure.2015.07.006"},{"key":"ref16","doi-asserted-by":"publisher","unstructured":"Pollanen MS, Onzivua S, McKeever PM, Robertson J, Mackenzie IR, Kovacs GG, et al. The spectrum of disease and tau pathology of nodding syndrome in Uganda. Brain: a Journal of Neurology. 2023; 146: 954\u2013967.","DOI":"10.1093\/brain\/awac137"},{"key":"ref17","doi-asserted-by":"publisher","unstructured":"Colebunders R, Hendy A, Nanyunja M, Wamala JF, van Oijen M. Nodding syndrome-a new hypothesis and new direction for research. International Journal of Infectious Diseases: IJID: Official Publication of the International Society for Infectious Diseases. 2014; 27: 74\u201377.","DOI":"10.1016\/j.ijid.2014.08.001"},{"key":"ref18","doi-asserted-by":"publisher","unstructured":"Spencer PS, Valdes Angues R, Palmer VS. Nodding syndrome: A role for environmental biotoxins that dysregulate MECP2 expression? Journal of the Neurological Sciences. 2024; 462: 123077.","DOI":"10.1016\/j.jns.2024.123077"}],"container-title":["Revista de Neurolog\u00eda"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.imrpress.com\/journal\/RN\/81\/2\/10.31083\/RN47379","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T12:11:15Z","timestamp":1772280675000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.imrpress.com\/journal\/RN\/81\/2\/10.31083\/RN47379"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,26]]},"references-count":18,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026]]}},"alternative-id":["S0210-0010(26)02190-0"],"URL":"https:\/\/doi.org\/10.31083\/rn47379","relation":{},"ISSN":["0210-0010","1576-6578"],"issn-type":[{"value":"0210-0010","type":"print"},{"value":"1576-6578","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,26]]},"article-number":"47379"}}