Abstract
Nodding syndrome is an epidemic neurologic disorder of unknown cause that affects children in the subsistence-farming communities of East Africa. We report the neuropathologic findings in five fatal cases (13-18 years of age at death) of nodding syndrome from the Acholi people in northern Uganda. Neuropathologic examination revealed tau-immunoreactive neuronal neurofibrillary tangles, pre-tangles, neuropil threads, and dot-like lesions involving the cerebral cortex, subcortical nuclei and brainstem. There was preferential involvement of the frontal and temporal lobes in a patchy distribution, mostly involving the crests of gyri and the superficial cortical lamina. The mesencephalopontine tegmental nuclei, substantia nigra, and locus coeruleus revealed globose neurofibrillary tangles and threads. We conclude that nodding syndrome is a tauopathy and may represent a newly recognized neurodegenerative disease.
References
May 1, 1991·Neurology·P S SpencerA C Ludolph
Mar 29, 2001·Acta Neuropathologica·T AraiP L McGeer
Dec 1, 1961·Brain : a Journal of Neurology·A HIRANOL T KURLAND
May 28, 2008·Epilepsia·Andrea S WinklerErich Schmutzhard
Jun 19, 2009·Journal of Neuropathology and Experimental Neurology·Ann C McKeeRobert A Stern
Jul 2, 2011·Journal of Molecular Neuroscience : MN·Dennis W DicksonKeith A Josephs
Sep 10, 2011·Brain : a Journal of Neurology·Maria ThomSanjay M Sisodiya
Jan 12, 2013·Lancet Neurology·James J SejvarDkw Lwamafa
May 7, 2013·BMJ Open·Richard IdroJames K Tumwine
Jul 5, 2013·PloS One·Jennifer L FoltzD K W Lwamafa
Aug 24, 2013·Emerging Infectious Diseases·Scott F DowellAnthony K Mbonye
Nov 16, 2013·African Health Sciences·S MusisiJ Okello
Nov 6, 2014·BMJ Open·Jesa L LandisPeter S Spencer
Sep 13, 2015·Seizure : the Journal of the British Epilepsy Association·Gianni de PoloRenato Borgatti
Dec 17, 2015·Acta Neuropathologica·Ann C McKeeUNKNOWN TBI/CTE group
Aug 9, 2016·Brain : a Journal of Neurology·Xin You TaiMaria Thom
Sep 23, 2016·Journal of the Neurological Sciences·Peter S SpencerUNKNOWN other Members of the Oregon-Uganda Nodding Syndrome Research Team
Feb 17, 2017·Science Translational Medicine·Tory P JohnsonAvindra Nath
Mar 25, 2017·Journal of Neuropathology and Experimental Neurology·Gabor G KovacsJohn Q Trojanowski
Jun 8, 2017·Journal of Neuropathology and Experimental Neurology·Gabor G KovacsJohn Q Trojanowski
Oct 25, 2017·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Maya MimuroYasumasa Kokubo
Citations
Sep 26, 2019·Journal of Neuropathology and Experimental Neurology·An HotterbeekxRobert Colebunders
Jan 25, 2020·Annual Review of Pathology·Tory P JohnsonAvindra Nath
Dec 24, 2019·Neuropathology and Applied Neurobiology·Z Jaunmuktane, S Brandner
Jun 21, 2019·PLoS Neglected Tropical Diseases·Julia IraniSarah O'Neill
Jul 9, 2020·PLoS Neglected Tropical Diseases·Gil BenedekMia Levite
Sep 29, 2018·Nature Reviews. Neurology·Charlotte Ridler
Mar 3, 2020·Infectious Diseases of Poverty·Nolbert GumisirizaRobert Colebunders
Sep 22, 2020·Brain Communications·Sam OlumNeil J Scolding
Jun 18, 2019·The Pediatric Infectious Disease Journal·Gasim Abd-Elfarag, Michaël Boele van Hensbroek
Aug 11, 2020·Movement Disorders Clinical Practice·Eoin MulroyKailash P Bhatia
Dec 7, 2019·Global Mental Health·D Geelhand de MerxemR Colebunders
Jan 8, 2021·PLoS Neglected Tropical Diseases·Robert ColebundersJoseph Nelson Siewe Fodjo
Jan 28, 2021·Pathogens·An HotterbeekxRobert Colebunders
Nov 23, 2019·Revue neurologique·P S SpencerM S Pollanen
May 4, 2020·Revue neurologique·P S SpencerM S Pollanen
Jan 19, 2021·Journal of Alzheimer's Disease : JAD·Eloi Magnin
Dec 17, 2020·Pathogens·David W NauenTory P Johnson
Dec 1, 2020·Frontiers in Neurology·Jacky Ganguly, Mandar Jog
Apr 4, 2021·Pathogens·Melissa Krizia VieriRobert Colebunders
May 1, 2021·Toxins·Jennifer DuringerPeter Spencer
May 18, 2021·Frontiers in Cellular and Infection Microbiology·An HotterbeekxSamir Kumar-Singh
Jul 6, 2021·Journal of Neural Transmission·Krisztina DanicsGabor G Kovacs