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Nodding syndrome in Uganda is a tauopathy.

Open access
Pollanen, Michael S., Onzivua, Sylvester, Robertson, Janice, McKeever, Paul M., Olawa, Francis, Kitara, David L., Fong, Amanda·2018·Acta Neuropathologica
DOI 10.1007/s00401-018-1909-9PMC PMC6208726
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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.

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  • P-tau pathology is found in patients with temporal lobe epilepsy, Dravet syndrome, Nodding syndrome, Niemann-Pick type C disease, focal cortical dysplasia IIB, and tuberous sclerosis complex, as well as animal models of post-traumatic epilepsy, Lafora disease, and autism spectrum disorder.

    “These include patients with TLE, Dravet syndrome, Nodding syndrome (Thom et al., 2011; Pollanen et al., 2018; Hotterbeekx et al., 2019), Niemann-Pick type C disease (NPC) (Auer et al., 1995; Love et al., 1995; Suzuki et al., 1995; Malnar et al., 2014), focal cortical dysplasia IIB (FCDIIb) (Sen et al., 2007; Iyer et al., 2014), and tuberous sclerosis complex (TSC) (Sarnat and Flores-Sarnat, 2015;”

    From Tauopathy and Epilepsy Comorbidities and Underlying Mechanisms (2022) · cites this paper as Pollanen et al., 2018

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Research works printed but not linked: 23

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Authors
Pollanen, M. S., Onzivua, S., Robertson, J., McKeever, P. M., Olawa, F., Kitara, D. L., & Fong, A.
Affiliations
  • University of Toronto (CA)
  • Makerere University (UG)
  • Gulu University (UG)
Corresponding author
Pollanen, Michael S.
ORCID
Venue
Acta Neuropathologica
Publisher
Springer Science and Business Media LLC
Published
2018-09-15
Volume
136
Issue
5
Pages
691-697
ISSN
  • 0001-6322 (ISSN-L)
  • 1432-0533
DOI
10.1007/s00401-018-1909-9
PMC
PMC6208726
OpenAlex
W2890168286
Cited by
54
References
1
Citations by year
2026: 2 · 2025: 1 · 2024: 7 · 2023: 4 · 2022: 10 · 2021: 10 · 2020: 10 · 2019: 8 · 2018: 2
FWCI
5.16
Type
journal article
Language
en
Open access
Licence
Locations
  • Acta Neuropathologica (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Keywords
nodding syndrome, tauopathy, neurofibrillary tangles, neuropil threads, Acholi people, pediatric neurodegenerative disease, substantia nigra, locus coeruleus, Uganda
MeSH terms
Adolescent, Brain, Female, Humans, Male, Uganda, Neurofibrillary Tangles, Tauopathies, Nodding Syndrome

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