Nodding syndrome in Uganda is a tauopathy

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. Ne...

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Bibliographic Details
Published in:Acta neuropathologica 2018-11, Vol.136 (5), p.691-697
Main Authors: Pollanen, Michael S., Onzivua, Sylvester, Robertson, Janice, McKeever, Paul M., Olawa, Francis, Kitara, David L., Fong, Amanda
Format: Article
Language:English
Subjects:
Brain
Epidemics
Medicine
Medicine & Public Health
Nervous system diseases
Neurons
Neuropathology
Neurosciences
Original Paper
Pathology
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Summary: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.
ISSN:0001-6322
1432-0533
DOI:10.1007/s00401-018-1909-9