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Nodding syndrome in Uganda is a tauopathy
Michael S Pollanen1,2, Sylvester Onzivua3, Janice Robertson4
1Department of Pathobiology and Laboratory Medicine, University of Toronto, Toronto, ON, Canada. Michael.Pollanen@ontario.ca.
Acta Neuropathologica
|September 17, 2018
Summary
Nodding syndrome, a mysterious neurologic disorder in East African children, is characterized by tau pathology in the brain. This suggests it is a newly identified tauopathy, a class of neurodegenerative diseases.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
- Pediatric Neurology
Background:
- Nodding syndrome is an epidemic neurologic disorder affecting children in East African subsistence-farming communities.
- The cause of nodding syndrome remains unknown.
Observation:
- This study examined five fatal cases of nodding syndrome in children (13-18 years) from the Acholi people in northern Uganda.
- Neuropathologic examination revealed tau-immunoreactive neurofibrillary tangles, pre-tangles, neuropil threads, and dot-like lesions.
Findings:
- The lesions were found in the cerebral cortex, subcortical nuclei, and brainstem, with preferential, patchy involvement of frontal and temporal lobes.
- Specific brain regions like the mesencephalopontine tegmental nuclei, substantia nigra, and locus coeruleus showed globose neurofibrillary tangles and threads.
Implications:
- The findings strongly indicate that nodding syndrome is a tauopathy.
- Nodding syndrome may represent a newly recognized neurodegenerative disease, necessitating further research into its etiology and pathogenesis.
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