Nodding syndrome in Uganda is a tauopathy

Michael S Pollanen,Francis Olawa,Sylvester Onzivua,David L Kitara,Amanda Fong,Janice Robertson,Paul M Mckeever

doi:10.1007/s00401-018-1909-9

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.

Highlights

Nodding syndrome (NS) is a neurologic disorder of children in East Africa
NS has been documented in the Republic of South Sudan [2, 20, 23, 24] and Tanzania [19], it is currently a major health problem in the subsistence-farming villages of the Acholi people in northern Uganda [5, 7, 16]
We present histologic evidence the NS is a novel tauopathy

Summary

Introduction

Nodding syndrome (NS) is a neurologic disorder of children in East Africa. NS is characterized by stereotypical head dropping movements, cognitive impairment, impaired growth, and seizures [2, 4, 5, 7, 16, 19, 23, 24]. NS has been documented in the Republic of South Sudan [2, 20, 23, 24] and Tanzania [19], it is currently a major health problem in the subsistence-farming villages of the Acholi people in northern Uganda [5, 7, 16]. NS emerged as an epidemic in the Kitgum district of Uganda in 1998 [5, 11] during internal armed conflict and displacement of children into camps.

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Conclusion