Department of Pathobiology and Laboratory Medicine, University of Toronto, Toronto, ON, Canada.
Ontario Forensic Pathology Service, Toronto, ON, Canada.
Acta Neuropathol. 2018 Nov;136(5):691-697. doi: 10.1007/s00401-018-1909-9. Epub 2018 Sep 15.
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.
点头综合征是一种病因不明的流行神经系统疾病，影响东非自给农业社区的儿童。我们报告了来自乌干达北部阿乔利人的 5 例点头综合征死亡病例（死亡时年龄为 13-18 岁）的神经病理学发现。神经病理学检查显示 Tau 免疫反应性神经元神经原纤维缠结、前缠结、神经丝和点状病变，涉及大脑皮层、皮质下核和脑干。额叶和颞叶呈斑片状分布，优先受累，主要累及脑回的隆起和皮质浅层。中脑脑桥被盖核、黑质和蓝斑显示出球状神经原纤维缠结和神经丝。我们得出结论，点头综合征是一种 Tau 病，可能代表一种新发现的神经退行性疾病。
