Vitaliani Roberta, Scaravilli Tomaso, Egarter-Vigl Eduard, Giometto Bruno, Klein Christine, Scaravilli Francesco, An Shu F, Pramstaller Peter P
Clinica Neurologica 2a, Università degli Studi di Padova, Padua, Italy.
J Neuropathol Exp Neurol. 2002 Mar;61(3):268-74. doi: 10.1093/jnen/61.3.268.
We describe the results of a study of the spinal cord of 5 patients with progressive supranuclear palsy (PSP). Examination of the 6th cervical, 7th thoracic, and 5th lumbar segments revealed variable degree of gliosis and density of neuropil threads (NTs), nerve cell loss, and tau-positive cytoplasmic staining of neurons, some of which was reminiscent of neurofibrillary tangles (NFT). Tau-positive neurons were seen at each spinal level and in the 3 zones in which each level was subdivided. Cells with the appearance of NFT predominated in the intermediate zone. Morphometric study revealed 47%, 52%, and 32% decrease in cell numbers in the motor area (lamina IX) at the 3 spinal levels, respectively, and 39% in the intermedio-lateral column. This is the first report describing severe neuronal loss throughout the whole spinal cord in patients with PSP and its results are in keeping with a previous study of the nucleus of Onufrowicz. The reasons why cell loss fails to produce clinical symptoms are analyzed and the clinico-pathological correlations between anatomical changes and dystonia are considered. On the basis of existing data, we conclude that previous suggestions implicating spinal interneurons in the pathogenesis of neck dystonia should not be supported.
我们描述了对5例进行性核上性麻痹（PSP）患者脊髓的研究结果。对第6颈椎、第7胸椎和第5腰椎节段的检查发现，胶质增生程度、神经毡丝（NTs）密度、神经细胞丢失以及神经元tau阳性细胞质染色情况各不相同，其中一些类似于神经原纤维缠结（NFT）。在每个脊髓节段以及每个节段细分的3个区域均可见到tau阳性神经元。具有NFT外观的细胞在中间区域占主导。形态计量学研究显示，在3个脊髓节段的运动区（第IX层），细胞数量分别减少了47%、52%和32%，中间外侧柱细胞数量减少了39%。这是首篇描述PSP患者整个脊髓严重神经元丢失的报告，其结果与之前对Onufrowicz核的研究一致。分析了细胞丢失未产生临床症状的原因，并探讨了解剖学变化与肌张力障碍之间的临床病理相关性。基于现有数据，我们得出结论，之前认为脊髓中间神经元参与颈部肌张力障碍发病机制的观点不应得到支持。
