Iwasaki Yasushi, Yoshida Mari, Hashizume Yoshio, Hattori Manabu, Aiba Ikuko, Sobue Gen
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan.
Neuropathology. 2007 Aug;27(4):331-40. doi: 10.1111/j.1440-1789.2007.00787.x.
We describe the histopathologic features of spinal cord lesions in 10 cases of progressive supranuclear palsy (PSP) and review the literature. Histologic examination revealed atrophy with myelin pallor in the anterior funiculus and anterolateral funiculus in the cervical and thoracic segments in eight of the 10 cases, whereas the posterior funiculus was well preserved. The degrees of atrophy of the anterior funiculus and the anterolateral funiculus correlated with that of the tegmentum of the medulla oblongata. Myelin pallor of the lateral corticospinal tract was observed in two of the 10 cases. Microscopic observation of the spinal white matter, particularly the cervical segment, revealed a few to several neuropil threads, particularly in the white matter surrounding the anterior horn after Gallyas-Braak (GB) staining or AT-8 tau immunostaining. However, the posterior funiculus was completely preserved from the presence of argyrophilic or tau-positive structures. In the spinal gray matter, widespread distribution of neurons with cytoplasmic inclusions and neuropil threads was observed, particularly in the medial division of the anterior horn and intermediate gray matter, especially in the cervical segment. Globose-type neurofibrillary tangles and pretangles were found. The distribution of GB- or AT-8 tau-positive small neurons and neuropil threads resembled that of the spinal interneurons. In conclusion, the spinal cord, especially the cervical segment, is constantly involved in the pathologic process of PSP. We speculate that spinal interneurons and their neuronal processes, particularly in the medial division of the anterior horn and intermediate gray matter of the cervical segment, are most severely damaged in the PSP spinal cord.
我们描述了10例进行性核上性麻痹（PSP）患者脊髓病变的组织病理学特征，并对相关文献进行了综述。组织学检查显示，10例患者中有8例在颈段和胸段的前索和前外侧索出现萎缩伴髓鞘苍白，而后索保存完好。前索和前外侧索的萎缩程度与延髓被盖的萎缩程度相关。10例患者中有2例观察到皮质脊髓侧束髓鞘苍白。对脊髓白质，尤其是颈段进行显微镜观察，在Gallyas-Braak（GB）染色或AT-8 tau免疫染色后，发现白质中有少量至数条神经毡丝，尤其是在前角周围的白质中。然而，后索未出现嗜银或tau阳性结构，完全保存完好。在脊髓灰质中，观察到有胞质内包涵体和神经毡丝的神经元广泛分布，尤其是在前角内侧部和中间带灰质，特别是在颈段。发现了球状神经原纤维缠结和前缠结。GB或AT-8 tau阳性小神经元和神经毡丝的分布与脊髓中间神经元相似。总之，脊髓，尤其是颈段，经常参与PSP的病理过程。我们推测，在PSP脊髓中，脊髓中间神经元及其神经突，尤其是颈段前角内侧部和中间带灰质中的神经突，受损最为严重。
