Pathological and Clinical Spectrum of Progressive Supranuclear Palsy: With Special Reference to Astrocytic Tau Pathology.
Pathological and Clinical Spectrum of Progressive Supranuclear Palsy: With Special Reference to Astrocytic Tau Pathology.
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进行性核上性麻痹的病理学和临床谱：特别参考星形细胞 Tau 病理学。
DOI：
10.1111/bpa.12265
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发表时间：
2016
期刊：
影响因子：
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通讯作者：
Takahashi H.
中科院分区：
文献类型：
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作者：
Yokoyama Y;Toyoshima Y;Shiga A;Tada M;Kitamura H;Hasegawa K;Onodera O;Ikeuchi T;Someya T;Nishizawa M;Kakita A;Takahashi H.
进行性核上性麻痹（PSP）是一种四重复tau蛋白病，具有tau蛋白阳性，嗜银簇状星形胶质细胞（TA）。我们对40例病理诊断为PSP或PSP样疾病的连续尸检日本患者进行了病理和临床研究。22例病例中存在明确的TA，所有这些病例均被确认为PSP。在其他18例病例中几乎未检测到此类TA，而是表现出tau阳性嗜银星形胶质细胞，表现为相对较小的簇，中央核具有不规则形状的粗糙结构（可疑TA）。对这18例病例的tau相关病理分布模式进行聚类分析，确定了两个亚组，苍白球黑质路易体萎缩（PNLA）1型（n = 9）和2型（n = 9），前者与后者的区别在于除了严重受累的PNL系统外，运动皮质、脑桥核和小脑齿状核中还存在tau相关病变。在PNLAT1型中，从症状发作到需要坐轮椅的持续时间显著更长。来自三种疾病的样品的免疫印迹显示了在约35 kDa处的低分子量tau片段的带型。这些发现进一步阐明了四重复tau蛋白病的广泛病理和临床谱，代表广义的PSP而不是经典的PSP。
Progressive supranuclear palsy (PSP) is a four‐repeat tauopathy with tau‐positive, argyrophilic tuft‐shaped astrocytes (TAs). We performed a pathological and clinical investigation in 40 consecutive autopsied Japanese patients with pathological diagnoses of PSP or PSP‐like disease. Unequivocal TAs were present in 22 cases, all of which were confirmed to be PSP. Such TAs were hardly detected in the other 18 cases, which instead exhibited tau‐positive, argyrophilic astrocytes, appearing as comparatively small clusters with central nuclei of irregularly shaped, coarse structures (equivocal TAs). Cluster analysis of the distribution pattern of tau‐related pathology for these 18 cases identified two subgroups, pallido‐nigro‐luysian atrophy (PNLA)Type 1(n = 9) andType 2(n = 9), the former being distinguished from the latter by the presence of tau‐related lesions in the motor cortex, pontine nucleus and cerebellar dentate nucleus in addition to the severely affected PNL system. The duration from symptom onset until becoming wheelchair‐bound was significantly longer in PNLAType 1. Immunoblotting of samples from the three disease conditions revealed band patterns of low‐molecular‐mass tau fragments at ∼35 kDa. These findings shed further light on the wide pathological and clinical spectrum of four‐repeat tauopathy, representing PSP in the broad sense rather than classical PSP.