Hauw J J, Verny M, Delaère P, Cervera P, He Y, Duyckaerts C
Laboratorie de Neuropathologie R. Escourolle, FRA Neuropathologie Expérimentale et Clinique Claude Bernard, Hôpital de la Salpêtrière, Paris, France.
Neurosci Lett. 1990 Nov 13;119(2):182-6. doi: 10.1016/0304-3940(90)90829-x.
Neocortical neurofibrillary tangles (NFT) revealed by Bodian technique and anti-tau immunolabelling were seen in 5/5 cases of progressive supranuclear palsy (PSP) aged 58-76 years. These lesions differed from Alzheimer's disease or age-related changes: (1) they were most frequent in the precentral gyrus (Brodmann's area 4) whereas associative areas are predominantly lesioned in Alzheimer's disease; (2) they affected mainly large pyramidal neurons and small cells, relatively sparing the cell population selectively involved in Alzheimer's disease; (3) they predominated in layers V and VI of area 4, whereas NFT are most dense in layers III and V in Alzheimer's disease; (4) mature senile plaques (1/5 cases) and beta-amyloid diffuse deposits (3/5 cases), which usually precede or go together with NFT in Alzheimer's disease were rare or absent (2/5) in PSP. Neuropil threads and tufts of abnormal fibres were also seen. In addition, NFT and neuropil threads were found in the hippocampus. PSP is thus another example of abnormal storage of tau developing in the neocortex in the absence of beta-amyloid deposits. It might prove a useful model for the understanding of the mechanisms of localization and spreading of tau storage in the brain.
通过博迪安技术和抗tau免疫标记显示，在5例年龄为58 - 76岁的进行性核上性麻痹（PSP）患者中均发现了新皮质神经原纤维缠结（NFT）。这些病变与阿尔茨海默病或年龄相关变化不同：（1）它们在中央前回（布罗德曼第4区）最为常见，而在阿尔茨海默病中主要受损的是联合区；（2）它们主要影响大锥体神经元和小细胞，相对较少累及阿尔茨海默病中选择性受累的细胞群体；（3）它们在第4区的V层和VI层占主导，而在阿尔茨海默病中NFT在III层和V层最为密集；（4）成熟的老年斑（1/5例）和β - 淀粉样蛋白弥漫性沉积（3/5例），在阿尔茨海默病中通常先于NFT出现或与NFT同时存在，在PSP中则罕见或不存在（2/5）。还可见神经毡丝和异常纤维束。此外，在海马体中也发现了NFT和神经毡丝。因此，PSP是在没有β - 淀粉样蛋白沉积的情况下新皮质中tau异常蓄积的另一个例子。它可能是理解tau在大脑中蓄积的定位和扩散机制的有用模型。
