Mackenzie I R, Hudson L P
Department of Pathology (Neuropathology), University of Western Ontario, London, Canada.
Acta Neuropathol. 1995;90(6):615-9. doi: 10.1007/BF00318574.
Achromatic neurons (AN) in the cerebral cortex are a characteristic feature of several neurodegenerative conditions including Pick's disease, corticobasal degeneration and some cases of primary progressive aphasia. Although AN are occasionally seen in some other diseases, their presence in progressive supranuclear palsy (PSP) has not been previously documented. We found significant numbers of AN in the cerebral cortex of five out of seven cases which had been diagnosed pathologically as PSP. The identification of AN was greatly facilitated by the use of neurofilament immunohistochemistry. The entorhinal and transentorhinal cortices were most frequently involved, but in several cases AN were also seen throughout the neocortex. The presence and number of AN roughly correlated with a history of clinical dementia. This suggests that cortical AN may be a common and important pathological finding in PSP.
大脑皮层中的无色神经元(AN)是包括Pick病、皮质基底节变性和某些原发性进行性失语病例在内的几种神经退行性疾病的特征性表现。虽然AN偶尔也见于其他一些疾病,但它们在进行性核上性麻痹(PSP)中的存在此前尚未有文献记载。我们在7例经病理诊断为PSP的病例中的5例大脑皮层中发现了大量的AN。神经丝免疫组织化学的应用极大地促进了AN的识别。内嗅皮质和跨内嗅皮质最常受累,但在一些病例中,整个新皮质也可见到AN。AN的存在和数量与临床痴呆病史大致相关。这表明皮质AN可能是PSP中常见且重要的病理表现。