Oyanagi K, Makifuchi T, Ohtoh T, Chen K M, Gajdusek D C, Chase T N, Ikuta F
Center for Materials of Brain Diseases, Niigata University, Japan.
Acta Neuropathol. 1994;88(2):122-8. doi: 10.1007/BF00294504.
The neostriatum, nucleus accumbens and basal nucleus of Meynert (bnM) in the parkinsonism-dementia complex of Guam (Guam PDC) were examined immunohistologically, ultrastructurally, quantitatively and topographically, and the results were compared with those in Alzheimer's disease (AD) and progressive supranuclear palsy (PSP). Compared to neurologically normal controls, the number of large neurons in Guam PDC was reduced by approximately 70% in the caudate nucleus and putamen and by more than 90% in the nucleus accumbens. The decreased number of large neurons in the neostriatum was significantly correlated to that in the bnM. The remaining large neurons and many of the medium-sized neurons in the neostriatum and nucleus accumbens were immunopositive for tau protein and contained varying amounts of 21- to 25-nm-wide paired helical filaments (PHFs) admixed with straight tubules. Curly fibers and circularly arranged reactive astrocytes were seen in the nucleus accumbens of many PDC patients. Collectively, these findings, which are similar in part to those of AD and differ from those of PSP, suggest that the large neurons in the neostriatum and nucleus accumbens in Guam PDC degenerate through PHF formation, and that extremely severe loss of large neurons in the nucleus accumbens may be linked to marked degeneration of the limbic and ventral tegmental areas and nucleus dorsal raphe.
对关岛帕金森痴呆综合征（关岛PDC）患者的新纹状体、伏隔核和迈内特基底核（bnM）进行了免疫组织化学、超微结构、定量和拓扑学检查，并将结果与阿尔茨海默病（AD）和进行性核上性麻痹（PSP）患者的结果进行了比较。与神经功能正常的对照组相比，关岛PDC患者尾状核和壳核中的大神经元数量减少了约70%，伏隔核中的大神经元数量减少了90%以上。新纹状体中大神经元数量的减少与bnM中的减少显著相关。新纹状体和伏隔核中剩余的大神经元以及许多中型神经元对tau蛋白呈免疫阳性，并含有不同数量的21至25纳米宽的双螺旋丝（PHF），与直小管混合存在。在许多PDC患者的伏隔核中可见卷曲纤维和呈环状排列的反应性星形胶质细胞。总体而言，这些部分与AD相似且与PSP不同的发现表明，关岛PDC患者新纹状体和伏隔核中的大神经元通过PHF形成而退化，伏隔核中大神经元的极度严重丧失可能与边缘叶、腹侧被盖区和中缝背核的明显退化有关。
