Probst A
Rev Neurol (Paris). 1977 Jun-Jul;133(6-7):417-28.
A case with wide-spread subcortical neurofibrillary changes, cell loss and gliosis in a 81 year old man is described. The most affected areas were both pallida, Luys subthalamic bodies, the substantia nigra and the denate nuclei of the cerebellum. The cerebral cortex contained only a few senile plaques. We interpreted this case as a variety of progressive supranuclear palsy despite the lack of the typical supranuclear ophtalmoplegia and of superior colliculi involvement. In the subthalamic body and in the substantia nigra fibrillary tangles were investigated ultrastructurally and found to consist of two kinds of fibrillary material: straight fine filaments of 100 A and twisted tubules with an average diameter of 200 A. We did not see 150 A straight filaments like those found in some cases of progressive supranuclear palsy. The possible significancy of each kind of neuro-fibrillary tangles and of their simultaneous occurrence in the subcortex of this case is discussed.
描述了一名81岁男性出现广泛皮质下神经原纤维变化、细胞丢失和胶质增生的病例。受影响最严重的区域是双侧苍白球、路易丘脑底核、黑质和小脑齿状核。大脑皮层仅含有少数老年斑。尽管缺乏典型的核上性眼肌麻痹和上丘受累，我们仍将此病例解释为进行性核上性麻痹的一种类型。对丘脑底核和黑质中的神经原纤维缠结进行了超微结构研究，发现其由两种纤维物质组成：直径100埃的直细纤维和平均直径200埃的扭曲小管。我们未见到在某些进行性核上性麻痹病例中发现的150埃直纤维。讨论了每种神经原纤维缠结及其在该病例皮质下同时出现的可能意义。
