Arai N, Honda Y, Amano N, Yagishita S, Misugi K
Department of Pathology, Yokohama City University School of Medicine, Japan.
J Neurol. 1988 Jul;235(6):330-4. doi: 10.1007/BF00314227.
An unusual case of recurrent attacks of peculiar twilight state persisting for 41 years is the subject of this clinicopathological report. During the attacks the patient had depersonalization, showing a stiff face, and the electroencephalogram showed constant 5 Hz diffuse theta waves. The unique and characteristic neuropathological finding were many foamy spheroid bodies (FSB) in the substantia nigra which sometimes contained varying numbers of fine or coarse eosinophilic granules. Ultrastructurally, the FSB contained various small electron-dense granules and/or membranous structures quite different from so-called spheroids (axonal swellings). Bodian staining demonstrated that some FSB were situated within the bundles of the neuronal processes, suggesting that the FSB has originated from the degeneration of the axon and/or dendrites in the substantia nigra.
本临床病理报告的主题是一例罕见的复发性特殊黄昏状态发作病例,该状态持续了41年。发作期间,患者出现人格解体,面部僵硬,脑电图显示持续的5Hz弥漫性θ波。独特且具有特征性的神经病理学发现是黑质中有许多泡沫样球体(FSB),其有时含有数量不等的细或粗嗜酸性颗粒。超微结构上,FSB含有各种小的电子致密颗粒和/或膜性结构,与所谓的球体(轴突肿胀)截然不同。博迪安染色显示,一些FSB位于神经突起束内,提示FSB起源于黑质中轴突和/或树突的变性。