Ono S, Inoue K, Mannen T, Kanda F, Jinnai K, Takahashi K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
J Neurol Sci. 1987 Nov;81(2-3):301-20. doi: 10.1016/0022-510x(87)90105-5.
Brain autopsy materials from 2 patients with myotonic dystrophy (MyD) were studied. The results obtained in these 2 cases were quite similar. Besides thalamic inclusion bodies and minor abnormalities in gyral architecture with a disordered cortical cellular arrangement, some new observations have been made. First, no more than one intracytoplasmic inclusion body per cell was present in the cerebral cortex, the thalamus, the caudate nucleus and the putamen; this inclusion body was oval or elongated with smooth, sharply defined contours and was usually located at the periphery of the cell. Second, irregular intracytoplasmic inclusion bodies, often multiple and not surrounded by a halo, were found at the periphery or within accumulations of neuromelanin granules in the pigmented cells of the substantia nigra. All the bodies described above stained highly eosinophilic with hematoxylin-eosin and the ultrastructure of the bodies in the thalamus and the substantia nigra was almost the same; these bodies were composed of stacks of alternating parallel, light and dark rectilinear profiles oriented perpendicularly to the longitudinal axis of the bodies. Third, Marinesco bodies were observed with a very high frequency in the pigmented cells of the substantia nigra.
对2例强直性肌营养不良（MyD）患者的脑尸检材料进行了研究。这2例患者的研究结果颇为相似。除了丘脑包涵体以及脑回结构的轻微异常伴皮质细胞排列紊乱外，还获得了一些新的观察结果。首先，在大脑皮质、丘脑、尾状核和壳核中，每个细胞内不超过一个胞质内包涵体；该包涵体呈椭圆形或细长形，轮廓光滑、界限清晰，通常位于细胞周边。其次，在黑质色素细胞的神经黑色素颗粒聚集处周边或内部发现了不规则的胞质内包涵体，这些包涵体通常多个存在且无晕环围绕。上述所有包涵体苏木精-伊红染色均呈强嗜酸性，丘脑和黑质中包涵体的超微结构几乎相同；这些包涵体由交替平行的明暗直线状结构堆叠而成，这些结构垂直于包涵体的纵轴排列。第三，在黑质色素细胞中高频观察到马里内斯科小体。
