Fukutani Y, Yasuda M, Saitoh C, Kyoya S, Kobayashi K, Miyazu K, Nakamura I
Division of Neuropsychiatry, National Sanatorium, Hokuriku Hospital, Toyama, Japan.
Histol Histopathol. 1992 Oct;7(4):709-14.
We report an autopsy case of tuberous sclerosis. A 19-year-old Japanese man had shown facial adenoma sebaceum, intractable convulsive seizures and severe mental retardation. Gross inspection of the brain showed a cortical tuber from the orbital frontal lobe to the rhinencephalon of the left side and a few subependymal nodules. Histological examination revealed many cortical tubers in the cerebral hemispheres, a few subependymal nodules with calcification and multifocal clusters of heterotopic cells in the white matter (white matter nodules). In these lesions, massive giant cells with abundant eosinophilic cytoplasm and without Nissl substances were found. Although the size and shape of the giant cells were variable, the majority of them were gemistcytic, ovoid or polygonal. Immunohistochemistry was employed in these lesions using antibodies against neurofilament protein (NFP), glial fibrillary acidic protein (GFAP), vimentin (VM) and myelin basic protein (MBP). In the cortical tuber, the majority of the giant cells were positive for both NFP and VM, but a few were positive for GFAP. All of them were negative for MBP. In the subependymal nodule and white matter nodule, the majority of the giant cells were positive for NFP, but a few were positive for VM, and none were positive for either GFAP and MBP. These findings suggest that the majority of the giant cells may be immature cells toward neuronal series and a few may be those toward astroglial series. These findings also indicate that the giant cells in the subependymal nodule and white matter nodule may be more differentiated than those in the cortical tuber. The nature of the giant cells in tuberous sclerosis is discussed.
我们报告一例结节性硬化症的尸检病例。一名19岁的日本男性有面部皮脂腺瘤、顽固性惊厥发作和严重智力迟钝。大脑大体检查显示左侧眶额叶至嗅脑有一个皮质结节以及一些室管膜下结节。组织学检查发现大脑半球有许多皮质结节、一些伴有钙化的室管膜下结节以及白质中多灶性异位细胞簇（白质结节）。在这些病变中，发现了大量具有丰富嗜酸性细胞质且无尼氏体的巨细胞。尽管巨细胞的大小和形状各异，但大多数为肥胖型、卵圆形或多边形。在这些病变中采用免疫组织化学方法，使用抗神经丝蛋白（NFP）、胶质纤维酸性蛋白（GFAP）、波形蛋白（VM）和髓磷脂碱性蛋白（MBP）的抗体。在皮质结节中，大多数巨细胞NFP和VM均呈阳性，但少数GFAP呈阳性。所有巨细胞MBP均为阴性。在室管膜下结节和白质结节中，大多数巨细胞NFP呈阳性，但少数VM呈阳性，GFAP和MBP均无阳性。这些发现表明，大多数巨细胞可能是向神经元系列分化的未成熟细胞，少数可能是向星形胶质细胞系列分化的细胞。这些发现还表明，室管膜下结节和白质结节中的巨细胞可能比皮质结节中的巨细胞分化程度更高。本文讨论了结节性硬化症中巨细胞的性质。
