Yamanouchi H, Jay V, Rutka J T, Takashima S, Becker L E
Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
Pediatr Neurol. 1997 Jul;17(1):49-53. doi: 10.1016/s0887-8994(97)00036-2.
To characterize the giant cells in tuberous sclerosis, we examined immunoreactivity for nestin, vimentin, microtubule-associated protein 1B (MAP1B), MAP2, neurofilament, and glial fibrillary acidic protein (GFAP) in cortical tubers detected in brain specimens from 6 patients with tuberous sclerosis who had undergone surgical resection for treatment of intractable epilepsy. Giant cells with a neuronal appearance, "neuron-like giant cells," had a round centrally-placed nucleus with a single, prominent nucleolus, and Nissl substance was commonly present in cortex. These neuron-like giant cells demonstrated consistently strong immunoreactivity for neurofilament and MAP1B and occasional immunopositivity for nestin and vimentin and were rarely positive for GFAP. "Indeterminate giant cells," characterized by abundant cytoplasm, an absence of Nissl substance, and one or more eccentric nuclei, demonstrated consistent immunoreactivity for nestin, vimentin, and MAP1B and were rarely positive for neurofilament, but more than half displayed immunoreactivity for GFAP. These observations suggest that the indeterminate giant cells exhibit limited neuronal and inconsistent astroglial characteristics, implying aberrant cellular differentiation in tuberous sclerosis.
为了描述结节性硬化症中的巨细胞特征，我们检测了6例因顽固性癫痫接受手术切除治疗的结节性硬化症患者脑标本中皮质结节内巢蛋白、波形蛋白、微管相关蛋白1B（MAP1B）、MAP2、神经丝和胶质纤维酸性蛋白（GFAP）的免疫反应性。具有神经元外观的巨细胞，即“神经元样巨细胞”，有一个位于中央的圆形细胞核，有一个单一的、突出的核仁，皮质中通常存在尼氏体。这些神经元样巨细胞对神经丝和MAP1B始终表现出强烈的免疫反应性，对巢蛋白和波形蛋白偶尔呈免疫阳性，对GFAP很少呈阳性。“不确定巨细胞”的特征是细胞质丰富、无尼氏体且有一个或多个偏心核，对巢蛋白、波形蛋白和MAP1B表现出一致的免疫反应性，对神经丝很少呈阳性，但超过一半对GFAP呈免疫反应性。这些观察结果表明，不确定巨细胞表现出有限的神经元特征和不一致的星形胶质细胞特征，这意味着结节性硬化症中存在异常的细胞分化。
