Adam J, Polivka M, Kaci R, Godfraind C, Gray F
Service Central d'Anatomie et de Cytologie Pathologiques, Hôpital Lariboisière-Université Paris 7, Paris France.
Clin Neuropathol. 2010 Jul-Aug;29(4):246-53. doi: 10.5414/npp29246.
Distinctive hyaline inclusion bodies in the cytoplasm of neocortical astrocytes were observed in surgical resection specimens of a frontal epileptic focus, in 2 patients aged 16 and 10 who had suffered intractable partial seizures since the age of 2 years. One case had minimal neurological impairment and no brain malformation on MRI and recovered completely following surgery. The second case had mental retardation and surgery reduced the frequency and generalization of seizures. In both cases, the astrocytic inclusions were strongly eosinophilic, hyaline and refractile. They were PAS negative. Electron microscopy in the first case, confirmed their granular osmiophilic structure. By immunohistochemistry, the inclusions were strongly positive for filamin in the first case, only some were weakly positive in the second case. They also variably expressed other proteins such as alpha-B-crystallin, GFAP, S-100 protein and cytoglobin. We compare our findings with previously reported cases and discuss the clinical significance of the inclusions and the pathophysiologic relevance of filamin A and other proteins accumulation in astrocytes.
在两名分别为16岁和10岁的患者的额叶癫痫病灶手术切除标本中，观察到新皮质星形胶质细胞胞质内有独特的透明包涵体。这两名患者自2岁起就患有难治性部分性癫痫。其中一例神经功能损害轻微，MRI检查未发现脑畸形，术后完全康复。第二例有智力障碍，手术降低了癫痫发作的频率和泛化程度。在这两例中，星形胶质细胞包涵体均呈强嗜酸性、透明且有折光性。它们对PAS呈阴性。第一例的电子显微镜检查证实了其颗粒状嗜锇结构。通过免疫组织化学方法，第一例中的包涵体对细丝蛋白呈强阳性，第二例中只有一些呈弱阳性。它们还不同程度地表达其他蛋白质，如α-B-晶状体蛋白、GFAP、S-100蛋白和细胞珠蛋白。我们将我们的发现与先前报道的病例进行比较，并讨论包涵体的临床意义以及细丝蛋白A和其他蛋白质在星形胶质细胞中积累的病理生理相关性。
