结节性硬化症

下,常伴钙质沉积,可出现异位症及血管增生等?大脑皮质有很多硬实结节,白质内有异位细胞团,脑室壁内亦有小结节?皮质结节数1~40个不等,以额叶最多,但也可发生于丘脑、基底节、小脑脑干和脊髓,?结节大小不一,有的直径可>3cm?脑部病损一般无恶性变的可能Р病理Р侧脑室扩张,室管膜壁附着大量异常的神经胶质结节,呈闪亮白色,质地坚硬,形成所谓的“烛泪”征,有时可阻塞脑脊液循环通路而引起脑积水Р病理Р脑皮质标本,表面见异常神经胶质结节,而表现为巨脑回畸形Р病理Р结节由非常致密的细胶元纤维组成,内含形态异常的胶质细胞及正常或不典型的神经元?结节内可有钙盐沉积或囊性变。正常的皮质结构常发生紊乱?白质内的异位细胞核团也是由于胶质细胞和变形的神经节细胞所组成火罐网,神经纤维较少,主要分布在脑室壁和大脑皮质间Р病理Р神经细胞肿胀,奇异形,似巨细胞Р病理РNecrosis, hemorrhage, and deeply stained giant cells. The astrocytes were postivie for GFAP. (HE, original magnification×400).Р影像学РFig. 1. Pre-contrast CT scan showing multiple calcifications at the bilateral basal ganglia. ?Fig. 2. Axial plain CT scan showing multiple calcifications on the lateral wall of the bilateral ventricles. The posterior lateral wall? of the left ventricle was ? compressed by a? hypointensity lesion.