INFANTILE CEREBRAL GLIOSIS WITH GIANT NERVE CELLS
INFANTILE CEREBRAL GLIOSIS WITH GIANT NERVE CELLS
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DOI：
10.1136/jnnp.20.2.117
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发表时间：
1957-01-01
影响因子：
11
通讯作者：
CROME, L
中科院分区：
文献类型：
--
作者：
CROME, L
先天性脑胶质增生症的常见形式是以脑回的硬化和萎缩以及相应的脑沟扩张为特征。每个脑回的收缩从一侧到另一侧比从上到下更大，因此在严重的病例中，脑膜剥离后，受影响的区域通常呈核桃状。组织学上，有神经元损失和广泛的纤维和细胞胶质增生。这通常在边缘层最为明显，但在较深的纹层之一，特别是第三纹层也可能很明显。“Ulegyria”，一个表示脑回疤痕的术语，通常适用于这种情况。其临床特征取决于病变的范围和部位，通常见于不同程度的智力低下、小头畸形、运动障碍和癫痫患者。在大多数情况下，没有明确的临床或形态学证据表明有进展的趋势。本文的目的是介绍三个病人，说明一个有点不同类型的尿失禁。其中两人前来尸检，并进行病理检查。第三个还活着。在进行尸检的两例病例中，受累区域的消瘦是一致的。虽然脑回的大小有所缩小，但仍倾向于保持其通常的轮廓和连续性，而脑沟也不会过于宽。脑部检查异常不明显。触诊更有用，因为受影响的区域非常坚硬。组织学上有明显的神经细胞和神经胶质增生的损失，最显着的表层皮质层。1例弥漫性纤维性胶质增生。第二种是分层的，在边缘层和中间皮层尤为明显。一个显著的形态学特征是存在大的，不规则形状的神经细胞与粗壮和曲折的过程。许多增生的胶质细胞也是非典型的。就像许多其他严重白痴的病例一样，很难确定是否有任何进行性脑损伤的临床证据。所有3例患者的头围均有所缩小。他们
The usual form of congenital cerebral gliosis is characterized by induration and shrinkage of gyri and corresponding dilatation of the sulci. The shrinkage in each gyrus appears to be greater from side to side than from above downwards so that in severe cases the affected area is often walnut-like after the meninges are stripped. Histologically, there is neuronal loss and widespread fibrous and cellular gliosis. This is usually most marked in the marginal layer but may also be conspicuous in one of the deeper laminae, particularly the third." Ulegyria", a term signifying scarring of gyri, is generally applied to this condition. Its clinical features depend on the extent andlocalization of the lesion, and it is usually found in patients with varying degrees of mental retardation, microcephaly, motor disability, and epilepsy. In most of these there is no definite clinical or morphological evidence of a tendency to progress.The object of this communication is to present three patients illustrating a somewhat different type of ulegyria. Of these two had come to necropsy and were examined pathologically. The third is still alive. In the two cases coming to necropsy wasting of the affected area was uniform. Although somewhat reduced in size, gyri tended to retain their usual outlines and contiguity, while the sulci were not unduly wide. The abnormality was not very obvious on inspection of the brain. Palpation was more useful, the affected area being very hard. Histologically there was marked loss of nerve cells and glial hyperplasia, most marked in the superficial cortical layers. Fibrous gliosis was diffuse in one case. Inthe second it was stratified and particularly conspicuous in the marginal and middle cortical layers. A striking morphological feature was the presence of large, irregularly shaped nerve cells with stout and tortuous processes. Many of the hyperplastic glial cells were also atypical. As in so manyother cases of gross idiocy it was difficult to determine whether there was any clinical evidence of a progressive brain lesion. The head circumference was somewhat reduced in all three cases. They