Lageman A, Dietz W, Ullrich K P
Zentralbl Allg Pathol. 1977;121(1-2):22-32.
Sural nerve biopsies taken from a pair of twins with metachromatic leukodystrophy were investigated by electron microscopy. The morphological findings showed distinct alterations. In the first twin the particularity was a large number of granules containing sulfatides. In the second twin, however, the main finding was a damage of myelin sheaths evidently caused by an excessive and morphologically as well as chemically defective myelin formation. According to the grading given by Ulrich the first case corresponds to the stadium one, in which a storage of sulfatides dominates, whereas case two showing a severe damage of myelin sheaths is characteristic of the second stadium of metachromatic leukodystrophy.
对一对患有异染性脑白质营养不良的双胞胎进行了腓肠神经活检,并通过电子显微镜进行研究。形态学结果显示出明显的改变。在第一个双胞胎中,其特点是存在大量含有硫脂的颗粒。然而,在第二个双胞胎中,主要发现是髓鞘的损伤,显然是由于髓鞘形成过多且在形态和化学上存在缺陷所致。根据乌尔里希给出的分级,第一个病例对应于第一阶段,其中硫脂的储存占主导,而第二个病例显示髓鞘严重受损是异染性脑白质营养不良第二阶段的特征。