Martin J J, Ceuterick C M, Leroy J G, Devos E A, Roelens J G
Neuropediatrics. 1984 Feb;15(1):43-8. doi: 10.1055/s-2008-1052339.
Neuropathological data from the autopsy of a 5 1/2 year-old female patient with the Wiedemann-Rautenstrauch or neonatal progeroid syndrome are reported. Extensive demyelination is found in the central nervous system with occasionally a tigroid pattern and with large amounts of neutral fats and intermediate debris of myelin breakdown in macrophages. These lesions are characteristic of pure sudanophilic leucodystrophy. Features differentiating the findings from those in Pelizaeus-Merzbacher disease and in other disorders associated with sudanophilic leucodystrophy with various special characteristics are presented and discussed.
报告了一名5岁半患有维德曼-劳滕施特劳赫或新生儿早老综合征女性患者尸检的神经病理学数据。在中枢神经系统中发现广泛的脱髓鞘，偶尔呈虎斑状，巨噬细胞中有大量中性脂肪和髓鞘崩解的中间碎片。这些病变是纯嗜苏丹性脑白质营养不良的特征。文中介绍并讨论了将这些发现与佩利措伊斯-梅茨巴赫病以及其他具有各种特殊特征的嗜苏丹性脑白质营养不良相关疾病的发现区分开来的特征。
