Lindboe C F, Lie A K, Aase S T, Schjetne O B, Haave I
Department of Pathology, Trondheim University Hospital, Norway.
APMIS. 1995 Jan;103(1):54-8. doi: 10.1111/j.1699-0463.1995.tb01079.x.
We report clinical, radiological and pathological findings in a 5-year-old girl who died of subacute necrotizing encephalomyelopathy (SNE) after 4 weeks of illness. Autopsy revealed endothelial swelling and vacuolar degeneration of the neuropil in the brain, brain stem and cerebellum. In addition, the affected areas showed degeneration of the neurons which was different from anoxic nerve cell damage both with regard to morphological picture and topographical distribution. This neuronal degeneration was probably due to the underlying metabolic defect in SNE per se and resembled in several aspects the nerve cell changes seen in the thalami and inferior olives in active Wernicke's encephalopathy. It is our opinion that more attention should be paid to the nerve cell degeneration in SNE rather than focusing on the relative preservation of these cells.
我们报告了一名5岁女孩的临床、放射学和病理学检查结果，该女孩在患病4周后死于亚急性坏死性脑脊髓病（SNE）。尸检显示大脑、脑干和小脑的神经纤维网存在内皮肿胀和空泡变性。此外，受累区域的神经元发生变性，在形态学表现和地形分布方面均与缺氧性神经细胞损伤不同。这种神经元变性可能是由于SNE本身潜在的代谢缺陷所致，在几个方面类似于活跃型韦尼克脑病中丘脑和下橄榄核所见的神经细胞变化。我们认为，对于SNE，应更多关注神经细胞变性，而不是聚焦于这些细胞的相对保存状态。
