Zinka Bettina, Buettner Andreas, Graw Matthias
Institute of Legal Medicine, University of Munich, Nussbaumstrasse 26, 80336 Munich, Germany.
Case Rep Med. 2010;2010:986302. doi: 10.1155/2010/986302. Epub 2010 Jun 10.
We report the untypical clinical course of a previously healthy two-year-old girl, who died suddenly and unexpectedly after an episode of vomiting. At forensic autopsy no other pathological findings could be diagnosed than multiple reddish, sunken areas in brain stem, mesencephalon, and pons. Histologically they presented as areas of spongiosis of the neuropil with prominent endothelial hyperplasia and vascular proliferation whereas nerve cells were well preserved. On the basis of the characteristic neuropathological findings in combination with the age of the child, we had to take into consideration that the child might have died from subacute necrotizing encephalomyelopathy (Leigh's Disease) despite the untypical, fulminant clinical course.
我们报告了一名此前健康的两岁女童的非典型临床病程，她在一次呕吐发作后突然意外死亡。法医尸检时，除了脑干、中脑和脑桥出现多个微红、凹陷区域外，未发现其他病理结果。组织学上，这些区域表现为神经纤维海绵状变，伴有显著的内皮细胞增生和血管增殖，而神经细胞保存完好。基于这些特征性的神经病理学发现以及患儿的年龄，尽管临床病程不典型且呈暴发性，我们仍不得不考虑该患儿可能死于亚急性坏死性脑脊髓病（ Leigh病）。
