成人坏死性出血性脑脊髓病： Leigh 病

Delgado G, Gállego J, Tuñón T, Zarranz J J, Villanueva J A

J Neurol Neurosurg Psychiatry. 1987 Feb;50(2):224-7. doi: 10.1136/jnnp.50.2.224.

A 21 year old male, well-nourished and non-alcoholic, died after five weeks illness. He had suffered epileptic fits, bilateral internuclear ophthalmoplegia, bulbar and pontine paralysis, tetraparesia, ataxia and dystonia. A CT brain scan showed low density lesions of the striatum bilaterally. Post-mortem studies revealed pathological anomalies compatible with Leigh's disease, although the presence of haemorrhages and involvement of the mamillary bodies could also suggest Wernicke's encephalopathy.

一名21岁男性，营养良好且无酗酒史，患病五周后死亡。他曾出现癫痫发作、双侧核间性眼肌麻痹、延髓和脑桥麻痹、四肢轻瘫、共济失调和肌张力障碍。脑部CT扫描显示双侧纹状体低密度病变。尸检研究发现病理异常符合 Leigh 病，尽管存在出血和乳头体受累也可能提示韦尼克脑病。