Ho K L, Piligian J T, Chason J L
Arch Pathol Lab Med. 1979 Jul;103(7):344-7.
Subacute necrotizing encephalomyelopathy (SNE) usually appears before the age of 2 years. Variability in its clinical presentation make antemortem diagnosis difficult. The morphologic diagnosis depends on the present of necrotizing lesions with a characteristic distribution within the CNS. We describe a patient with the uncommon adult form of SNE. The pathologic changes are those accepted as diagnostic of the more common infantile SNE, and permit a separation from Wernicke's encephalopathy. In the adult, the disease is thought to be due to a less severe thiamine-related metabolic defect than that of the infantile form. The infrequent clinical recognition in the adult form may be due in part to lack of medical awareness.
亚急性坏死性脑脊髓病(SNE)通常在2岁前出现。其临床表现的变异性使得生前诊断困难。形态学诊断取决于中枢神经系统内具有特征性分布的坏死性病变的存在。我们描述了一名患有罕见成人型SNE的患者。病理变化是那些被认为可诊断更常见婴儿型SNE的变化,并可与韦尼克脑病相鉴别。在成人中,该疾病被认为是由于与硫胺素相关的代谢缺陷比婴儿型轻。成人型临床认识不常见可能部分归因于医学意识的缺乏。