Nakamura Y, Miura K, Yamada I, Ino H, Mizobata T
Department of Neurology Sakai Hospital, Kinki University School of Medicine.
Rinsho Shinkeigaku. 2000 Aug;40(8):827-31.
This report describes a 46-year-old Japanese woman with bilateral symmetric thalamic necrosis. The unusual radiologic findings are discussed in relation with acute necrotizing encephalopathy (ANE) of childhood, a rare disease proposed by Mizuguchi et al. ANE affects young children and the incidence is highest between 6 and 18 months of ages. There is only one report of an adult case. The acute stage pathology in ANE can be summarized as acute edema and necrosis involving both gray and white matter by local breakdown of the blood-brain barrier. The radiologic findings in our case were similar to those in ANE of childhood. Though the pathogenesis between our adult case and ANE of childhood might be different, severe hypoalbuminea in our case could cause the alteration of permeability of the thalamic vessels, which might accelerate breakdown of the blood-brain barrier in the thalamus.
本报告描述了一名患有双侧对称性丘脑坏死的46岁日本女性。结合儿童急性坏死性脑病（ANE）对这些不寻常的影像学表现进行了讨论，ANE是水谷等人提出的一种罕见疾病。ANE影响幼儿，发病率在6至18个月大时最高。仅有一例成人病例报告。ANE的急性期病理可概括为血脑屏障局部破坏导致灰质和白质均出现急性水肿和坏死。我们病例的影像学表现与儿童ANE相似。尽管我们的成人病例与儿童ANE之间的发病机制可能不同，但我们病例中的严重低白蛋白血症可能导致丘脑血管通透性改变，这可能加速丘脑血脑屏障的破坏。
