Yasukohchi S, Akabane T, Mori T, Tamagawa K, Morimatsu Y
Brain Dev. 1986;8(6):609-14. doi: 10.1016/s0387-7604(86)80008-0.
We report here an autopsy case, an 8-year-old boy diagnosed as having infantile striatal necrosis, characterized by a preceding febrile illness followed by acute encephalopathy with abrupt obtundation, seizures and dystonia, with remarkable improvement of the disturbed consciousness and intelligence after TRH-T therapy. These clinical symptoms were linked with bilateral necrosis of the striata on CT scanning. The presented case belonged to a newly described subgroup of the heredogenous disorders that produce necrosis of the putamina in children.
我们在此报告一例尸检病例，一名8岁男孩被诊断为患有婴儿纹状体坏死，其特征为先前有发热性疾病，随后出现急性脑病，伴有突然的意识迟钝、癫痫发作和肌张力障碍，经促甲状腺素释放激素（TRH - T）治疗后意识和智力障碍有显著改善。这些临床症状与CT扫描显示的双侧纹状体坏死有关。该病例属于一种新描述的遗传性疾病亚组，这类疾病会导致儿童壳核坏死。
