Kunming General Hospital of Chengdu Military Command, 212 Daguan Road, Kunming 650032, China.
J Clin Neurosci. 2012 Dec;19(12):1746-8. doi: 10.1016/j.jocn.2011.12.036. Epub 2012 Sep 19.
Idiopathic hypereosinophilic syndrome (HES) is characterized by persistent hypereosinophilia ( ≥ 1500/mm(3)) with evidence of end-organ damage without a definite underlying cause. Hypereosinophilia-induced encephalopathy is a rare clinical syndrome. We present a male patient with idiopathic HES with distinctive encephalopathy who had hypereosinophilia for more than 6 months. Eosinophils in repeated blood tests were more than 1500/mm(3). He had hematological, brain, bone-marrow, and possible cardiac involvement. Although numerous efforts were made to identify the underlying cause of hypereosinophilia, specific causes could not be found in this patient. Bone-marrow analysis confirmed the diagnosis. The unique features were the prominent involvement of the cerebral cortex and the dramatic response to steroids with marked improvement of eosinophilia and brain function. The mechanisms of hypereosinophilia-induced encephalopathy are discussed.
特发性嗜酸性粒细胞增多综合征（HES）的特征为持续性嗜酸性粒细胞增多症（≥1500/mm³），伴有明确的终末器官损伤，但无明确的潜在病因。嗜酸性粒细胞增多症相关性脑病是一种罕见的临床综合征。我们报告了一例特发性 HES 伴有特征性脑病的男性患者，其嗜酸性粒细胞增多超过 6 个月，多次血常规检查嗜酸性粒细胞均超过 1500/mm³。他存在血液系统、脑部、骨髓和可能的心脏受累。尽管进行了大量努力以确定嗜酸性粒细胞增多的潜在病因，但该患者仍未能找到明确病因。骨髓分析明确了诊断。其独特特征为大脑皮质显著受累，以及对类固醇的显著反应，导致嗜酸性粒细胞增多和脑功能明显改善。讨论了嗜酸性粒细胞增多症相关性脑病的发病机制。
