Xu Dingkang, Xu Hongen, Wang Fang, Wang Guoqing, Wei Qingjie, Lei Shixiong, Gao Qiang, Zhang Qi, Guo Fuyou
Department of Neurosurgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Center for Precision Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Front Neurol. 2019 Mar 11;10:213. doi: 10.3389/fneur.2019.00213. eCollection 2019.
Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare rheumatic immune disease characterized by vasculitis of small- and medium-sized blood vessels. Central nervous system (CNS) involvement frequently consists of cerebrovascular disease; a manifestation with multiple demyelinating lesions has never been reported in detail. This report describes a 38-year-old man, who presented with progressive memory deterioration and underwent microsurgery; EGPA was subsequently confirmed. Unique clinical and radiological features as well as immunohistological outcomes and DNA sequencing revealed a potential disease-associated human leukocyte antigen (HLA) type, and single-nucleotide polymorphisms (SNPs) are described for this uncommon case. Although EGPA rarely involves the CNS, this differential diagnosis should be considered when patients present with a history of nasosinusitis, elevated eosinophil percentage, clinical pulmonitis, and neurological manifestations. Microsurgery is necessary for precise diagnosis and effective treatment.
嗜酸性肉芽肿性多血管炎（EGPA）是一种极其罕见的风湿性免疫疾病，其特征为中小血管血管炎。中枢神经系统（CNS）受累常表现为脑血管疾病；从未有过关于多个脱髓鞘病变表现的详细报道。本报告描述了一名38岁男性，他出现进行性记忆减退并接受了显微手术；随后确诊为EGPA。独特的临床和放射学特征以及免疫组织学结果和DNA测序揭示了一种潜在的疾病相关人类白细胞抗原（HLA）类型，并描述了该罕见病例的单核苷酸多态性（SNP）。尽管EGPA很少累及中枢神经系统，但当患者有鼻窦炎病史、嗜酸性粒细胞百分比升高、临床肺炎和神经表现时，应考虑这种鉴别诊断。显微手术对于精确诊断和有效治疗是必要的。
