中枢神经系统曲霉菌病  

Central nervous system aspergillosis

作　　者：倪品菲 范思远[1] 李佳 有慧[2] 赵大春[3] 任海涛[1] 关鸿志[1] NI Pin-fei;FAN Si-yuan;LI Jia;YOU Hui;ZHAO Da-chun;REN Hai-tao;GUAN Hong-zhi(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China;Department of Radiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China;Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院神经科,100730 [2]中国医学科学院,北京协和医学院,北京协和医院放射科,100730 [3]中国医学科学院,北京协和医学院,北京协和医院病理科,100730

出　　处：《中国现代神经疾病杂志》2024年第4期258-264,共7页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：北京协和医院中央高水平医院临床科研专项(项目编号:2022-PUMCH-B-120)。

摘　　要：研究背景中枢神经系统曲霉菌感染临床罕见,本文报道1例经病理确诊的中枢神经系统曲霉菌病患者,分析其临床特点并复习文献,总结诊断与治疗要点。方法与结果女性患者,50岁,临床主要表现为进行性四肢无力、癫痫发作、认知功能下降、性格改变,系统性炎症及免疫学指标未见明显异常,脑脊液常规、生化、细胞学、病原学检测未见明显异常;头部MRI显示双侧额顶叶异常信号伴片状、环形强化,脑膜强化;脑组织活检提示血管炎及脑组织坏死伴出血,六胺银染色可见有分隔的真菌菌丝,分叉呈45°;最终确诊为中枢神经系统曲霉菌病,经抗真菌治疗后症状好转,复查MRI病灶较前缩小。结论中枢神经系统曲霉菌病可发生于免疫功能正常人群,临床表现缺乏特异性,脑脊液可无炎症改变,宏基因组学第二代测序可呈阴性,脑组织活检术是诊断的“金标准”,早期、足量、足疗程予以伏立康唑抗真菌治疗可以改善预后。Background Central nervous system aspergillosis is clinically rare.We present one case of central nervous system aspergillosis diagnosed by pathology,analyze its clinical features,review the literature,and summarize key diagnostic and therapeutic points.Methods and Results The patient,a 50-year-old woman,presented clinically with progressive weakness in limbs,epileptic seizures,and cognitive decline.Systemic inflammation-immune markers and cerebrospinal fluid(CSF)analysis showed no significant abnormalities.Head MRI revealed abnormal signals in both frontal and parietal lobes with patchy and ring-enhancing lesions and meningeal enhancement.Neuropathology suggested vasculitis and brain tissue necrosis with hemorrhage.Periodic acid methenamine staining revealed fungal hyphae with apparent septation and branching at 45°angles.The final diagnosis was central nervous system aspergillosis.Following antifungal and other symptomatic treatments,the patient's symptoms improved,and follow-up brain MRI showed reduction in lesion size.Conclusions Patients with central nervous system aspergllosis may not have a clear underlying immunodeficiency,and clinical manifestations are lack of specificity.CSF may show no inflammatory changes,and metagenomic next-generation sequencing(mNGS)may be negative.Brain biopsy is the primary diagnostic method.Early,adequate and full-course antifungal treatment with voriconazole can improve the prognosis.

关 键 词：曲霉菌病 中枢神经系统真菌感染 伏立康唑 活组织检查 病理学 

分 类 号：R519[医药卫生—内科学]