国内外203例中枢神经系统暗色丝孢霉病回顾分析

Central nervous system phaeohyphomycosis:literature review of 203 cases

目的本文就国内外203例中枢神经系统暗色丝孢霉病进行系统分析,旨在帮助早期诊断,降低死亡率。方法收集2017年8月之前国内外报道的203例中枢神经系统暗色丝孢霉病患者性别、年龄、地域、早期临床表现、伴随疾病、治疗方法及预后等资料,进行总结分析。结果本病在免疫力健全和缺陷人群中均可发生,多见于男性青壮年;主要致病菌为斑替枝孢瓶霉、麦氏喙枝孢、皮炎外瓶霉;早期缺乏特异的临床特征,主要表现为颅内高压症状。目前治疗以药物治疗联合手术切除为主;一般医务工作者常对中枢神经系统暗色丝孢霉病缺乏了解,且因缺乏特异性快速性的诊断手段,许多患者被误诊误治,死亡率高达80%。结论中枢神经系统暗色丝孢霉病是一种临床症状不典型、早期诊断率低、治疗方案无统一标准、病死率极高的疾病。

Objective To review 203 central nervous system phaeohyphomycosis（CPHM）cases at home and abroad to help early diagnosis and reduce mortality.Methods Sex composition,age trait,geographical features,early clinical manifestations,associated diseases,therapies and outcome in 203 CPHM cases before August 2017 in home and abroad were collected.Results CPHM occured more commonly in immunocompetent patients than in immunedebilitated patients,predoiminantly in young and adult males.Cladophilophora bantiana was the most common etiologic agent followed by Ramichloridium mackenziei and Exophiala dermatitidis.There were no early specific symptoms of CPHM,except increased intracranial pressure.The combination of antifungal therapy and surgical resection could improve survival.However,few knowledge of CPHM and lacking specific detecting method might led to misdiagnosis or delayed treatment.An overall mortality was 80%.Conclusions With untypical features,CPHM had high mortality due to delayed diagnosis and the lack of standardized therapy.