婴儿中枢神经系统白色念珠菌病5例并文献复习

目的总结婴儿中枢神经系统白色念珠菌病的临床特点和诊断治疗经验。方法收集首都医科大学附属北京儿童医院2009至2011年收治的婴儿中枢神经系统白色念珠菌病连续病例（5例）的临床资料,总结其临床表现、实验室检查、影像学特征、治疗和随访情况,并文献复习。结果男4例,女1例,年龄3～5月龄（平均4月龄）,均无明显免疫缺陷证据及基础疾病。发病至诊断时间为2d至2个月。1例以脑梗死（局限性抽搐、面瘫）起病,4例以反复发热起病,精神反应相对较好,感染中毒症状不严重,无明显颅内压增高症状;4例合并抽搐发作。5例患儿均经CSF培养发现白色念珠菌生长。1例头颅MRI检查未见异常;4例病程中头颅MRI检查先后出现颅内血管受累,包括脑梗死和静脉窦栓塞,其中1例后期出现梗死部位脑软化,1例CT示基底节梗死。CSF常规改变均为WBC总数正常或轻度升高,分类以多核为主,CSF生化示糖明显降低,蛋白显著升高。1例应用氟康唑治疗2.5个月痊愈,随访1年无复发;2例先后应用氟康唑、两性霉素B和氟胞嘧啶联合治疗2周至1个月,临床好转出院,出院后均随访1年,其中1例出院口服伏立康唑2个月痊愈,另1例间断口服氟康唑2个月,病情好转,遗留智力和语言发育障碍;2例放弃治疗并失访。结论婴儿中枢神经系统白色念珠菌病临床表现不典型,感染中毒症状常不严重,脑脊液改变与化脓性脑膜炎类似,糖降低明显,较易出现颅内血管病变,应早期进行病原学检查明确诊断和指导治疗。

Report of 5 infants with central nervous system candidiasis and literature review

Objective To summarize the clinical characteristics of infants with central nervous system（CNS） candidiasis.Methods Associated literatures and case histories of 5 cases were analyzed retrospectively,who were diagnosed as CNS candidiasis in Chilren＇s Hospital affiliated to Capital Medical University from 2009 to 2011.Results The age of 5 cases ranged from 3 to 5 months,averaged 4 months.Four patients were boys and 1 was a girl.None of them had immune defects and underlying diseases.The time interval between presenting initial clinical symptoms and getting a definite diagnosis varied from 2 days to 2 months.Four cases started with recurrent fever.Their mental status was relatively fine without severe toxic symptoms caused by infection.No any apparent symptoms revealing increased intracranial pressure.Four cases experienced convulsive seizure,1 started with hemiplegia and cerebral infarction.All cases except 1 had abnormal MRI manifestation;4 cases had cerebrovascular impairment with CNS infection progressing including cerebral infarction and venous sinus infarction,and 1 of these 4 cases developed encephalomalacia where cerebral infarction occurred.The change of cerebrospinal fluid（CSF） with CNS candidiasis was similar with those of purulent meningitis.All cases had increased number of white blood cells with polykaryocytes dominant,decreased glucose concentration and apparently increased protein concentration.One case was treated with fluconazole for 2.5 months and recovered fully without any recurrence in a year.Two cases were treated with fluconazole,amphotericin B and fluorouracil successively for 2 weeks to 1 month and got improved when discharged from hospital.Follow-up study was done for one year as well.One of them was fully healed by taking voriconazole for 2 months,the other one got improved by taking fluconazole for 2 months but with sequelae of disturbance in intelligence and language.The other 2 cases gave up therapy.Conclusions Infants with CNS candidiasis lacked of typical clinical manifestations.The characteristics of CSF were similar with that of purulent meningitis.The glucose concentration of CSF for patients with CNS candidiasis was decreased remarkably without obvious toxic symptoms.Involvement of cerebrovascular disease might happen in CNS candidiasis cases.Etiological investigation earlier was important for definite diagnosis and proper therapy.