摘要
目的探讨急性坏死性脑病(ANE)临床及影像学特点,以便提高对该疾病的认识,早期进行诊断及治疗。方法回顾性分析9例ANE患儿的临床资料。结果本研究共纳入9例ANE患儿,3~5岁年龄段患儿较多,9例患儿均无急性脑病的家族史及类似病史,发病月份多集中发生在3~5月。9例(100.0%)患儿均有发热现象,持续时间1~10 d,中位持续时间3 d,热峰(39±1)℃。8例(88.9%)伴有反复惊厥,且惊厥为脑病的首发表现。9例(100.0%)均在病程早期出现昏迷,多数在发病第3天,最快在发病24 h出现昏迷。脑脊液检查发现8例(88.9%)出现蛋白升高(微量蛋白>0.45 g/L),2例(22.2%)出现脑脊液压力>200 mm H2O(1 mm H2O=0.0098 k Pa)。9例(100.0%)脑电图均呈现慢波背景。6例死亡患儿中4例出现基底节豆状核受累,而3例存活患儿中1例出现豆状核受累。另外死亡患儿头颅磁共振成像(MRI)受累区域数目较存活患儿多。病变性质9例(100.0%)均可见水肿病变,包括血管性、间质及细胞内水肿,还可见2例(22.2%)出血性病灶。在病原学检查中,所有患儿均进行了病原咽拭子脱氧核糖核酸(DNA)检测(包括流感A+B型、肠道病毒、单纯疱疹、肺炎支原体、衣原体、腺病毒、合胞病毒)。入院行血生化检查,6例(66.7%)出现肝酶升高,有4例(44.4%)低白蛋白(<40 g/L),有1例(11.1%)乳酸升高。9例(100.0%)血浆氨基酸、血酰基肉碱、尿气相色谱-质谱分析(GC/MS)检查未见异常。血炎症指标检查,8例(88.9%)血降钙素原明显升高。5例(55.6%)完善外周血淋巴细胞计数检查,其中4例(80.0%)出现自然杀伤性T细胞(NKT细胞)计数下降,而其他类别淋巴细胞计数正常。治疗上,所有患儿均予以甘露醇及3%氯化钠交替降颅压,流感病毒B感染患儿予磷酸奥司他韦抗病毒,单纯疱疹病毒感染患儿予阿昔洛韦,支原体感染患儿予阿奇霉素,但人免疫球蛋白和糖皮质激素治疗上差别很大,8例(88.9%)患儿予人�
Objective To investigate the clinical manifestations and neurological features of acute necrotizing encephalopathy（ANE） in order to raise awareness of the disease,early diagnosis and treatment.Methods The clinical data of 9 children with ANE were retrospectively analyzed.Results A total of 9 children with ANE were included in this study.There were more children in 3-5 years of age.There were no family history and similar history of acute encephalopathy in 9 cases.The months of onset were mostly in 3-5 months.Fever occurred in 9 cases（100.0%）,with a duration of 1-10 d,median duration was 3 d,and thermal peak was（39±1）℃.8 cases（88.9%） had recurrent seizures,and convulsion was the first manifestation of encephalopathy.9 cases（100.0%） had coma at the early stage of the disease,most of them were on the third day of onset,and the coma occurred 24 h at the earliest onset.Cerebrospinal fluid examination found that 8 cases（88.9%） had increased protein（microprotein 0.45 g/L）,2 cases（22.2%） had cerebrospinal fluid pressure 200 mm H2 O（1 mm H2 O=0.0098 k Pa）.The electroencephalogram of 9 cases（100.0%） showed slow wave background.In 6 cases of death,4 cases had basal ganglia lenticular nucleus involvement,while 1 of 3 cases survived with lenticular nucleus involvement.In addition,the number of Cranial magnetic resonance imaging（MRI） affected children was more than that of the surviving children.Lesions in 9 cases（100.0%） showed edema lesions,including vascular,interstitial and intracellular edema,and 2 cases（22.2%） hemorrhagic lesions were observed.In the aetiology examination,all the children were detected by the pathogenic swab deoxyribonucleic acid（DNA） detection（including influenza A＋B,enterovirus,herpes simplex,mycoplasma pneumoniae,chlamydia,adenovirus,and syncytial virus）.Blood biochemical examination showed that 6 cases（66.7%） had elevated liver enzymes,4 cases（44.4%） had low albumin（40 g/L）,and 1 case（11.1%） had elevated lactic acid.9 ca
作者
甘颖妍
麦坚凝
曾意茹
吴汶霖
GAN Ying-yan;MAI Jian-ning;ZENG Yi-ru(Department of Neurological Rsehabilitation,Guangzhou Women and Children＇s Medical Centre,Guangzhou 510000,China)
出处
《中国实用医药》
2018年第20期19-22,共4页
China Practical Medicine
