儿童急性坏死性脑病的临床与MRI表现-现代医学

儿童急性坏死性脑病的临床与MRI表现

单位：哈尔滨市儿童医院影像科, 黑龙江哈尔滨 150010

分类号：R445.2;R718

出版年·卷·期（页码）：2022·50·第八期（1028-1032）

摘要：

目的：分析儿童急性坏死性脑病(ANEC)的临床和磁共振成像(MRI)特征，早期诊断指导临床。方法：收集10例ANEC患儿的临床及影像资料，起病后1~3 d内进行MRI检查，观察病变部位，随访MRI观察各序列的信号变化特点。结果：6例患儿脑脊液蛋白异常，7例患儿血清转氨酶异常。MR检查显示，早期双侧丘脑对称性肿胀，并出现典型“同心圆”征象，其他病变部位有脑室周围白质异常7例、脑干异常7例、基底节区异常7例、胼胝体异常1例、小脑异常5例及颞叶异常3例。随访显示，死亡1例，7例持续进行康复治疗中，2例预后良好。结论： ANEC进展迅速，脑脊液蛋白及血清转氨酶水平不同高低程度的变化可供临床诊断参考，特征性影像学表现有助于指导临床早期诊断，随访连续监测MRI结果有利于判断预后。

Objective: To investigate the clinical and MRI findings of acute necrotizing encephalopathy in children to guide the diagnosis.Methods: Ten children with ANE were included in this study,MR was performed after onset 1-3 days,the location of the lesions and characteristic neuroimaging findings of abnormal signal intensity were observed.Results: Six out of 10 cases showed increased cerebrospinal fluid protein,7 out of 10 cases showed abnormal serum aminotransferase.In the early stage,MRI revealed "concentric circles" in bilateral thalamus in all children,but there also were lesions in other areas including periventricular white matter (7 cases),brainstem (7 cases),basal ganglia (7 cases),corpus callosum (1 case),small brain (5 cases),temporal lobe (3 cases).Follow-up at different time indicated that one child died,7 children were given rehabilitation therapy and two children had a good prognosis.Conclusions:ANEC is a rapidly progressing encephalopathy,increased cerebrospinal fluid proteina and a variable degree of serum aminotransferase can be used as a reference for clinical diagnosis.The characteristic of MR imagings are helpful to guide early clinical diagnosis,follow-up MRI can predict the prognosis.

参考文献：

[1] MIZUGUCHI M,ABE J,MIKKAICHI K,et al.Acute necrotising encephalopathy of childhood:a new syndrome presenting with multifocal,symmetric brain lesions[J].J Neurol Neurosurg Psychiatry,1995,58(5):555-561.
[2] NEILSON D E.The interplay of infection and genetics in acute necrotizing encephalopathy[J].Curr Opin Pediatr,2010,22(6):751-757.
[3] 周景,洪丽英,赵洋洋,等.南京市儿童医院2013-2017年流感流行病学特征分析[J].东南大学学报(医学版),2018,37(6):1005-1009.
[4] VIRHAMMAR J,KUMLIEN E,FALLMAR D,et al.Acute necrotizing encephalopathy with SARS-CoV-2 RNA confirmed in cerebrospinal fluid[J].Neurology,2020,95(10):445-449.
[5] LEE Y J,HWANG S K,LEE S M,et al.Familial acute necrotizing encephalopathy with RANBP2 mutation:the first report in Northeast Asia[J].Brain Dev,2017,39(7):625-628.
[6] NISHIMURA N,HIGUCHI Y,KIMURA N,et al.Familial acute necrotizing encephalopathy without RANBP2 mutation:poor outcomet[J].Pediatr Int,2016,58(11):1215-1218.
[7] NEILSON D E,ADAMS M D,ORR C M,et al.Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore,RANBP2[J].Am J Hum Genet,2009,84(1):44-51.
[8] CHOW C K,MA C K L.Presentation and outcome of acute necrotizing encephalopathy of childhood:a 10-year single-center retrospective study from Hong Kong[J].Child Neurol,2020,35(10):674-680.
[9] WOODROOFEM N.Cytokine production in the central nervous system[J].Neurology,1995,45(6):6-10.
[10] HOLLA V V,GOHEL A B,KARTIK N,et al.Acute necrotizing encephalopathy as a complication of chikungunya infection[J].Neurol India,2021,69(2):490-492.
[11] 侯健宁,刘鸿圣,张娜,等.儿童急性坏死性脑病在不同时间窗的MRI表现[J].影像诊断与介入放射学,2021,30(2):137-142.
[12] LEE Y J,HWANG S K,KWON S.Acute necrotizing encephalopathy in children:a long way to go[J].J Korean Med Sci,2019,34(19):143.
[13] YOSHIDA T,TAMURA T,NAGAI Y,et al.MRI gadolinium enhancement precedes neuroradiological findings in acute necrotizing encephalopathy[J].Brain Dev,2013,35(10):921-924.
[14] AKSOY E,OZTOPRAK U,ÇELIK H,et al.Acute necrotizing encephalopathy of childhood:a single-center experience[J].Turk J Med Sci,2021,51(2):706-715.
[15] STEVANOVICV,BARUSICZ,VISKOVIC K,et al.Acute necrotizing encephalopathy of childhood associated with human herpes virus 6 in Croatia[J].Neurol Sci,2019,40(3):639-641.
[16] SHIRAI T,FUJII H,ONO M,et al.A novel autoantibody against ephrin type B receptor 2 in acute necrotizing encephalopathy[J].J Neuroinflammation,2013,10(1):128.

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