抗N-甲基-D-天冬氨酸受体脑炎患者影像学特征分析
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摘要
目的分析抗N-甲基-D-天冬氨酸(NMDA)受体脑炎患者的临床、影像特点。方法收集2015年1月—2017年2月于首都医科大学宣武医院诊治的53例抗NMDA受体脑炎患者的临床资料,分析并总结抗NMDA受体脑炎的临床特征和影像学特征。结果 53例抗NMDA受体脑炎患者中女24例(45.3%),男29例(54.7%);年龄6～72岁(其中<18岁16例,>45岁8例),中位年龄23岁;病程7 d～2年。颅脑磁共振成像(MRI)表现异常者占69.8%(37/53),其中12例可见T2加权像(T2WI)、液体衰减反转恢复序列(FLAIR)异常信号,累及大脑皮质及皮质下白质、海马、岛叶、小脑、脑桥、胼胝体、基底核、扣带回、丘脑。10例患者行扩散加权成像(DWI),8例可见轻度扩散受限;11例行增强MRI检查,2例可见轻度强化;33例MRI检查示双侧或单侧颞角扩大,提示海马萎缩,其中6例伴全脑萎缩。7例患者行氟代脱氧葡萄糖(~(18)F-FDG)PET-CT检查,3例显示葡萄糖代谢减低,MRI检查表现为脑萎缩、海马萎缩。1例患者行氢质子磁共振波谱(~1H-MRS)检查,与对侧正常脑组织相比,异常信号区N-乙酰天冬氨酸(NAA)/肌酸(Cr)降低,胆碱(Cho)/Cr升高。结论抗NMDA受体脑炎患者MRI检查显示包括边缘系统在内的多个脑区异常信号;首次就诊时MRI检查显示颞角扩大,提示抗NMDA受体脑炎发病前海马已受累。
Objective To investigate the clinical and imaging features of anti-N-methyl-D-aspartate receptor(anti-NMDAR) encephalitis.Methods A total of 53 patients with anti-NMDAR encephalitis admitted to Xuanwu Hospital Capital Medical University from January to February 2015 were enrolled in this study.Their clinical and imaging features were analyzed retrospectively.Results Among the 53 patients with anti-NMDAR encephalitis,24 were female(45.3%) and 29 were male(54.7%).Their age ranged from 6 to 72 years with a median age of 23 years(16 cases aged <18 years and 8 cases aged >45 years).The clinical course was 7 days to 2 years.Patients with abnormal MRI manifestation accounted for 69.8%(37/53),and abnormal signal in cerebral cortex and subcortical white matter, hippocampus, insula, cerebellum, pons, corpus callosum, basal nucleus, cingulate gyrus, thalamus were observed in 12 cases in T2 weighted image(T2 WI) and fluid attenuated inversion recovery(FLAIR) sequence.Diffusion weighted imaging(DWI) was performed in ten patients,eight of whom had lightly restricted diffusion.Eleven cases underwent enhanced MRI,two of whom had lightly enhancement.Bilateral or unilateral temporal horn enlargement were shown in 33 cases,six of whom were accompanied by total brain atrophy.~(18)FFDG PET-CT was performed in seven cases,and three cases had cerebral hypometabolism and MRI showed brain atrophy and hippocampal atrophy.1 case underwent proton magnetic resonance spectroscopy(~1H-MRS).N-acetylaspartic acid(NAA)/creatine(Cr) decreased and choline(Cho)/Cr increased in the abnormal signal region compared with the normal brain area on the opposite side.Conclusion Multiple brain areas,including limbic system,get involved in anti-NMDAR encephalitis.Enlarged temporal horn observed in more than 60.0% cases revealed by the first MRI examination may suggest that hippocampus was involved early before clinical onset of anti-NMDAR encephalitis.
Objective To investigate the clinical and imaging features of anti-N-methyl-D-aspartate receptor(anti-NMDAR) encephalitis.Methods A total of 53 patients with anti-NMDAR encephalitis admitted to Xuanwu Hospital Capital Medical University from January to February 2015 were enrolled in this study.Their clinical and imaging features were analyzed retrospectively.Results Among the 53 patients with anti-NMDAR encephalitis,24 were female(45.3%) and 29 were male(54.7%).Their age ranged from 6 to 72 years with a median age of 23 years(16 cases aged <18 years and 8 cases aged >45 years).The clinical course was 7 days to 2 years.Patients with abnormal MRI manifestation accounted for 69.8%(37/53),and abnormal signal in cerebral cortex and subcortical white matter, hippocampus, insula, cerebellum, pons, corpus callosum, basal nucleus, cingulate gyrus, thalamus were observed in 12 cases in T2 weighted image(T2 WI) and fluid attenuated inversion recovery(FLAIR) sequence.Diffusion weighted imaging(DWI) was performed in ten patients,eight of whom had lightly restricted diffusion.Eleven cases underwent enhanced MRI,two of whom had lightly enhancement.Bilateral or unilateral temporal horn enlargement were shown in 33 cases,six of whom were accompanied by total brain atrophy.~(18)FFDG PET-CT was performed in seven cases,and three cases had cerebral hypometabolism and MRI showed brain atrophy and hippocampal atrophy.1 case underwent proton magnetic resonance spectroscopy(~1H-MRS).N-acetylaspartic acid(NAA)/creatine(Cr) decreased and choline(Cho)/Cr increased in the abnormal signal region compared with the normal brain area on the opposite side.Conclusion Multiple brain areas,including limbic system,get involved in anti-NMDAR encephalitis.Enlarged temporal horn observed in more than 60.0% cases revealed by the first MRI examination may suggest that hippocampus was involved early before clinical onset of anti-NMDAR encephalitis.
引文
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[14]GRANEROD J,AMBROSE H E,DAVIES N W,et al.Causes of encephalitis and differences in their clinical presentations in England:a multicentre,population-based prospective study[J].Lancet Infect Dis,2010,10(12):835-844.DOI:10.1016/S1473-3099(10)70222-X.
[15]GABLE M S,SHERIFF H,DALMAU J,et al.The frequency of autoimmune N-Methyl-D-Aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the california encephalitis project[J].Clin Infect Dis,2012,54(7):899-904.DOI:10.1093/cid/cir1038.
[16]TITULAER M J,MCCRACKEN L,GABILONDO I,et al.Treatmenst and prognositic factors for long-term outcome in patients with anti-NMDA receptor encephalitis:an observational cohort study[J].Lancet Neurol,2013,12(2):157-165.DOI:10.1016/S1474-4422(12)70310-1.
[17]冯雪丹,胡永强,冯文良,等.抗NMDA受体脑炎45例特点和临床转归分析[J].脑与神经疾病杂志,2016,24(7):425-429.FENG X D,HU Y Q,FENG W L,et al.The clinical characteristics and outcomes of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis:a study of 45 cases[J].Journal of Brain and Nervous Diseases,2016,24(7):425-429.
[18]温晓艳,张迎娜,方华,等.20例抗N-甲基-D-天冬氨酸受体脑炎患者临床特点分析[J].中国实用神经疾病杂志,2017,20(13):37-40.DOI:10.3969/j.issn.1673-5110.2017.13.011.WEN X Y,ZHANG Y N,FANG H,et al.The analysis of characteristics in 20 patients with anti-NMDA receptor encephalitis[J].Chinese Journal of Practical Nervous Diseases,2017,20(13):37-40.DOI:10.3969/j.issn.1673-5110.2017.13.011.
[19]DEMAEREL P,WILMS G,ROBBERECHT W,et al.MRI of herpes simplex encephalitis[J].Neuroradiology,1992,34(6):490-493.
[20]隋邦生,吴恩惠,陈雁冰.磁共振诊断学[M].北京:人民卫生出版社,1994:389.
[21]鱼博浪,王泽忠,杨广夫,等.病毒性脑炎的CT和MRI诊断[J].中华放射学杂志,1995,29(12):837-840.
[22]SHYU C S,LEE H F,CHI C S,et al.Acute encephalitis with refractory,repetitive partial seizures[J].Brain Dev,2008,30(5):356-361.
[23]GABLE MS,GAVALI S,RADNER A,et al.Anti-NMDAreceptor encephalitis:report of ten cases and comparison with viral encephalitis[J].Eur J Clin Microbiol Infect Dis,2009,28(12):1421-1429.DOI:10.1007/s10096-009-0799-0.
[2]DALMAU J,ROSENFELD M R.Autoimmune encephalitis update[J].Neuro Oncol,2014,16(6):771-778.DOI:10.1093/neuonc/nou030.
[3]GRAUS F,TITULAER M J,BALU R,et al.A clinical approach to diagnosis of autoimmune encephalitis[J].Lancet Neurol,2016,15(4):391-404.DOI:10.1016/S1474-4422(15)00401-9.
[4]DALMALL J,GLEIEHMAN A J,HUGHES E G,et al.AntiNMDA-receptor encephalitis:case series and analysis of the effects of antibodies[J].Lancet Neurol,2008,7(12):1091-1098.DOI:10.1016/S1474-4422(08)70224-2.
[5]贺菲菲,叶静,董凯,等.抗N-甲基-D-天冬氨酸受体脑炎患者的临床、磁共振成像和脑脊液特征分析[J].中华神经科杂志,2014,47(10):691-694.DOI:10.3760/cma.j.issn.1006-7876.2014.10.005.HE F F,YE J,DONF K,et al.The characteristics of clinical manifestations,brain MRI and cerebrospinal fluid findings in patients with anti-N-methyl-D-aspartate receptor encephalitis[J].Chinese Journal of Neurology,2014,47(10):691-694.DOI:10.3760/cma.j.issn.1006-7876.2014.10.005.
[6]GASS A,AY H,SZABO K,et al.Diffusion-weighted MRI for the“small stuff”:the details of acute cerebral ischaemia[J].Lancet Neurol,2004,3(1):39-45.DOI:10.1016/S1474-4422(03)00621-5.
[7]张德清,王俭,牛俊巧,等.获得性免疫缺陷综合征局灶性脑炎1H-MRS分析[J].中国医学影像技术,2011,27(8):1541-1545.DOI:10.13929/j.1003-3289.2011.08.032.ZHANG D Q,WANG J,NIU J Q,et al.1H-MRS of acquired immunodeficiency syndrome related focal encephalitis[J].Chinese Medical Imaging Technology,2011,27(8):1541-1545.DOI:10.13929/j.1003-3289.2011.08.032.
[8]MAEDER-INGAVAR M,PRIOR J O,IRANI S R,et al.FDG-PET hyperactivity in basal ganglia correlating with clinical course in anti-NMDA-R antibodies encephalitis[J].J Neurol Neurosurg Psychiatry,2011,82(2):235-236.DOI:10.1136/jnnp.2009.198697.
[9]PILLAI S C,GILL D,WEBSTER R,et al.Cortical hypometabolism demonstrated by PET in relapsing NMDA receptor encephalitis[J].Pediatr Neurol,2010,43(3):217-220.DOI:10.1016/j.pediatrneurol.2010.04.019.
[10]LLORENS V,GABILONDO I,GóMEZ-ESTEBAN J C,et al.Abnormal multifocal cerebral blood flow on Tc-99m HMPAO SPECT in a patient with anti-NMDA-receptor encephalitis[J].J Neurol,2010,257(9):1568-1569.DOI:10.1007/s00415-010-5546-z.
[11]IIZUKA T,YOSHII S,KAN S,et al.Reversible brain atrophy in anti-NMDA receptor encephalitis:a long-term observational study[J].J Neurol,2010,257(10):1686-1691.DOI:10.1007/s00415-010-5604-6.
[12]TOBIN W O,STRAND E A,CLARK H M,et al.NMDAreceptor encephalitis causing reversible caudate changes on MRIand PET imaging[J].Neurol Clin Pract,2014,4(6):470-473.DOI:10.1212/CPJ.0000000000000074.
[13]FINKE C,KOPP U A,PAJKERT A,et al.Structural hippocampal damage following Anti-N-Methyl-D-Aspartate receptor encephalitis[J].Biol Psychiatry,2016,79(9):727-734.DOI:10.1016/j.biopsych.2015.02.024.
[14]GRANEROD J,AMBROSE H E,DAVIES N W,et al.Causes of encephalitis and differences in their clinical presentations in England:a multicentre,population-based prospective study[J].Lancet Infect Dis,2010,10(12):835-844.DOI:10.1016/S1473-3099(10)70222-X.
[15]GABLE M S,SHERIFF H,DALMAU J,et al.The frequency of autoimmune N-Methyl-D-Aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the california encephalitis project[J].Clin Infect Dis,2012,54(7):899-904.DOI:10.1093/cid/cir1038.
[16]TITULAER M J,MCCRACKEN L,GABILONDO I,et al.Treatmenst and prognositic factors for long-term outcome in patients with anti-NMDA receptor encephalitis:an observational cohort study[J].Lancet Neurol,2013,12(2):157-165.DOI:10.1016/S1474-4422(12)70310-1.
[17]冯雪丹,胡永强,冯文良,等.抗NMDA受体脑炎45例特点和临床转归分析[J].脑与神经疾病杂志,2016,24(7):425-429.FENG X D,HU Y Q,FENG W L,et al.The clinical characteristics and outcomes of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis:a study of 45 cases[J].Journal of Brain and Nervous Diseases,2016,24(7):425-429.
[18]温晓艳,张迎娜,方华,等.20例抗N-甲基-D-天冬氨酸受体脑炎患者临床特点分析[J].中国实用神经疾病杂志,2017,20(13):37-40.DOI:10.3969/j.issn.1673-5110.2017.13.011.WEN X Y,ZHANG Y N,FANG H,et al.The analysis of characteristics in 20 patients with anti-NMDA receptor encephalitis[J].Chinese Journal of Practical Nervous Diseases,2017,20(13):37-40.DOI:10.3969/j.issn.1673-5110.2017.13.011.
[19]DEMAEREL P,WILMS G,ROBBERECHT W,et al.MRI of herpes simplex encephalitis[J].Neuroradiology,1992,34(6):490-493.
[20]隋邦生,吴恩惠,陈雁冰.磁共振诊断学[M].北京:人民卫生出版社,1994:389.
[21]鱼博浪,王泽忠,杨广夫,等.病毒性脑炎的CT和MRI诊断[J].中华放射学杂志,1995,29(12):837-840.
[22]SHYU C S,LEE H F,CHI C S,et al.Acute encephalitis with refractory,repetitive partial seizures[J].Brain Dev,2008,30(5):356-361.
[23]GABLE MS,GAVALI S,RADNER A,et al.Anti-NMDAreceptor encephalitis:report of ten cases and comparison with viral encephalitis[J].Eur J Clin Microbiol Infect Dis,2009,28(12):1421-1429.DOI:10.1007/s10096-009-0799-0.