切换至 "中华医学电子期刊资源库"

中华诊断学电子杂志 ›› 2024, Vol. 12 ›› Issue (02) : 116 -119. doi: 10.3877/cma.j.issn.2095-655X.2024.02.009

病例诊断思维

抗二肽基肽酶样蛋白-6抗体相关脑炎的诊断学特征并文献复习
林立荣1, 郝洪军2,()   
  1. 1. 014010 包头,内蒙古包钢医院神经内科
    2. 100032 北京大学第一医院神经内科
  • 收稿日期:2023-11-11 出版日期:2024-05-26
  • 通信作者: 郝洪军

Diagnostic characteristics and literature review of anti-dipeptidyl-peptidase-like protein 6 antibody associated encephalitis

Lirong Lin1, Hongjun Hao2,()   

  1. 1. Department of Neurology, Inner Mongolia Baogang Hospital, Baotou 014010, China
    2. Department of Neurology, Peking University First Hospital, Beijing 100034, China
  • Received:2023-11-11 Published:2024-05-26
  • Corresponding author: Hongjun Hao
引用本文:

林立荣, 郝洪军. 抗二肽基肽酶样蛋白-6抗体相关脑炎的诊断学特征并文献复习[J]. 中华诊断学电子杂志, 2024, 12(02): 116-119.

Lirong Lin, Hongjun Hao. Diagnostic characteristics and literature review of anti-dipeptidyl-peptidase-like protein 6 antibody associated encephalitis[J]. Chinese Journal of Diagnostics(Electronic Edition), 2024, 12(02): 116-119.

目的

探讨抗二肽基肽酶样蛋白-6(DPPX)抗体相关脑炎的诊断学特征。

方法

回顾性分析2021年6月25日内蒙古包钢医院神经内科收治的1例血清抗DPPX抗体阳性患者的既往及现有临床资料,总结抗DPPX抗体相关脑炎的诊断学特征并复习相关文献。

结果

患者男性,53岁,因"右眼渐进性失明10 d"入院。眼科查体示双侧瞳孔不等大,左侧3.0 mm,右侧4.0 mm,右侧直接、间接光反射消失;右眼无光感,左眼0.4;眼底、黄斑区及眼底荧光造影未见异常;右眼视诱发未引出。头颅磁共振成像(MRI)未见新发梗死及占位性病变;动态脑电图大致正常;血清抗DPPX抗体++(1∶20滴度稀释),其他N-甲基-D-天冬氨酸(NMDA)受体脑炎抗体均阴性;脑脊液余项检查大致正常。住院期间给予营养神经治疗,住院6 d后下方视野有所恢复,有光感。出院诊断为抗DPPX抗体脑炎。患者既往于2018年3月8日因左侧肢体肌力减弱住院治疗,左侧肢体肌力为4级,左上肢痛觉减退,余查体阴性。头颅MRI未见新发梗死;颈部MRI示颈5/6水平髓内占位,诊断为星形细胞瘤,未予特殊治疗,自行康复锻炼,2020年3月左侧肢体肌力及感觉均完全恢复正常。

结论

本例抗DPPX抗体脑炎患者以偏侧肢体肌力减弱起病,且两次临床症状均有不同程度自行缓解情况,进一步证明该病可能存在自限性。

Objective

To explore the diagnostic characteristics of anti-dipeptidyl-peptidase-like protein 6 (DPPX) antibody associated encephalitis.

Methods

A retrospective analysis was conducted on the past and current clinical data of a patient with serum anti-DPPX antibody positive admitted to the Neurology Department of Inner Mongolia Baogang Hospital on June 25, 2021. The diagnostic characteristics of anti-DPPX antibody related encephalitis were summarized and relevant literatures were reviewed.

Results

The patient, a 53 year old male, was hospitalized due to progressive blindness in the right eye for 10 days. Ophthalmic examination showed that the pupils of both sides had unequal sizes, 3.0 mm on the left side and 4.0 mm on the right side, and the direct and indirect light reflection of right eye disappeared. Light perception of right eye disappeared, and the vision of the left eye was 0.4. The fundus, macular area and fundus fluorescein showed no abnormality. Visual evoked response in the right eye was not elicited. No new infarctions or occupying lesions were found on cranial MRI. The dynamic electroencephalogram was generally normal. Serum anti-DPPX antibody + + (diluted at 1∶20 titers), all other N-methyl-D-aspartic acid (NMDA) receptors encephalitis antibodies were negative. The remaining items of the cerebrospinal fluid examination were generally normal. During hospitalization, nutritional nerve therapy was given, and after 6 days of hospitalization, the lower field of vision and light perception recovered and there was a sense of light. The discharge diagnosis was anti-DPPX antibody encephalitis. The patient was admitted to the hospital for treatment on March 8, 2018 due to left limb strength. At the time of admission, a neurological examination showed that the left limb strength was level 4, with reduced pain sensation in the left upper limb and negative results on other examinations. Complete cranial MRI showed no new infarctions. Neck MRI showed a intramedually mass at the C5/6 level of the neck, diagnosed as astrocytoma. No special treatment was given, and only self rehabilitation exercise was performed. In March 2020, the left limb strength and sensation fully returned to normal.

Conclusion

The patient with anti-DPPX antibody encephalitis in this case started with weak unilateral limb strength, and both clinical symptoms showed varying degrees of self relief, further proving that the disease may exhibit self-limiting characteristics.

图1 抗DPPX抗体脑炎患者颈椎MRI图像注:a、b图分别为颈椎磁共振T2、T1加权像,均无异常信号影;DPPX为二肽基肽酶样蛋白-6;患者因右眼渐进性失明住院
图2 抗DPPX抗体脑炎颈椎MRI图像注:a、b图为T1、T2加权像;c图为增强序列;a~c图示颈5/6水平脊髓肿胀,内可见片状长T1长T2信号,边界不清,增强后病灶明显强化;DPPX为二肽基肽酶样蛋白-6;患者因左侧肢体肌力减弱住院
[1]
Boronat AGelfand JMGresa-Arribas N,et al.Encephalitis and antibodies to dipeptidyl-peptidase-like protein-6,a subunit of Kv 4.2 potassium channels[J].Ann Neurol201373(1):120-128.DOI:10.1002/ana.23756.
[2]
Hara MAriño HPetit Pedrol M,et al.DPPX antibody associated encephalitis:main syndrome and antibody effects[J].Neurology201788(14):1340 1348.DOI:10.1212/WNL.0000000000003796.
[3]
理二闯,章殷希,丁美萍.二肽基肽酶样蛋白-6抗体相关脑炎临床诊治进展[J].中华神经科杂志202154(4):424-428.DOI:10.3760/cma.j.cn113694-20200702-00495.
[4]
Wang XTang HWei EQ,et al.Conditional knockout of Fgf13 in murine hearts increases arrhythmia susceptibility and reveals novel ion channel modulatory roles[J].J Mol Cell Cardiol2017(104):63-74.DOI:10.1016/j.yjmcc.2017.01.009.
[5]
Piepgras JHöltje MMichel K,et al.Anti-DPPX encephalitis:pathogenic effects of antibodies on gut and brain neurons[J].Neurology201585(10):890-897.DOI:10.1212/WNL.0000000000001907.
[6]
Doherty LGold DSolnes L,et al.Anti-DPPX encephalitis:prominent nystagmus reflected by extraocular muscle FDG-PET avidity[J].Neurol Neuroimmunol Neuroinflamm20174(4):e361.DOI:10.1212/NXI.0000000000000361.
[7]
古祺,毛悦时.自身免疫性脑炎的新类型与新进展[J].中国临床医学201522(6):832-836.
[8]
Tobin WOLennon VAKomorowski L,et al.DPPX potassium channel antibody:frequency,clinical accompaniments,and outcomes in 20 patients[J].Neurology201483(20):1797-1803.DOI:10.1212/WNL.0000000000000991.
[9]
Micieli JANewman NJKase CS,et al.Teaching video neuroimages:opsoclonus in anti DPPX encephalitis[J].Neurology201992(19):e2298.DOI:10.1212/WNL.0000000000007463.
[10]
Cellucci TVan Mater HGraus F,et al.Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient[J].Neurol Neuroimmunol Neuroinflamm20207(2):e663.DOI:10.1212/NXI.0000000000000663.
[11]
Stoeck KCarstens POJarius S,et al.Prednisolone and azathioprine are effective in DPPX antibody-positive autoimmune encephalitis[J].Neurol Neuroimmunol Neuroinflamm20152(3):e86.DOI:10.1212/NXI.0000000000000086.
[1] 徐燕, 茹彤, 郑明明, 顾燕, 朱湘玉, 严陈晨, 陈玲, 戴晨燕. Miller-Dieker综合征胎儿产前超声、磁共振影像学特征及遗传学分析[J]. 中华医学超声杂志(电子版), 2024, 21(03): 281-287.
[2] 罗敏华, 王文平, 孔文韬. 肝脏炎性假瘤的超声造影表现及其诊断价值[J]. 中华医学超声杂志(电子版), 2024, 21(03): 297-303.
[3] 钟佩芝, 杜宇. 龋病诊断方法的研究进展[J]. 中华口腔医学研究杂志(电子版), 2024, 18(02): 73-79.
[4] 杜彦斌, 黄涛, 寇天阔, 石英. 双镜联合根治术与腹腔镜根治术在早期结肠癌患者中的应用效果[J]. 中华普外科手术学杂志(电子版), 2024, 18(03): 275-278.
[5] 焦盛宇, 雷庆军, 周圣赢, 王兵, 孔智渊, 张剑, 徐小慧. 35例闭孔疝患者临床诊断和治疗分析[J]. 中华疝和腹壁外科杂志(电子版), 2024, 18(02): 202-207.
[6] 杨智義, 赵成俊, 胡欣芫, 潘佰猛, 张秋雨, 张挽乾, 曹芮, 张灵强. 外周血cfDNA液体活检技术在肝棘球蚴病诊治中的应用进展[J]. 中华肝脏外科手术学电子杂志, 2024, 13(03): 389-393.
[7] 白睿, 孙备. 自身免疫性胰腺炎和胰腺导管腺癌鉴别的策略与思考[J]. 中华肝脏外科手术学电子杂志, 2024, 13(02): 121-126.
[8] 中国医师协会骨科医师分会肩肘外科学组. 桡骨头骨折诊疗中国专家共识(2024版)[J]. 中华肩肘外科电子杂志, 2024, 12(02): 97-102.
[9] 王素霞. IgA肾病的病理诊断与鉴别诊断[J]. 中华肾病研究电子杂志, 2024, 13(02): 120-120.
[10] 鲁悦, 李伟, 庄宗, 王娟, 赵鹏来, 杭春华. 脑出血继发吉兰-巴雷综合征二例报道并文献复习[J]. 中华脑科疾病与康复杂志(电子版), 2024, 14(02): 120-123.
[11] 汪久宏, 丁莉, 梁丽. 腹部彩超联合高频彩超用于小儿肠套叠诊断经验及价值[J]. 中华消化病与影像杂志(电子版), 2024, 14(03): 226-228.
[12] 安亚楠, 王端然, 郭甜甜, 武希润. 幽门螺杆菌阴性胃黏膜相关淋巴组织淋巴瘤的研究进展[J]. 中华消化病与影像杂志(电子版), 2024, 14(03): 268-274.
[13] 陈娟, 胡晓华, 李洪梅, 王志军. CT小肠造影对克罗恩病的诊断评估价值[J]. 中华消化病与影像杂志(电子版), 2024, 14(01): 41-44.
[14] 刘兆全, 张芳芳, 宋洪浩, 王刚, 崔明宇. 儿童腹腔炎性肌纤维母细胞瘤的诊断学特征并文献复习[J]. 中华诊断学电子杂志, 2024, 12(02): 101-106.
[15] 谌燕, 冯宗辉, 姜淑敏, 李敏, 易凤梅, 谭颖. 结节性硬化症一家系的TSC2基因变异分析和产前诊断[J]. 中华诊断学电子杂志, 2024, 12(02): 112-115.
阅读次数
全文


摘要