Journal of Shandong University (Health Sciences) ›› 2019, Vol. 57 ›› Issue (5): 105-109.doi: 10.6040/j.issn.1671-7554.0.2018.1405

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A clinical study of 5 cases of leucine-rich glioma inactivated 1 protein antibody associated limbic encephalitis

LI Hongyan1, FANG Lei2, ZHANG Hongying3, SU Yuan1, LI Ling1   

  1. 1. Department of Neurology, Qilu Hospital of Shandong University(Qingdao), Qingdao 266035, Shandong, China;
    2. Department of Neurology, Qingdao Central Hospital, Qingdao 266042, Shandong, China;
    3. Department of Neurosurgery, Qilu Hospital of Shandong University(Qingdao), Qingdao 266035, Shandong, China
  • Published:2022-09-27

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Abstract: Objective To explore the clinical features, auxiliary examination results and therapeutic outcomes of leucine-rich glioma inactived-1 antibody(anti-LGI1)associated limbic encephalitis. Methods The clinical data of 5 cases of anti-LGI1 associated limbic encephalitis treated during July 2017 and May 2018 were retrospectively analyzed, including the clinical manifestations, laboratory examinations, radiological features, long-term video-electroencephalogram, treatments and prognosis. Results The patients were three male and two female. Memory deficit was observed in 4 cases, seizure in 3 cases, faciobrachial dystonic seizures(FBDS)in all cases, hyponatremia in 3 cases, and positive anti-LGI1 antibodies both in the cerebrospinal fluid and blood serum in all cases. Magnetic resonance imaging(MRI)showed no T2WI or flair-phase high signal in the medial temporal lobes in 5 cases, but high signal in basal ganglia in T1WI in 1 case. Long-term video-electroencephalographs of 4 cases showed frequent FBDS. All patients received immunotherapy. During the follow-up, seizure recurred in 2 cases, and mental disturbance recurred in 1 case. Conclusion Anti-LGI1 associated limbic encephalitis is an autoimmune encephalitis characterized by early memory loss, FBDS and seizure. It is often complicated with hyponatremia. Temporallobe and hippocampus are often involved. Immunotherapy is effective but relapses are common.

Key words: Leucine-rich glioma inactivated-1 protein, Limbic encephalitis, Memory loss, Faciobrachial dystonic seizures

CLC Number: 

  • R742
[1] Demaerel P, Van Dessel W, Van paesschen W, et al. Autoimmune- mediated encephalitis [J]. Neuroradiology, 2011, 53(11): 837-851.
[2] Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis [J]. Annals of Neurology, 2011, 69(5): 892-900.
[3] 张元杏, 杨辉丽, 吴颖颖, 等. 抗富亮氨酸胶质瘤失活1蛋白抗体相关边缘性脑炎九例临床分析[J]. 中华医学杂志, 2017, 97(17): 1295-1298. ZHANG Yuanxing, YANG Huili, WU Yingying, et al. Clinical analysis of 9 cases with Anti-leucine-rich glioma inactivated 1 protein antibody associated limbic encephalitis [J]. Zhonghua Yi Xue Za Zhi, 2017, 97(17): 1295-1298.
[4] Irani SR, Alexander S, Waters P, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvans syndrome and acquired neuromyotonia [J]. Brain, 2010, 133: 2734-2748.
[5] Lai M, Huijbers MG, Lancaster E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series [J]. Lancet Neurol, 2010, 9(8): 776-785.
[6] Fukata Y, Lovero KL, Iwanaga T, et al. Disruption of LGI1-linked synaptic complex causes abnormal synaptic transmission and epilepsy [J]. Proc Natl Acad Sci USA 2010, 107(8): 3799-3804.
[7] Ohkawa T, Fukata Y, Yamasaki M, et al. Autoantibodies to epilepsy-related LGI1 in limbic encephalitis neutralize LGI1-ADAM22 interaction and reduce synaptic AMPA receptors [J]. J Neurosci, 2013, 33(46): 18161-18174.
[8] van Sonderen A, Petit-Pedrol M, Dalmau J, et al. The value of LGI1,Caspr2 and voltage-gated potassium channel antibodies in encephalitis [J]. Nat Rev Neurol, 2017, 13(5): 290-301.
[9] Dalmau J, Graus F. Antibody-mediated encephalitis [J]. N Engl J Med, 2018, 378(9): 840-851.
[10] Aradillas E, Schwartzmann RJ. Kinesigenic dyskinesia in a case of voltage-gated potassium channel-complex protein antibody encephalitis [J]. Arch Neurol, 2011, 68(4): 529-532.
[11] Striano P. Faciobrachial dystonic attacks: seizures or movement disorder? [J]. Ann Neurol, 2011, 70(1): 179-180.
[12] Beimer NJ, Selwa LM. Seizure semiology of anti-LGI1 antibody encephalitis [J]. Epileptic Disord, 2017, 19(4): 461-464.
[13] Iyer RS, Ramakrishnan TCR, Karunakaran, et al. Faciobrachial dystonic seizures result from fronto-temporo-basalganglial network involvement [J]. Epilepsy Behav Case Rep, 2017, 8: 47-50. doi: 10.1016/j.ebcr.2017.06.001.
[14] Wennberg R, Steriade C, Chen R, et al. Frontal infraslow activity marks the motor spasms of anti-LGI1 encephalitis [J]. Clin Neurophysiol, 2018, 129(1): 59-68.
[15] Aurangzeb S, Symmonds M, Knight RK, et al. LGI1-antibody encephalitis is characterised by frequent, multifocal clinical and subclinical seizures [J]. Seizure, 2017, 50(8): 14-17.
[16] Grativvol RS, Simabukuro MM, Spera RR, et al. Imaging findings in faciobrachial dystonic seizures associated with LGI1 antibodies [J]. Arq Neuropsiquiatr, 2016, 74(11): 947.
[17] Flanagan EP, Kotsenas AL, Britton JW, et al. Basal ganglia T1 hyperintensity in LGI1-autoantibody faciobrachial dystonic seizures[J]. Neurol Neuroimmunol Neuroinflamm, 2015, 2(6): e161. doi: 10.1212/NXI.0000000000000161.
[18] Finke C, Prüss H, Heine J, et al. Evaluation of cognitive deficits and structural hippocampal damage in encephalitis with leucine-rich, glioma-inactivated 1 antibodies [J]. JAMA Neurol, 2017, 74(1): 50-59.
[19] Steriade C, Mirsattari SM, Murray BJ, et al. Subclinical temporal EEG seizure pattern in LGI1-antibody-mediated encephalitis [J]. Epilepsia, 2016, 57(8): e155-160. doi: 10.1111/epi.13436.
[20] Yu J, Yu X, Fang S, et al. The Treatment and Follow-Up of Anti-LGI1 Limbic Encephalitis [J]. Eur Neurol, 2016, 75(1-2): 5-11.
[21] Shin YW, Lee ST, Shin JW, et al. VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy [J]. J Neuroimmunol, 2013, 265(1-2): 75-81.
[22] van Sonderen A, Thijs RD, Coenders EC, et al. Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up [J]. Neurology, 2016, 87(14): 1449-1456.
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