Clinical Neurology: Research Article

Li C.a· Cai H.b· Zhao X.c· Xi X.d· Zhou Q.a· Luo H.e· Tang Z.a· Kang H.a· Kirsch H.E.f

Author affiliations

aDepartment of Neurology, Tongji Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
bDepartment of Neurology, Department of Pneumology, No. 9 Hospital of Wuhan City, Wuhan, China
cDepartment of Radiology, Tongji Hospital Affiliated to Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
dDepartment of Radiology and Intervention, No. 6 Hospital of Shanghai City, Shanghai, China
eDepartment of Pathophysiology, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
fDepartment of Neurology and Radiology & Biomedical Imaging, Epilepsy Center, University of California, San Francisco, CA, USA

Log in to MyKarger to check if you already have access to this content.

Buy FullText & PDF Unlimited re-access via MyKarger Unrestricted printing, no saving restrictions for personal use read more

CHF 38.00 *
EUR 35.00 *
USD 39.00 *

Select

KAB

Buy a Karger Article Bundle (KAB) and profit from a discount!

If you would like to redeem your KAB credit, please log in.

Save over 20% compared to the individual article price.

Learn more

Access via DeepDyve Unlimited fulltext viewing Of this article Organize, annotate And mark up articles Printing And downloading restrictions apply

Select

Subscribe Access to all articles of the subscribed year(s) guaranteed for 5 years Unlimited re-access via Subscriber Login or MyKarger Unrestricted printing, no saving restrictions for personal use read more

Subcription rates

Select

* The final prices may differ from the prices shown due to specifics of VAT rules.

Article / Publication Details

First-Page Preview

Received: January 11, 2022
Accepted: April 30, 2022
Published online: July 27, 2022

Number of Print Pages: 9
Number of Figures: 2
Number of Tables: 2

ISSN: 0014-3022 (Print)
eISSN: 1421-9913 (Online)

For additional information: https://www.karger.com/ENE

Abstract

Introduction: Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is clinically heterogeneous, especially at presentation, and though it is sometimes found in association with tumor, this is by no means the rule. Methods: Clinical data for 10 patients with anti-LGI1 encephalitis were collected including one case with teratoma and nine cases without and compared for clinical characteristics. Microscopic pathological examination and immunohistochemical assay of the LGI1 antibody were performed on teratoma tissue obtained by laparoscopic oophorocystectomy. Results: In our teratoma-associated anti-LGI1 encephalitis case, teratoma pathology was characterized by mostly thyroid tissue and immunohistochemical assay confirmed positive nuclear staining of LGI1 in some tumor cells. The anti-LGl1 patient with teratoma was similar to the non-teratoma cases in many ways: age at onset (average 47.3 in non-teratoma cases); percent presenting with rapidly progressive dementia (67% of non-teratoma cases) and psychiatric symptoms (33%); hyponatremia (78%); normal cerebrospinal fluid results except for positive LGI1 antibody (78%); bilateral hippocampal hyperintensity on magnetic resonance imaging (44%); diffuse slow waves on electroencephalography (33%); good response to immunotherapy (67%); and mild residual cognitive deficit (22%). Her chronic anxiety and presentation with status epilepticus were the biggest differences compared with the non-teratoma cases. Conclusion: In our series, anti-LGI1 encephalitis included common clinical features in our series: rapidly progressive dementia, faciobrachial dystonic seizures, behavioral disorders, hyponatremia, hippocampal hyperintensity on magnetic resonance imaging, and residual cognitive deficit. We observed some differences (chronic anxiety and status epilepticus) in our case with teratoma, but a larger accumulation of cases is needed to improve our knowledge base.

© 2022 S. Karger AG, Basel

References Suh-Lailam BB, Haven TR, Copple SS, Knapp D, Jaskowski TD, Tebo AE. Anti-NMDA-receptor antibody encephalitis: performance evaluation and laboratory experience with the anti-NMDA-receptor IgG assay. Clin Chim Acta. 2013;421:1–6. van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, de Bruijn MA, et al. Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up. Neurology. 2016;87(14):1449–56. Sun L, Cao J, Liu C, Lv Y. Creutzfeldt-Jakob disease versus anti-LGI1 limbic encephalitis in a patient with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal electroencephalogram: a case report. Neuropsychiatr Dis Treat. 2015;11:1427–30. Oyanguren B, Sánchez V, González FJ, de Felipe A, Esteban L, López-Sendón JL, et al. Limbic encephalitis: a clinical-radiological comparison between herpetic and autoimmune etiologies. Eur J Neurol. 2013;20(12):1566–70. Ramalho J, Castillo M. Hashimoto’s encephalopathy. Radiol Case Rep. 2011;6(1):445. McKeon A. Autoimmune encephalopathies and dementias. Continuum. 2016;22(2 Dementia):538–58. Kaas B, Zeiler SR, Bahouth MN, Llinas RH, Probasco JC. Autoimmune limbic encephalitis in association with acute stroke. Neurol Clin Pract. 2018;8(4):349–51. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000;123(Pt 7):1481–94. Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36. Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, et al. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol. 2009;66(1):11–8. Lai M, Huijbers MG, Lancaster E, Graus F, Bataller L, Balice-Gordon R, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9(8):776–85. Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, 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. Brain. 2010;133(9):2734–48. Gadoth A, Pittock SJ, Dubey D, McKeon A, Britton JW, Schmeling JE, et al. Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients. Ann Neurol. 2017;82(1):79–92. Zhao X, Zhou ZQ, Xiong Y, Chen X, Xu K, Li J, et al. Reduced interhemispheric white matter asymmetries in medial temporal lobe epilepsy with hippocampal sclerosis. Front Neurol. 2019;10:394. Hou TY, Zhou Y, Zhu LS, Wang X, Pang P, Wang DQ, et al. Correcting abnormalities in miR-124/PTPN1 signaling rescues tau pathology in Alzheimer’s disease. J Neurochem. 2020;154:441. Fröhlich E, Wahl R. Thyroid autoimmunity: role of anti-thyroid antibodies in thyroid and extra-thyroidal diseases. Front Immunol. 2017;8:521. Gresa-Arribas N, Titulaer MJ, Torrents A, Aguilar E, McCracken L, Leypoldt F, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol. 2014;13(2):167–77. Lynch DR, Rattelle A, Dong YN, Roslin K, Gleichman AJ, Panzer JA. Anti-NMDA receptor encephalitis: clinical features and basic mechanisms. Adv Pharmacol. 2018;82:235–60. van Sonderen A, Petit-Pedrol M, Dalmau J, Titulaer MJ. The value of LGI1, Caspr2 and voltage-gated potassium channel antibodies in encephalitis. Nat Rev Neurol. 2017;13(5):290–301. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7(12):1091–8. Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011;10(1):63–74. Maureille A, Fenouil T, Joubert B, Picard G, Rogemond V, Pinto AL, et al. Isolated seizures are a common early feature of paraneoplastic anti-GABAB receptor encephalitis. J Neurol. 2019;266(1):195–206. Ariño H, Armangué T, Petit-Pedrol M, Sabater L, Martinez-Hernandez E, Hara M, et al. Anti-LGI1-associated cognitive impairment: presentation and long-term outcome. Neurology. 2016;87(8):759–65. Ng AC, Tripic M, Mirsattari SM. Teratoma-negative anti-NMDA receptor encephalitis presenting with a single generalized tonic-clonic seizure. Epilepsy Behav Case Rep. 2018;10:29–31. Petit-Pedrol M, Armangue T, Peng X, Bataller L, Cellucci T, Davis R, et al. Encephalitis with refractory seizures, status epilepticus, and antibodies to the GABAA receptor: a case series, characterisation of the antigen, and analysis of the effects of antibodies. Lancet Neurol. 2014;13(3):276–86. Chen X, Liu F, Li JM, Xie XQ, Wang Q, Zhou D, et al. Encephalitis with antibodies against the GABAB receptor: seizures as the most common presentation at admission. Neurol Res. 2017;39(11):973–80. Wingfield T, McHugh C, Vas A, Richardson A, Wilkins E, Bonington A, et al. Autoimmune encephalitis: a case series and comprehensive review of the literature. QJM. 2011;104(11):921–31. Li LH, Ma CC, Zhang HF, Lian YJ. Clinical and electrographic characteristics of seizures in LGI1-antibody encephalitis. Epilepsy Behav. 2018;88:277–82. Höftberger R, Titulaer MJ, Sabater L, Dome B, Rózsás A, Hegedus B, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology. 2013;81(17):1500–6. Article / Publication Details

First-Page Preview

Received: January 11, 2022
Accepted: April 30, 2022
Published online: July 27, 2022

Number of Print Pages: 9
Number of Figures: 2
Number of Tables: 2

ISSN: 0014-3022 (Print)
eISSN: 1421-9913 (Online)

For additional information: https://www.karger.com/ENE

Copyright / Drug Dosage / Disclaimer Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.