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Unique association of anti-GABAA receptor encephalitis and myasthenia gravis in a patient with type A thymoma

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Abstract

Association between anti-GABAAR encephalitis and myasthenia gravis is extremely rare with few reported cases. Herein, we report a case of a female patient diagnosed with anti-GABAAR encephalitis and thymoma at the first admission. She was administered glucocorticoids for long-term immunotherapy, and thymectomy with biopsy demonstrated a type A thymoma. After 4 months, the symptoms of encephalitis were relieved, but she then developed post-thymectomy myasthenia gravis with anti-AChR and anti-titin dual positivity. Antibodies to connective tissue (anti-ANA, anti-PCNA) and those characteristics of paraneoplastic syndrome (anti-Ma2/Ta) were also positive. She received oral glucocorticoids and tacrolimus as immunosuppressive therapy, and myasthenic symptoms were stable during a 2-year follow-up. Our case revealed that anti-GABAAR encephalitis and myasthenia gravis can appear in patient with type A thymoma at different periods, which alerts physicians to take long-term follow-up for anti-GABAAR encephalitis with thymoma, even after thymectomy. Concurrent positivity for more than one antibody after thymectomy is rarely observed, and their contribution to the clinical course and treatment decision remains to be further investigated.

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The original contributions of this study are included in the article and Supplementary Material. Further inquiries can be directed to the corresponding author.

References

  1. Abboud H, Probasco JC, Irani S et al (2021) Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry 92(7):757–768. https://doi.org/10.1136/jnnp-2020-325300

    Article  PubMed  Google Scholar 

  2. Ohkawa T, Satake S, Yokoi N et al (2014) Identification and characterization of GABA(A) receptor autoantibodies in autoimmune encephalitis. J Neurosci 34(24):8151–8163. https://doi.org/10.1523/JNEUROSCI.4415-13.2014

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Spatola M, Petit-Pedrol M, Simabukuro MM et al (2017) Investigations in GABA(A) receptor antibody-associated encephalitis. Neurology 88(11):1012–1020. https://doi.org/10.1212/WNL.0000000000003713

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Petit-Pedrol M, Armangue T, Peng X et al (2014) 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 13(3):276–286. https://doi.org/10.1016/S1474-4422(13)70299-0

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Guo CY, Gelfand JM, Geschwind MD (2020) Anti-gamma-aminobutyric acid receptor type A encephalitis: a review. Curr Opin Neurol 33(3):372–380. https://doi.org/10.1097/wco.0000000000000814

    Article  CAS  PubMed  Google Scholar 

  6. Uy CE, Binks S, Irani SR (2021) Autoimmune encephalitis: clinical spectrum and management. Pract Neurol 21(5):412–423. https://doi.org/10.1136/practneurol-2020-002567

    Article  PubMed  PubMed Central  Google Scholar 

  7. Lancaster E (2019) Encephalitis, severe seizures, and multifocal brain lesions: recognizing autoimmunity to the GABA(A) receptor. Neurol Neuroimmunol Neuroinflamm 6(3):e554. https://doi.org/10.1212/NXI.0000000000000554

    Article  PubMed  PubMed Central  Google Scholar 

  8. Spatola M, Dalmau J (2017) Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis. Curr Opin Neurol 30(3):345–353. https://doi.org/10.1097/wco.0000000000000449

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Menon D, Katzberg H, Barnett C et al (2021) Thymoma pathology and myasthenia gravis outcomes. Muscle Nerve 63(6):868–873. https://doi.org/10.1002/mus.27220

    Article  PubMed  Google Scholar 

  10. Chen P, Wang YP, Mou DL et al (2018) Pathological findings in myasthenia gravis patients with thymic hyperplasia and thymoma. Pathol Oncol Res 24(1):67–74. https://doi.org/10.1007/s12253-017-0213-7

    Article  CAS  PubMed  Google Scholar 

  11. Kim DJ, Yang WI, Kim SH et al (2005) Expression of neurotrophin receptors in surgically resected thymic epithelial tumors. Eur J Cardiothorac Surg 28(4):611–616. https://doi.org/10.1016/j.ejcts.2005.06.034

    Article  PubMed  Google Scholar 

  12. Rath J, Taborsky M, Moser B et al (2022) Short-term and sustained clinical response following thymectomy in patients with myasthenia gravis. Eur J Neurol 29(8):2453–2462. https://doi.org/10.1111/ene.15362

    Article  PubMed  PubMed Central  Google Scholar 

  13. Mineo TC, Tamburrini A, Schillaci O et al (2018) Onset and evolution of clinically apparent myasthenia gravis after resection of non-myasthenic thymomas. Semin Thorac Cardiovasc Surg 30(2):222–227. https://doi.org/10.1053/j.semtcvs.2018.02.027

    Article  PubMed  Google Scholar 

  14. Kato T, Kawaguchi K, Fukui T et al (2020) Risk factors for the exacerbation of myasthenic symptoms after surgical therapy for myasthenia gravis and thymoma. Semin Thorac Cardiovasc Surg 32(2):378–385. https://doi.org/10.1053/j.semtcvs.2019.09.002

    Article  PubMed  Google Scholar 

  15. Ronchi NR, Silva GD (2022) Comparison of the clinical syndromes of anti-GABAa versus anti-GABAb associated autoimmune encephalitis: a systematic review. J Neuroimmunol 363:577804. https://doi.org/10.1016/j.jneuroim.2021.577804

    Article  CAS  PubMed  Google Scholar 

  16. Zhang Y, Yu L, Ke J (2022) Pathological features and prognosis of thymoma with or without myasthenia gravis. Front Surg 9:726673. https://doi.org/10.3389/fsurg.2022.726673

    Article  PubMed  PubMed Central  Google Scholar 

  17. Deng B, Cai M, Qiu Y et al (2022) MRI characteristics of autoimmune encephalitis with autoantibodies to GABAA receptor: a case series. Neurol Neuroimmunol Neuroinflamm 9(3):e1158. https://doi.org/10.1212/NXI.0000000000001158

    Article  PubMed  PubMed Central  Google Scholar 

  18. Nakajima J, Murakawa T, Fukami T et al (2008) Postthymectomy myasthenia gravis: relationship with thymoma and antiacetylcholine receptor antibody. Ann Thorac Surg 86(3):941–945. https://doi.org/10.1016/j.athoracsur.2008.04.070

    Article  PubMed  Google Scholar 

  19. Uzawa A, Kanai T, Oda F et al (2020) Frequency and features of myasthenia gravis developing after thymectomy. Eur J Neurol 27(1):175–180. https://doi.org/10.1111/ene.14052

    Article  CAS  PubMed  Google Scholar 

  20. Kurihara M, Sasaki T, Sakuishi K et al (2020) Isolated seizure as initial presentation of GABA(A) receptor antibody-associated encephalitis. J Neurol Sci 410:116666. https://doi.org/10.1016/j.jns.2020.116666

    Article  CAS  PubMed  Google Scholar 

  21. Yasumizu Y, Ohkura N, Murata H et al (2022) Myasthenia gravis-specific aberrant neuromuscular gene expression by medullary thymic epithelial cells in thymoma. Nat Commun 13(1):4230. https://doi.org/10.1038/s41467-022-31951-8

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Rotondo Dottore G, Leo M, Ricciardi R et al (2021) Disappearance of anti-thyroid autoantibodies following thymectomy in patients with myasthenia gravis. Eur Thyroid J 10(3):237–247. https://doi.org/10.1159/000510701

    Article  CAS  PubMed  Google Scholar 

  23. Vincent A, Irani SR (2010) Caspr2 antibodies in patients with thymomas. J Thorac Oncol 5(10 Suppl 4):S277–S280. https://doi.org/10.1097/JTO.0b013e3181f23f04

    Article  PubMed  Google Scholar 

  24. Asakawa H, Kashihara T, Fukuda H et al (2002) A patient with thymoma and four different organ-specific autoimmune diseases. Neth J Med 60(7):292–295

    CAS  PubMed  Google Scholar 

  25. Mollaeian A, Haas C (2020) A tale of autoimmunity: thymoma, thymectomy, and systemic lupus erythematosus. Clin Rheumatol 39(7):2227–2234. https://doi.org/10.1007/s10067-020-05061-z

    Article  PubMed  PubMed Central  Google Scholar 

  26. Chen Y, Zhang XS, Wang YG et al (2021) Imbalance of Th17 and Tregs in thymoma may be a pathological mechanism of myasthenia gravis. Mol Immunol 133:67–76. https://doi.org/10.1016/j.molimm.2021.02.011

    Article  CAS  PubMed  Google Scholar 

  27. Shelly S, Agmon-Levin N, Altman A et al (2011) Thymoma and autoimmunity. Cell Mol Immunol 8(3):199–202. https://doi.org/10.1038/cmi.2010.74

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Prüss H (2021) Autoantibodies in neurological disease. Nat Rev Immunol 21(12):798–813. https://doi.org/10.1038/s41577-021-00543-w

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Funding

This study was supported by grants from the Natural Science Foundation of Hunan Province (Grant Numbers: 2022JJ40724, 2023JJ30062, and 2023JJ30868), Key Research and Development Program in Hunan Province (Grant Number: 2023SK2019), Science and Technology Innovation Guidance Project of Hunan Province (Grant Number: 2020SK53009), and the Changsha Municipal Natural Science Foundation (Grant Number: kq2007037).

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Authors

Contributions

SO and WY drafted the original manuscript and funded this study. XM, FW, and QZ participated in the data collection and analysis. HT, WL, and WP performed the data analysis and interpretation. WD and QZ assisted with the literature review. All authors have read the final manuscript and approved the submitted version.

Corresponding author

Correspondence to Weifan Yin.

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Ethics statement

The studies involving human participants were reviewed and approved by the Medical Ethics Committee of the Second Xiangya Hospital Central South University. We have obtained written informed consent from the study participant. All of the procedures were performed in accordance with the Declaration of Helsinki and relevant policies in China. Human participants and/or animals were not involved in this research.

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Ouyang, S., Wu, X., Zhan, Q. et al. Unique association of anti-GABAA receptor encephalitis and myasthenia gravis in a patient with type A thymoma. Neurol Sci 45, 2203–2209 (2024). https://doi.org/10.1007/s10072-023-07239-0

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