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Clinical characteristics and factors associated with short-term prognosis in adult patients with autoimmune encephalitis of non-neoplastic etiology

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Abstract

Background

Reports that autoimmune encephalitis (AE) is associated with antibodies have increased; however, little is known about the distribution of clinical symptoms, imaging changes, and prognostic factors in patients with AE of non-neoplastic etiology. Accordingly, we evaluated the clinical characteristics and factors associated with short-term prognosis.

Methods

From January 2016 to June 2018, 31 adult patients were diagnosed with AE of non-neoplastic etiology at Shengjing Hospital of China Medical University and their demographic and clinical characteristics were abstracted. Factors affecting disease severity and predictors of prognosis were analyzed.

Results

Among 31 patients, 19 had anti-NMDAR, 5 had anti-GABABR, and 7 had anti-LGI1 antibody encephalitis. Status epilepticus, ataxia, and cognitive dysfunction were the most common neurological symptoms. Deep white matter (DWM) abnormalities were the most common changes observed on MRI. Logistic regression analysis indicated that conscious disturbance (odds ratio = 11.67, 95%, confidence interval 2.13–64.04; p = 0.005) is an independent factor associated with poor prognosis in AE.

Conclusion

The clinical manifestations of AE are diverse; status epilepticus, ataxia, and cognitive dysfunction are most common. The DWM of the brain, rather than the limbic lobe system, was most prone to MR signal abnormalities. Conscious disturbance may be an important predictor of poor short-term prognosis in patients with AE of non-neoplastic etiology.

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References

  1. Newman MP, Blum S, Wong RCW, Scott JG, Prain K, Wilson RJ, Gillis D (2016) Autoimmune encephalitis. Intern Med J 46(2):148–157. https://doi.org/10.1111/imj.12974

    Article  CAS  PubMed  Google Scholar 

  2. Lancaster E (2016) The diagnosis and treatment of autoimmune encephalitis. J Clin Neurol 12(1):1–13. https://doi.org/10.3988/jcn.2016.12.1.1

    Article  PubMed  Google Scholar 

  3. Lancaster E, Dalmau J (2012) Neuronal autoantigens-pathogenesis, associated disorders and antibody testing. Nat Rev Neurol 8(7):380–390. https://doi.org/10.1038/nrneurol.2012.99

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Graus F, Saiz A, Dalmau J (2010) Antibodies and neuronal autoimmune disorders of the CNS. J Neurol 257(4):509–517. https://doi.org/10.1007/s00415-009-5431-9

    Article  CAS  PubMed  Google Scholar 

  5. Lancaster E, Martinez-Hernandez E, Dalmau J (2011) Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology 77(2):179–189. https://doi.org/10.1212/WNL.0b013e318224afde

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Dash D, Ihtisham K, Tripathi M, Tripathi M (2018) Proportion and spectrum of movement disorders in adolescent and adult patients of autoimmune encephalitis of non-neoplastic aetiology. J Clin Neurosci 59:185–189. https://doi.org/10.1016/j.jocn.2018.10.076

    Article  PubMed  Google Scholar 

  7. Hermetter C, Fazekas F, Hochmeister S (2018) Systematic review: syndromes, early diagnosis, and treatment in autoimmune encephalitis. Front Neurol 9. https://doi.org/10.3389/fneur.2018.00706

  8. Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, Cortese I, Dale RC, Gelfand JM, Geschwind M, Glaser CA, Honnorat J, Hoeftberger R, Iizuka T, Irani SR, Lancaster E, Leypoldt F, Pruess H, Rae-Grant A, Reindl M, Rosenfeld MR, Rostasy K, Saiz A, Venkatesan A, Vincent A, Wandinger K-P, Waters P, Dalmau J (2016) A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 15(4):391–404. https://doi.org/10.1016/s1474-4422(15)00401-9

    Article  PubMed  PubMed Central  Google Scholar 

  9. Bamford JM, Sandercock PA, Warlow CP, Slattery J (1989) Interobserver agreement for the assessment of handicap in stroke patients. Stroke 20(6):828–828. https://doi.org/10.1161/01.str.20.6.828

    Article  CAS  PubMed  Google Scholar 

  10. Teasdale G, Jennett B (1974) Assessment of coma and impaired consciousness. A practical scale. Lancet 2(7872):81–84

    Article  CAS  PubMed  Google Scholar 

  11. Dalmau J, Tuzun E, Wu H-y, Masjuan J, Rossi JE, Voloschin A, Baehring JM, Shimazaki H, Koide R, King D, Mason W, Sansing LH, Dichter MA, Rosenfeld MR, Lynch DR (2007) Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 61(1):25–36. https://doi.org/10.1002/ana.21050

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Kalman B (2017) Autoimmune encephalitides: a broadening field of treatable conditions. Neurologist 22(1):1–13. https://doi.org/10.1097/nrl.0000000000000087

    Article  PubMed  Google Scholar 

  13. Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, Suzuki K, Lynch DR, Suzuki N, Hata T, Dalmau J (2008) Anti-NMDA receptor encephalitis in Japan. Neurology 70(7):504–511. https://doi.org/10.1212/01.wnl.0000278388.90370.c3

    Article  CAS  PubMed  Google Scholar 

  14. Takano S, Takahashi Y, Kishi H, Taguchi Y, Takashima S, Tanaka K, Muraguchi A, Mori H (2011) Detection of autoantibody against extracellular epitopes of N-methyl-D-aspartate receptor by cell-based assay. Neurosci Res 71(3):294–302. https://doi.org/10.1016/j.neures.2011.07.1834

    Article  CAS  PubMed  Google Scholar 

  15. Ishiura H, Matsuda S, Higashihara M, Hasegawa M, Hida A, Hanajima R, Yamamoto T, Shimizu J, Dalmau J, Tsuji S (2008) Response of anti-NMDA receptor encephalitis without tumor to immunotherapy including rituximab. Neurology 71(23):1921–1923. https://doi.org/10.1212/01.wnl.0000336648.43562.59

    Article  CAS  PubMed  Google Scholar 

  16. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, Dessain SK, Rosenfeld MR, Balice-Gordon R, Lynch DR (2008) Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 7(12):1091–1098. https://doi.org/10.1016/s1474-4422(08)70224-2

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Viaccoz A, Desestret V, Ducray F, Picard G, Cavillon G, Rogemond V, Antoine J-C, Delattre J-Y, Honnorat J (2014) Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis. Neurology 82(7):556–563. https://doi.org/10.1212/wnl.0000000000000126

    Article  CAS  PubMed  Google Scholar 

  18. van Sonderen A, Schreurs MWJ, Wirtz PW, Smitt PAES, Titulaer MJ (2016) From VGKC to LGI1 and Caspr2 encephalitis: the evolution of a disease entity over time. Autoimmun Rev 15(10):970–974. https://doi.org/10.1016/j.autrev.2016.07.018

    Article  CAS  PubMed  Google Scholar 

  19. Ramanathan S, Mohammad SS, Brilot F, Dale RC (2014) Autoimmune encephalitis: recent updates and emerging challenges. J Clin Neurosci 21(5):722–730. https://doi.org/10.1016/j.jocn.2013.07.017

    Article  CAS  PubMed  Google Scholar 

  20. Irani SR, Michell AW, Lang B, Pettingill P, Waters P, Johnson MR, Schott JM, Armstrong RJE, Zagami AS, Bleasel A, Somerville ER, Smith SMJ, Vincent A (2011) Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol 69(5):892–900. https://doi.org/10.1002/ana.22307

    Article  PubMed  Google Scholar 

  21. Arino H, Armangue T, Petit-Pedrol M, Sabater L, Martinez-Hernandez E, Hara M, Lancaster E, Saiz A, Dalmau J, Graus F (2016) Anti-LGI1-associated cognitive impairment: presentation and long-term outcome. Neurology 87(8):759–765. https://doi.org/10.1212/wnl.0000000000003009

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Benarroch EE (2012) GABA(B) receptors structure, functions, and clinical implications. Neurology 78(8):578–584. https://doi.org/10.1212/WNL.0b013e318247cd03

    Article  CAS  PubMed  Google Scholar 

  23. Kim T-J, Lee S-T, Shin J-W, Moon J, Lim J-A, Byun J-I, Shin Y-W, Lee K-J, Jung K-H, Kim Y-S, Park K-I, Chu K, Lee SK (2014) Clinical manifestations and outcomes of the treatment of patients with GABA(B) encephalitis. J Neuroimmunol 270(1–2):45–50. https://doi.org/10.1016/j.jneuroim.2014.02.011

    Article  CAS  PubMed  Google Scholar 

  24. Hoeftberger R, Sabater L, Kruer M, Boyero S, de Felipe A, Saiz A, Graus F, Dalmau J (2013) Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. J Neurol 260:S21–S21

    Article  Google Scholar 

  25. Heine J, Pruess H, Bartsch T, Ploner CJ, Paul F, Finke C (2015) Imaging of autoimmune encephalitis - relevance for clinical practice and hippocampal function. Neuroscience 309:68–83. https://doi.org/10.1016/j.neuroscience.2015.05.037

    Article  CAS  PubMed  Google Scholar 

  26. Liao S, Qian Y, Hu H, Niu B, Guo H, Wang X, Miao S, Li C, Cao B (2017) Clinical characteristics and predictors of outcome for onconeural antibody-associated disorders: a retrospective analysis. Front Neurol 8. https://doi.org/10.3389/fneur.2017.00584

  27. Aungsumart S, Ha A, Apiwattanakul M (2018) Abnormal level of consciousness predicts outcomes of patients with anti-NMDA encephalitis. J Clin Neurosci 62:184–187. https://doi.org/10.1016/j.jocn.2018.11.033

    Article  PubMed  Google Scholar 

  28. Yang S, Qin J, Li J, Gao Y, Zhao L, Wu J, Song B, Xu Y, Sun S (2016) Rapidly progressive neurological deterioration in anti-AMPA receptor encephalitis with additional CRMP5 antibodies. Neurol Sci 37(11):1853–1855. https://doi.org/10.1007/s10072-016-2680-0

    Article  CAS  PubMed  Google Scholar 

  29. Shin Y-W, Lee S-T, Shin J-W, Moon J, Lim J-A, Byun J-I, Kim T-J, Lee K-J, Kim Y-S, Park K-I, Jung K-H, Lee SK, Chu K (2013) VGKC-complex/LGI1-antibody encephalitis: clinical manifestations and response to immunotherapy. J Neuroimmunol 265(1–2):75–81. https://doi.org/10.1016/j.jneuroim.2013.10.005

    Article  CAS  PubMed  Google Scholar 

  30. Wang M, Cao X, Liu Q, Ma W, Guo X, Liu X (2017) Clinical features of limbic encephalitis with LGI1 antibody. Neuropsychiatr Dis Treat 13:1589–1596. https://doi.org/10.2147/ndt.s136723

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Sai Y, Zhang X, Feng M, Tang J, Liao H, Tan L (2018) Clinical diagnosis and treatment of pediatric anti-N-methyl-D-aspartate receptor encephalitis: a single center retrospective study. Exp Ther Med 16(2):1442–1448. https://doi.org/10.3892/etm.2018.6329

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Funding

This work was supported by Liaoning Provincial Key R&D Program Guidance Plan (grant no. 2018225091).

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Authors and Affiliations

  1. Department of Neurology, Shengjing Hospital, China Medical University, 36 Sanhao Street, Heping District, Shenyang, Liaoning, 110004, People’s Republic of China

    Xiaoyu Dong, Dongming Zheng & Jianfei Nao

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  1. Xiaoyu Dong
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Correspondence to Jianfei Nao.

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Dong, X., Zheng, D. & Nao, J. Clinical characteristics and factors associated with short-term prognosis in adult patients with autoimmune encephalitis of non-neoplastic etiology. Neurol Sci 40, 1567–1575 (2019). https://doi.org/10.1007/s10072-019-03883-7

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