Clinical characteristics and factors associated with short-term prognosis in adult patients with autoimmune encephalitis of non-neoplastic etiology
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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.
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.
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.
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.
KeywordsAutoimmune encephalitis Magnetic resonance imaging Conscious disturbance
This work was supported by Liaoning Provincial Key R&D Program Guidance Plan (grant no. 2018225091).
Compliance with ethical standards
Conflict of interest
The authors declare that there is no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
- 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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 CrossRefGoogle 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
- 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 CrossRefGoogle Scholar