Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Observational Study
. 2023 Dec 12:14:1213532.
doi: 10.3389/fimmu.2023.1213532. eCollection 2023.

Clinical characteristics and prognosis in patients with neuronal surface antibody-mediated autoimmune encephalitis: a single-center cohort study in China

Teng Huang  1   2 , Fei Liu  2 , Baojie Wang  2 , Chunjuan Wang  3 , Maolin Hao  2 , Shougang Guo  1   3
Affiliations
Observational Study

Clinical characteristics and prognosis in patients with neuronal surface antibody-mediated autoimmune encephalitis: a single-center cohort study in China

Teng Huang et al. Front Immunol. .

Abstract

Objective: This retrospective observational study primarily aimed to analyse the clinical characteristics of patients with neuronal surface antibody-mediated autoimmune encephalitis (AE) in China and report their prognosis after immunotherapy.

Methods: Clinical characteristics, laboratory or imaging examinations, and treatment outcomes of 103 patients diagnosed with AE between 1 September 2014 and 31 December 2020 were collected. Univariate and multivariate logistic regression analyses were performed to determine the predictors of poor prognosis.

Results: Overall, 103 patients were enrolled in the study. The main clinical symptoms included seizures (74.8%), psychiatric and behavior disorders (66.0%), cognitive deficits (51.5%), disturbances of consciousness (45.6%), and movement disorders/involuntary movements (26.2%). The distribution of clinical syndromes also differed for different AE subtypes. The efficacy rates of first-line immunotherapy for anti-NMDAR, anti-LGI1, anti-GABABR, and anti-CASPR2 encephalitis were 70.2%, 92.3%, 70%, and 83.3%, respectively, and rituximab was administered to 21 patients as second-line immunotherapy, including 14 patients with anti-NMDAR encephalitis, 4 with anti-LGI1 encephalitis, 2 with anti-GABABR encephalitis, and 1 with anti-CASPR2 encephalitis. Five patients with poor effect of the second-line treatment received bortezomib. According to the results of the last follow-up, 78 patients had a good prognosis (mRS 0-2), and 21 patients had a poor prognosis (mRS 3-6). The proportion of patients with a poor prognosis was significantly higher in anti-GABABR encephalitis compared to the other AE subtypes (p<0.001). Multivariate analysis indicated that elevated neutrophil-to-lymphocyte ratio (NLR) and tumour presence were independent risk factors for poor prognosis. The regression equation of the model was logit(P)=-3.480 + 0.318 NLR+2.434 with or without tumour (with assignment =1, without assignment =0). The prediction probability generated by the regression model equation was used as the independent variable for receiver operating curve (ROC) analysis. The results showed that the area under the curve (AUC) of the prediction probability was 0.847 (95% CI, 0.733-0.961; p < 0.001).

Conclusions: Different AE subtypes demonstrated different clinical symptom spectra throughout the disease stage. Anti-LGI1 encephalitis and anti-CASPR2 encephalitis were more sensitive to first-line and second-line treatments. Anti-GABABR encephalitis had the worst prognosis among the abovementioned subtypes. The regression equation constructed using NLR and tumour presence effectively predicted the poor prognosis.

Keywords: autoimmune encephalitis; clinical characteristics; immunotherapy; neutrophil-to-lymphocyte ratio; prognosis.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Symptom distribution of different antibody types.
Figure 2
Figure 2
MRI features of different antibody type.
Figure 3
Figure 3
Immunotherapy of different antibody type. (A) Proportion of first-line immunotherapy and efficacy rate; (B) Proportion of second-line immunotherapy and efficacy rate.
Figure 4
Figure 4
Distribution of mRS scores upon admission and the last follow-up.
Figure 5
Figure 5
Receiver operating curve of the predictive value of the regression model equation for poor prognosis of autoimmune encephalitis.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Dalmau J, Graus F. Antibody-mediated encephalitis. N Engl J Med (2018) 378:840–51. doi: 10.1056/NEJMra1708712 - DOI - PubMed
    1. Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms, and hypoventilation in ovarian teratoma. Ann Neurol (2005) 58:594–604. doi: 10.1002/ana.20614 - DOI - PMC - PubMed
    1. 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) 61:25–36. doi: 10.1002/ana.21050 - DOI - PMC - PubMed
    1. Graus F, Dalmau J. CNS autoimmunity: New findings and pending issues. Lancet Neurol (2012) 11:17–9. doi: 10.1016/S1474-4422(11)70280-0 - DOI - PMC - PubMed
    1. Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. . A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol (2016) 15:391–404. doi: 10.1016/S1474-4422(15)00401-9 - DOI - PMC - PubMed

Publication types

LinkOut - more resources