Highlights
- •Poor prognosis is associated with elevated WBC of the CSF in anti-NMDAR encephalitis.
- •Increased EEG slow waves and abnormal brain MRI may be risk factors of poor prognosis.
- •First-line immunotherapy less than 3 months after first onset may be a risk factor for recurrence.
Abstract
Background
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type
of autoimmune encephalitis. Here, we investigated the factors associated with poor
prognosis and relapse in patients with anti-NMDAR encephalitis.
Methods
In this single-center observational cohort study, we retrospectively analyzed 51 patients
with anti-NMDAR encephalitis treated in our hospital from January 2014 to October
2022. The demographic data, clinical characteristics, scale scores, results of auxiliary
examination, and treatment details were statistically analyzed. Based on modified
Rankin Scale (mRS) scores measured before final discharge, patients were divided into
groups with good (mRS score 0–2) and poor (mRS score 3–6) prognoses for functional
evaluation. The chi-squared test or Fisher's exact test was used to compare categorical
data, and the t-test and Mann–Whitney U test were used to compare normally and non-normally distributed
continuous data, respectively. Binary logistic regression was used to identify the
risk factors for prognosis and relapse.
Results
At admission, the main clinical manifestations observed were psychobehavioral disorders
(50 cases, 98.0%), consciousness disorders (28 cases, 54.9%), epilepsy (33 cases,
64.7%), motor disorders (28 cases, 54.9%), speech disorders (24 cases, 47.1%), and
dysfunction of the autonomic nervous system (15 cases, 29.4%). All 51 patients (100%)
had mRS scores of 3–5 at admission, and 50 were treated with intravenous methylprednisolone
and human immunoglobulin. A total of 22 patients (43.1%) had an mRS score of 3–6 at
discharge, which was significantly lower than those at admission. One patient died
(mRS score 6) after developing septic shock (fatality rate 1.9%). Binary logistic
regression analysis showed that movement disorders/involuntary movement (odds ratios
[OR] 3.778, p = 0.029), abnormal brain magnetic resonance imaging (OR 4.817, p = 0.013), electroencephalogram slow wave activity of >50% (OR 8.400, p = 0.001), a white blood cell count of >10 × 106/L in the cerebrospinal fluid (OR 3,210, p = 0.048), and male sex (OR 3.282, p = 0.050) were risk factors for poor prognosis. A duration of disease of >12 months
(OR 8.800, p = 0.001) and first-line-immunotherapy for less than 3 months after first onset (OR
3.719, p = 0.048) were identified as risk factors for relapse.
Conclusion
Motor disorders or involuntary movement, abnormal brain magnetic resonance imaging,
electroencephalogram slow wave activity >50%, and elevated white blood cell counts
in cerebrospinal fluid were associated with poor prognosis in patients with NMDAR
encephalitis. First-line immunotherapy less than 3 months after first onset may be
a risk factor for relapse.
Keywords
Abbreviations:
NMDAR (N-methyl-D-aspartate receptor), mRS (modified Rankin Scale), OR (odds ratios), MRI (magnetic resonance imaging), EEG (electroencephalogram), WBC (white blood cell), CSF (cerebrospinal fluid), IQR (interquartile range), ICU (intensive care unit), IVIG (intravenous human immunoglobulin)To read this article in full you will need to make a payment
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References
- Risk factors for mortality in patients with anti-NMDA receptor encephalitis.Acta Neurol. Scand. 2017; 136: 298-304https://doi.org/10.1111/ane.12723
- Pearls & oy-sters: relapse of anti-NMDA receptor encephalitis after prior first- and second-line immunotherapy.Neurology. 2018; 90: 936-939https://doi.org/10.1212/WNL.0000000000005517
- Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies.Lancet Neurol. 2008; 7: 1091-1098https://doi.org/10.1016/S1474-4422(08)70224-2
- Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis.Lancet Neurol. 2011; 10: 63-74https://doi.org/10.1016/S1474-4422(10)70253-2
- Analysis of relapses in anti-NMDAR encephalitis.Neurology. 2011; 77: 996-999https://doi.org/10.1212/WNL.0b013e31822cfc6b
- The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project.Clin. Infect. Dis. 2012; 54: 899-904https://doi.org/10.1093/cid/cir1038
- Long-term functional outcomes and relapse of anti-NMDA receptor encephalitis: a cohort study in Western China.Neurol. Neuroimmunol. Neuroinflamm. 2021; 8: e958https://doi.org/10.1212/NXI.0000000000000958
- A clinical approach to diagnosis of autoimmune encephalitis.Lancet Neurol. 2016; 15: 391-404https://doi.org/10.1016/S1474-4422(15)00401-9
- Epidemiology of antibody-positive autoimmune encephalitis in Southwest China: a multicenter study.Front. Immunol. 2019; 10: 2611https://doi.org/10.3389/fimmu.2019.02611
- N-methyl-D-aspartate antibody encephalitis: temporal progression of clinical and paraclinical observations in a predominantly non-paraneoplastic disorder of both sexes.Brain. 2010; 133: 1655-1667https://doi.org/10.1093/brain/awq113
- Validation of the NEOS score in Chinese patients with anti-NMDAR encephalitis.Neurol. Neuroimmunol. Neuroinflamm. 2020; 7: e860https://doi.org/10.1212/NXI.0000000000000860
- Clinical manifestations, treatment outcomes, and prognostic factors of pediatric anti-NMDAR encephalitis in tertiary care hospitals: a multicenter retrospective/prospective cohort study.Brain Dev. 2019; 41: 436-442https://doi.org/10.1016/j.braindev.2018.12.009
- Clinical characteristics, treatment and long-term prognosis in patients with anti-NMDAR encephalitis.Neurol. Sci. 2021; 42: 4683-4696https://doi.org/10.1007/s10072-021-05174-6
- Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study.Lancet Neurol. 2013; 12: 157-165https://doi.org/10.1016/S1474-4422(12)70310-1
- Anti-NMDA receptor encephalitis: clinical characteristics, predictors of outcome and the knowledge gap in southwest China.Eur. J. Neurol. 2016; 23: 621-629https://doi.org/10.1111/ene.12911
- Treatment and outcome of children and adolescents with N-methyl-D-aspartate receptor encephalitis.J. Neurol. 2015; 262: 1859-1866https://doi.org/10.1007/s00415-015-7781-9
- Analysis of electroencephalogram characteristics of anti-NMDA receptor encephalitis patients in China.Clin. Neurophysiol. 2017; 128: 1227-1233https://doi.org/10.1016/j.clinph.2017.04.015
Article info
Publication history
Published online: April 03, 2023
Accepted:
April 1,
2023
Received in revised form:
March 30,
2023
Received:
October 26,
2022
Identification
Copyright
© 2023 Elsevier B.V. All rights reserved.