A score that predicts 1-year functional status in patients with anti-NMDA receptor encephalitis

Ramani Balu, Lindsey McCracken, Eric Lancaster, Francesc Graus, Josep Dalmau, Maarten J Titulaer, Ramani Balu, Lindsey McCracken, Eric Lancaster, Francesc Graus, Josep Dalmau, Maarten J Titulaer

Abstract

Objective: To construct a grading score that predicts neurologic function 1 year after diagnosis of anti-NMDA receptor (NMDAR) encephalitis.

Methods: Three hundred eighty-two patients with detailed information and functional status at 1 year were studied. Factors associated with poor status (defined as modified Rankin Scale score ≥3) were identified and incorporated into a multivariate logistic regression model. This model was used to develop a 5-point prediction score, termed the anti-NMDAR Encephalitis One-Year Functional Status (NEOS) score.

Results: Intensive care unit admission (p < 0.001), treatment delay >4 weeks (p = 0.012), lack of clinical improvement within 4 weeks (p < 0.001), movement disorder (p = 0.001), central hypoventilation (p < 0.001), elevated CSF white blood cell count (p < 0.001), elevated CSF protein level (p = 0.027), and abnormal MRI (p = 0.002) were associated with 1-year functional status in univariate analysis. Intensive care unit admission, treatment delay >4 weeks, lack of clinical improvement within 4 weeks, abnormal MRI, and CSF white blood cell count >20 cells/μL were independent predictors for outcome in multivariate regression modeling. These 5 variables were assigned 1 point each to create the NEOS score. NEOS score strongly associated with the probability of poor functional status at 1 year (3% for 0 or 1 point to 69% for 4 or 5 points, p < 0.001).

Conclusions: The NEOS score accurately predicts 1-year functional status in patients with anti-NMDAR encephalitis. This score could help estimate the clinical course following diagnosis and may aid in identifying patients who could benefit from novel therapies.

Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

Figures

Figure 1. NEOS score predicts 1-year functional…
Figure 1. NEOS score predicts 1-year functional status
(A) Graph shows the probability of good functional status at 1 year vs NEOS score for all patients in the analyzed cohort. Increasing NEOS score is strongly associated with lower probability of good functional status at 1 year (p < 0.001, Cuzick nonparametric test for trend). Error bars represent 95% confidence intervals for each measurement. (B) Distribution of mRS values across NEOS score values. There is a progressive shift toward higher 1-year mRS values with increasing NEOS score. mRS = modified Rankin Scale; NEOS = anti-NMDAR Encephalitis One-Year Functional Status.
Figure 2. Impact of first- and second-line…
Figure 2. Impact of first- and second-line therapies on NEOS score
(A) Distribution of NEOS scores in patients who received only first-line therapies and in patients who received second-line therapies. There is a shift toward higher NEOS scores in patients who received second-line therapies. (B) Relationship between NEOS score and probability of good functional status at 1 year is similar in patients who received only first-line therapies compared to those who received second-line therapies. NEOS = anti-NMDAR Encephalitis One-Year Functional Status.
Figure 3. Distribution of NEOS score and…
Figure 3. Distribution of NEOS score and relationship to outcome over time
(A) Distribution of NEOS scores stratified by year of symptom onset. Over time, there is a shift toward lower NEOS score values in patients with more recent symptom onset. (B) Relationship between NEOS score and probability of good functional status at 1 year is similar across different groups. NEOS = anti-NMDAR Encephalitis One-Year Functional Status.

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