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Validation of a risk score to differentiate autoimmune and viral encephalitis: a Nationwide Cohort Study in Denmark

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Abstract

Background

A score to differentiate autoimmune (AE) and viral encephalitis (VE) early upon admission has recently been developed but needed external validation. The objective of this study was to evaluate the performance of the score in a larger and more diagnostically diverse patient cohort.

Methods

We conducted a retrospective nationwide and population-based cohort study including all adults with encephalitis of definite viral (2015–2022) or autoimmune aetiology (2009–2022) in Denmark. Variables included in the score-model were extracted from patient records and individual risk scores were assessed. The performance of the score was assessed by receiver-operating characteristics (ROC) curve analyses and calculation of the area under the curve (AUC).

Results

A total of 496 patients with encephalitis [AE n = 90, VE n = 287 and presumed infectious encephalitis (PIE) n = 119] were included in the study. The score was highly accurate in predicting cases of AE reaching an AUC of 0.94 (95% CI 0.92–0.97). Having a score ≥ 3 predicted AE with a PPV of 87% and an NPV of 91%. The risk score was found to perform well across aetiological subgroups and applied to the PIE cohort resulted in an AUC of 0.88 (95% CI 0.84–0.93).

Conclusion

The excellent performance of the score as reported in the development study was confirmed in this significantly larger and more diverse cohort of patients with encephalitis in Denmark. These results should prompt further prospective testing with wider inclusion criteria.
Title
Validation of a risk score to differentiate autoimmune and viral encephalitis: a Nationwide Cohort Study in Denmark
Authors
Lasse Fjordside
Mette Scheller Nissen
Anna Maria Florescu
Merete Storgaard
Lykke Larsen
Lothar Wiese
Hans Rudolf von Lüttichau
Micha Phill Grønholm Jepsen
Birgitte Rønde Hansen
Christian Østergaard Andersen
Jacob Bodilsen
Henrik Nielsen
Morten Blaabjerg
Anne-Mette Lebech
Helene Mens
Publication date
18-05-2024
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 8/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-024-12392-3
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