Jordan Benderoth1, Silvia Leblanc2, Roba El Zibaoui1, Sophia Zhao1, Ralph Habis3, Rajesh Gupta4, Arun Venkatesan5, Rodrigo Hasbun4, John Probasco6
1Johns Hopkins University School of Medicine, 2McGovern Medical School at UTHealth Houston, 3Johns Hopkins School of Medicine, 4UTHealth, 5Johns Hopkins Hospital, 6The Johns Hopkins Hospital
Objective:
Compare paraneoplastic encephalitis (PE) patients to non-paraneoplastic autoimmune encephalitis (AE) patients to identify distinguishing features of PE at clinical presentation.
Background:
PE is an immune-mediated syndrome that is a remote consequence of a systemic immune response to cancer and can even occur years before cancer detection. The PNS-Care Score integrates phenotype, antibody risk, and cancer association to classify cases as definite, probable, or possible paraneoplastic cases. At presentation, overlap between PE and AE may delay cancer diagnosis.
Design/Methods:
This retrospective multicenter study included 647 encephalitis patients across two Johns Hopkins (2006–2022) and two UTHealth institutions (2005–2023). The retrospective design enabled assessment of cancer incidence after presentation. Patients meeting PNS-Care Score criteria for probable PE were compared by demographic and clinical features to patients with AE using non-parametric tests. Variables that were statistically significant in univariate analyses were included in a multivariate logistic regression analysis.
Results:
Compared with AE patients (N=95), PE patients (N=30) tended to be younger (13.3% vs 30.5% >60 years; p =0.06), and were more likely to present with acute onset (<5 days, 93.1% vs 68.8%; p =0.009) and fever (46.4% vs 25.3%; p =0.04), but were less likely to have abnormal movements (33.3% vs 61.4%; p =0.03). Moreover, PE patients had stronger inflammatory activity with higher CSF leukocyte counts (median 19 [5–39] vs 8 [2–25] cells/mm³; p =0.04). In multivariate analysis, pleocytosis (>5 cells/mm³) (OR 3.3, 95% CI 1.2–9.2, p =0.023) and acute onset (OR 8.6, 95% CI 1.8–33.3, p =0.007) independently predicted PE, while fever was positively associated with PE but did not reach statistical significance (OR 2.3, 95% CI 0.9–6.1, p =0.09).
Conclusions:
Early inflammatory markers, including CSF pleocytosis and rapid onset of encephalitis symptoms within five days, may help distinguish PE from AE. These results support the link between a tumor related immune response and CSF inflammation.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.