Abstract

Background: Diagnosis of autoimmune encephalitis (AE) is complicated by issues with sensitivity/specificity of antibody testing, non-specific MRI/EEG/CSF findings, and competing differential diagnoses. We explored practice differences in AE diagnosis and management. Methods: We utilized a worldwide electronic survey with practice-related demographic questions, and clinical questions about 2 cases: (1) a 20-year-old woman with a neuropsychiatric presentation strongly suspicious of AE, (2) a 40-year-old man with new temporal lobe seizures and cognitive impairment. Responses among different groups were compared using multi-variable logistic regression. Results: We received 1,333 responses from 94 countries; 12.0% identified as neuro-immunologists. Case 1: Those treating >5 AE cases/year were more likely to send antibodies in both serum and CSF (aOR vs 0/year: 3.29,95%CI 1.31-8.28,p=0.011), pursue empiric immunotherapy (aOR:2.42,1.33-4.40,p=0.004), and continue immunotherapy despite no response and negative antibodies at 2-weeks (aOR:1.65,1.02-2.69,p=0.043). Case 2: Neuro-immunologists were more likely to send antibodies in both serum and CSF (aOR:1.80,1.12-2.90,p=0.015). Those seeing >5 AE cases/year (aOR:1.86,1.22-2.86,p=0.004) were more likely to start immunotherapy without waiting for antibody results. Conclusions: Our findings highlight the heterogenous management of AE. Neuroimmunologists and those treating more AE cases generally take a more proactive approach to testing and immunotherapy than peers. Results emphasize the need for higher-quality treatment/outcome data and evidence-based guidelines.

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