ObjectiveTo explore practice differences in the diagnosis and management of autoimmune encephalitis (AE), which is complicated by issues with sensitivity/specificity of antibody testing, nonspecific MRI/EEG/ CSF findings, and competing differential diagnoses.
MethodsWe used a worldwide electronic survey with practice-related demographic questions and clinical questions about 2 cases: (1) a 20year-old woman with a neuropsychiatric presentation strongly suspicious of AE and (2) a 40-year-old man with new temporal lobe seizures and cognitive impairment. Responses among different groups were compared using multivariable logistic regression.
ResultsWe received 1,333 responses from 94 countries; 12.0% identified as neuroimmunologists. Case 1: those treating >5 AE cases per year were more likely to send antibodies in both serum and CSF (adjusted odds ratio [aOR] vs 0 per year: 3.29, 95% CI 1.31-8.28, p = 0.011), pursue empiric immunotherapy (aOR: 2.42, 95% CI 1.33-4.40, p = 0.004), and continue immunotherapy despite no response and negative antibodies at 2 weeks (aOR: 1.65, 95% CI 1.02-2.69, p = 0.043). Case 2: neuroimmunologists were more likely to send antibodies in both serum and CSF (aOR: 1.80, 95% CI 1.12-2.90, p = 0.015). Those seeing >5 AE cases per year (aOR: 1.86, 95% CI 1.22-2.86, p = 0.004) were more likely to start immunotherapy without waiting for antibody results.
ConclusionsOur results highlight the heterogeneous management of AE. Neuroimmunologists and those treating more AE cases generally take a more proactive approach to testing and immunotherapy than peers. Results highlight the need for higher-quality cohorts and trials to guide empiric immunotherapy, and evidence-based guidelines aimed at both experts and nonexperts. Because the average AE patient is unlikely to be first seen by a neuroimmunologist, ensuring greater uniformity in our approach to suspected cases is essential to ensure that patients are appropriately managed.Autoimmune encephalitis (AE) is a type of noninfectious neuroinflammation that is an increasingly recognized cause of acute or subacute progressive alteration in mental status with various presentations. Some cases are associated with specific autoantibodies to cell surface molecules and intracellular targets. 1 However, antibody testing is not always available at many institutions, and results are of variable sensitivity and specificity, depending in part on the type of MORE ONLINE Infographic Npub.org/NCP/pc06research Explore this topic Npub.org/NCP/pc6 Interactive world map NPub.org/NCP/map08 More Practice Current NPub.org/NCP/ practicecurrent