• Josep Dalmau and Francesc Graus.
• Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Neurology Department, Institute of Neuroscience, Hospital Clínic de Barcelona, University of Barcelona, Barcelona, Spain; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA; Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain. Electronic address: jdalmau@clinic.cat.
• Lancet Neurol. 2023 Jun 1; 22 (6): 529540529-540.

AbstractIncreased awareness of autoimmune encephalitis has led to two unintended consequences: a high frequency of misdiagnoses and the inappropriate use of diagnostic criteria for antibody-negative disease. Misdiagnoses typically occur for three reasons: first, non-adherence to reported clinical requirements for considering a disorder as possible autoimmune encephalitis; second, inadequate assessment of inflammatory changes in brain MRI and CSF; and third, absent or limited use of brain tissue assays along with use of cell-based assays that include only a narrow range of antigens. For diagnosis of possible autoimmune encephalitis and probable antibody-negative autoimmune encephalitis, clinicians should adhere to published criteria for adults and children, focusing particularly on exclusion of alternative disorders. Moreover, for diagnosis of probable antibody-negative autoimmune encephalitis, the absence of neural antibodies in CSF and serum should be well substantiated. Neural antibody testing should use tissue assays along with cell-based assays that include a broad range of antigens. Live neuronal studies in specialised centres can assist in resolving inconsistencies with respect to syndrome-antibody associations. Accurate diagnosis of probable antibody-negative autoimmune encephalitis will identify patients with similar syndromes and biomarkers, which will provide homogeneous populations for future assessments of treatment response and outcome.Copyright © 2023 Elsevier Ltd. All rights reserved.

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