Hacohen, Yael; Ciccarelli, Olga; Kunchok, Amy C; (2025) Diagnostic Criteria for Autoimmune Encephalitis in Children: Challenges and Need for Clinical Expertise. Neurology , 105 (4) , Article e213946. 10.1212/WNL.000000000021394.

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Abstract

Autoimmune encephalitis (AE) affects all age groups, with varying prevalence of neural antibodies. Despite broad testing, some children remain seronegative. Consensus-based criteria published in 2016¹ classify patients into definite, probable, and possible AE. Definite AE requires well-characterized neural autoantibodies with clinical and paraclinical evidence. Possible AE is diagnosed based on subacute onset of cognitive or psychiatric symptoms plus at least 1 supportive feature (e.g., seizures, MRI changes, and CSF pleocytosis) and exclusion of alternative causes. Probable antibody-negative AE (ANAE) can be diagnosed in seronegative patients with >2 supporting features of autoimmunity (MRI, CSF, biopsy). Pediatric-specific criteria² published later reduced the number of required supporting features and classified limbic encephalitis, acute disseminated encephalomyelitis (ADEM), and Bickerstaff brainstem encephalitis as probable ANAE.

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