Autoimmune Encephalitis and Epilepsy: Evolving Definition and Clinical Spectrum

Advances in autoimmune encephalitis studies in the past 10 years have headed to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neural-specific autoantibodies targeting both intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are very dependent on antibody testing and response to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve children with multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than in adults, and the incorporation of clinical, immunological, electrophysiological and neuroradiological results is critical for a diagnostic approach. In this review, we document the most appropriate data both clinical and immunologic characteristics of the autoimmune encephalitis known so far, with the goals of assisting clinicians in the differential diagnosis and providing a promptly effective treatment.