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Clin Exp Pediatr > Accepted Articles
DOI: https://doi.org/10.3345/kjp.2019.00598    [Accepted]
Published online August 16, 2019.
Autoimmune Encephalitis and Epilepsy: Evolving Definition and Clinical Spectrum
Joo Hee Seo1  , Yun-Jin Lee1,2, Ki Hyeong Lee1, Elakkat Gireesh1, Holly Skinner1, Michael Westerveld1
1Comprehensive Epilepsy Center, AdventHealth for Children, Orlando, Florida, USA
2Department of Pediatrics, Pusan National University Children’s Hospital, Pusan National University College of Medicine, Yangsan, Korea of Republic
Correspondence: 
Joo Hee Seo, Tel: +1-407-303-8127, Fax: +1-407-303-8197, Email: joohee.seo.md@adventhealth.com
Received: 30 May 2019   • Revised: 23 July 2019   • Accepted: 13 August 2019
Abstract
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.
Key Words: Autoimmune encephalitis, Autoimmune epilepsy, Anti-neuronal antibodies, Immunosuppressive therapy, Pediatric epilepsy




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