Clin Exp Pediatr.  2020 Aug;63(8):291-300. 10.3345/kjp.2019.00598.

Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum

Affiliations
  • 1Comprehensive Epilepsy Center, AdventHealth for Children, Orlando, FL, USA
  • 2Department of Pediatrics, Pusan National University Children’s Hospital, Pusan National University College of Medicine, Yangsan, Korea

Abstract

Advances in autoimmune encephalitis studies in the past 10 years have led 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 neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are quite dependent on antibody testing and responses to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve the multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than that in adults, and the incorporation of clinical, immunological, electrophysiological, and neuroradiological results is critical to the diagnostic approach. In this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

Keyword

Autoimmune encephalitis; Autoimmune epilepsy; Antineuronal antibodies; Immunosuppression therapy; Epilepsy in children
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