An evolving redefinition of autoimmune encephalitis

Abstract

Autoimmune encephalitis encompasses a wide variety of protean pathologic processes associated with the presence of antibodies against neuronal intracellular proteins, synaptic receptors, ion channels and/or neuronal surface proteins. This type of encephalitis can also involve children with complex patterns of seizures and unexpected behavioural changes, which jeopardize their prompt recognition and treatment. Many epidemiological studies have shown that numerous immune-based forms of encephalitis can be encountered, almost surpassing the rate of postinfectious encephalitides. However, the overall exact prevalence of autoimmune encephalopathies remains underestimated, and the definition of diagnostic algorithms results muddled. The spectrum of neuropsychiatric manifestations in the pediatric population with autoimmune encephalitis is less clear than in adults, but the integration of clinical, immunological, electrophysiological and neuroradiological data is essential for a general approach to patients. In this review we report the most relevant data about both immunologic and clinical characteristics of the main autoimmune encephalitides recognized so far, with the aim of assisting clinicians in the differential diagnosis and favouring an early effective treatment. Correlations between phenotype and autoantibodies involved in the neurological damage of autoimmune encephalitis are largely unknown in the first years of life, because of the relatively small number of pediatric patients adequately studied. Future multicenter collaborative studies are needed to improve the diagnostic approach and tailor personalized therapies in the long-term.

Keywords: Autoimmune encephalitis; Autoimmune epilepsy; Child; Immunosuppressive therapy; Innovative biotechnologies; Personalized medicine.