[PS18] Parallel Session 18: Encephalopathy

[PS18-3B-K] Recent Advances in the Research of Acute Encephalopathy

^○Masashi MIZUGUCHI (Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Japan)

Acute encephalopathy (AE) is a generic term for conditions presenting with acute clinical course with severe impairment of consciousness and widespread, non-inflammatory brain edema. By around the year 2007, Japanese investigators have classified AE into various distinct syndromes, such as acute necrotizing encephalopathy (ANE), acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), mild encephalitis/encephalopathy with reversible splenial lesion (MERS) and acute encephalitis with refractory, repetitive partial seizures (AERRPS/FIRES) In 2010, a nationwide epidemiological study elucidated the incidence and prognosis of these syndromes, as well as the relationship between viruses and syndromes. Since the year 2009, a collaborative study supported by the Japanese Society of Child Neurology (JSCN) has identified multiple risk gene variants for these syndromes, including mutation and polymorphism of genes regulating mitochondrial metabolism (enzymes), natural immunity (cytokines and HLA) and neural excitation (ion channels and neuromodulators). In 2016, JSCN published a guideline on the diagnosis and treatment of AE in children.