Recent progress in autoimmune encephalitis and its related disorders

Abstract

After the discovery of a series of autoantibodies against neuronal cell surface antigens (NSAs) of the CNS in the past 10 years, the concept of encephalitis has changed dramatically. Accordingly, a practical, syndrome-based diagnostic approach to autoimmune encephalitis was proposed in 2016. These autoantibodies have also been identified in a subset of overlapping encephalitis and demyelinating syndrome, epilepsy, first episode psychosis, movement disorders, post-herpes simplex encephalitis, progressive dementia, postpartum psychosis, stiff-person spectrum disorders, or non-REM/REM sleep behavior disorder. Although not all neuronal antibody tests are available in Japan, we have entered a new era that we have to make a correct diagnosis and start appropriate immunotherapy based on initial neurological assessment and conventional tests, without being constrained by conventional fixed ideas or normal-appearing brain MRIs while waiting for neuronal antibody test results. Although many issues need to be resolved in Japan in terms of diagnosis and treatment in autoimmune encephalitis, this review focusses on recent progress in autoimmune encephalitis and its related disorders closely related to clinical practice, including Hashimoto encephalopathy and new-onset refractory status epilepticus (NORSE).