LAUSR

Autoimmune encephalitis/encephalopathy (AE)-related conditions, such as paraneoplastic syndrome or Hashimoto’s encephalopathy, are not rare diseases. These AE are often treatable with conventional immunosuppressive therapy, but this requires detailed diagnosis of the disease entity. AE sometimes shows a slowly progressive disease course that is not shown by abnormal blood tests, or even brain magnetic resonance images. Changes in personal characteristics or psychotic signs often appear as the main symptoms, which make a diagnosis of AE difficult. Unfortunately, some AE patients have been incorrectly diagnosed with psychosis or pathomimesis because of inadequate physical examination or immunological testing. Over the past 10 years, various autoantibodies have been identified in the sera of AE patients that can be used as diagnostic markers (Table 1). However, the types of antibody that can be measured are limited, and the sensitivity and specificity of tests can be insufficient. It might take several weeks to several months for test results to be obtained. An important paper published in 2016 by Graus et al. addressed some issues in the clinical approach to the diagnosis of AE. Autoantibody test results and response to immunotherapy are not available at disease onset; therefore, Graus et al. provided guidelines (Table 2) to navigate through the differential diagnosis. This logical differential diagnosis can lead to prompt immunotherapy for AE. In this focused review series, three distinguished authors provide comprehensive review articles. Dr Akio Kimura from Gifu University reviews new findings on the pathomechanisms of autoimmune glial fibrillary acidic protein astrocytopathy, which is characterized by meningoencephalitis with