• 1 Department of Neurology, Seoul National University Hospital, Seoul, South Korea
  • 2 Division of Neurology, Department of Medicine, University Malaya Medical Center, Kuala Lumpur, Malaysia
  • 3 Department of Neurology, Keimyung University Dongsan Medical Center, School of Medicine, Daegu, South Korea
  • 4 Department of Neurology, Gyeongsang National University Changwon Hospital, Gyeongsang National University School of Medicine, Changwon, South Korea
  • 5 Department of Neurology, Soonchunhyang University Bucheon Hospital, Bucheon, South Korea
  • 6 Department of Neurology, Konkuk University School of Medicine, Seoul, South Korea
  • 7 Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea
  • 8 Department of Neurology, Chonbuk National University Hospital, Jeonju, South Korea
  • 9 Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
  • 10 Department of Neurology and Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Seoul, South Korea
  • 11 Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Ulsan, South Korea
  • 12 Department of Neurology, Ulsan University Hospital, Ulsan University College of Medicine, Ulsan, South Korea
Ann Neurol, 2019 03;85(3):352-358.
PMID: 30675918 DOI: 10.1002/ana.25421


OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale.

METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38).

RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.