한빛사 논문
Jung-Ah Lim,1,2,* Soon-Tae Lee,1,* Jangsup Moon,1,3 Jin-Sun Jun,1,4 Tae-Joon Kim,1,5 Yong-Won Shin,1 Suhailah Abdullah,6 Jung-Ick Byun,1,7 Jun-Sang Sunwoo,1,8 Keun Tae Kim,9 Tae-Won Yang,10 Woo-Jin Lee,1 Hye-Jin Moon,11 Dong Wook Kim,12 Byung Chan Lim,13 Yong Won Cho,9 Tae-Ho Yang,14 Hee Jin Kim,15,16 Young-Soo Kim,17 Yong Seo Koo,18 Byeongsu Park,19 Keun-Hwa Jung,1 Manho Kim,1,20 Kyung-Il Park,1,21 Ki-Young Jung,1 Kon Chu,1 and Sang Kun Lee1
1 Department of Neurology, Seoul National University Hospital, Seoul, South Korea
2 Department of Neurology, Gangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul, South Korea
3 Department of Neurolosurgery, Seoul National University Hospital, Seoul, South Korea
4 Department of Neurology, School of Medicine, Kyungpook National University, Kyungpook National University Chilgok Hospital, Daegu, South Korea
5 Department of Neurology, Ajou University School of Medicine, Ajou University Medical Center, Suwon, South Korea.
6 Division of Neurology, Department of Medicine, University Malaya Medical Centre, Kuala Lumpur, Malaysia
7 Department of Neurology, Kyung Hee University Hospital at Gangdong, Seoul, South Korea
8 Department of Neurology, Soonchunhyang University School of Medicine, Seoul, South Korea
9 Department of Neurology, Keimyung University Dongsan Medical Center, School of Medicine, Daegu, South Korea
10 Department of Neurology, Gyeongsang National University Changwon Hospital, Gyeongsang National University School of Medicine, Changwon, South Korea
11 Department of Neurology, Soonchunhyang University Bucheon Hospital, South Korea
12 Department of Neurology, Konkuk University School of Medicine, Seoul, South Korea
13 Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea
14 Department of Neurology, Chonbuk National University Hospital, Jeonju, South Korea
15 Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
16 Neuroscience Center, Samsung Medical Center, Seoul, South Korea
17 Department of Neurology and Institute of Health Science, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Seoul Korea
18 Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, South Korea
19 Department of Neurology, Ulsan University Hospital, Ulsan University College of Medicine, Ulsan, South Korea
20 Center for Neuroscience and protein metabolism, Seoul National University College of Medicine, Seoul, Korea21Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Seoul, South Korea
*J -A.L. and S-T. L. share first authorship.
Correspondence to:Sang Kun Lee, MD, PhD, Department of Neurology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea. Kon Chu, MD, PhD, Department of Neurology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.
Abstract
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 (ICC = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with mRS (r = 0.86, p<0.001), and had acceptable internal consistency (Cronbach's alpha = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach's alpha = 0.92).
Interpretation
CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE.
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