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Ann Neurol. 2019 Mar;85(3):352-358. doi: 10.1002/ana.25421. Epub 2019 Feb 10.

Development of the clinical assessment scale in autoimmune encephalitis.

Author information

1
Department of Neurology, Seoul National University Hospital, Seoul, South Korea.
2
Department of Neurology, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul, South Korea.
3
Department of Neurosurgery, 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 Center, 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, Bucheon, 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, South Korea.
18
Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Ulsan, 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, South Korea.
21
Department of Neurology, Seoul National University Hospital Healthcare System Gangnam Center, Seoul, 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 (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 < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 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. ANN NEUROL 2019;85:352-358.

PMID:
30675918
DOI:
10.1002/ana.25421

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