Department of Neurology, Seoul National University Hospital, Seoul, South Korea.
Department of Neurology, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul, South Korea.
Ann Neurol. 2019 Mar;85(3):352-358. doi: 10.1002/ana.25421. Epub 2019 Feb 10.
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.
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).
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).
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.
目前尚无用于评估自身免疫性脑炎（AE）严重程度的量表。本研究旨在开发一种用于评估不同 AE 综合征患者严重程度的新型量表，并验证所开发量表的可靠性和有效性。
专家组提出关键项目，并根据内容效度比进行选择。初步将开发的量表应用于 50 例 AE 患者（开发队列），以评估其可接受性、可重复性、内部一致性和结构有效性。然后，该量表应用于另一独立队列（验证队列，n=38）。
开发了一个包含 9 个项目的新量表（发作、记忆功能障碍、精神症状、意识、语言问题、运动障碍/肌张力障碍、步态不稳和共济失调、脑干功能障碍和无力）。每个项目的赋值为 0-3 分，总分可达 0-27 分。我们将该量表命名为自身免疫性脑炎临床评估量表（CASE）。新量表的总评分具有极好的观察者间（组内相关系数 [ICC] = 0.97）和观察者内（ICC = 0.96）可靠性，与改良 Rankin 量表高度相关（r = 0.86，p < 0.001），内部一致性可接受（Cronbach α = 0.88）。此外，在验证队列中，该量表具有很高的观察者间可靠性（ICC = 0.99）和内部一致性（Cronbach α = 0.92）。
CASE 是一种用于 AE 的新型临床量表，具有较高的临床计量学特性。它适用于临床实践，并可能有助于克服当前 AE 结局量表的局限性。
