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Reply to “Grading the severity of autoimmune encephalitis: advances and pitfalls”
Annals of Neurology ( IF 11.2 ) Pub Date : 2019-05-20 , DOI: 10.1002/ana.25497
Jung-Ah Lim 1, 2 , Soon-Tae Lee 1 , Kon Chu 1 , Sang Kun Lee 1
Affiliation  

Grading the Severity of Autoimmune Encephalitis: Advances and Pitfalls Gregorio Spagni, MD, and Raffaele Iorio, MD, PhD 1,2 We read with great interest the study by Lim et al, which aimed to develop an easily applicable scale to grade the severity of autoimmune encephalitis (AE). Nine key clinical features were identified and included in the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). CASE showed good interobserver and intraobserver reliability, and internal consistency, as well as a high correlation with the modified Rankin Scale (mRS). We applied CASE retrospectively to a cohort of 60 patients with AE, admitted to our institution between 2012 and 2018, and prospectively in 3 additional patients. Median age at onset was 55 years (range = 3 months–88 years), in 13 of 63 (21%) onset was before 18 years of age, and 34 of 63 (54%) were female. All patients fulfilled the clinical diagnostic criteria for AE: definite AE was diagnosed in 16 of 63 patients (25%), definite anti-NMDA receptor encephalitis in 11 of 63 (17%), definite autoimmune limbic encephalitis in 6 of 63 (10%), antibody-negative probable AE in 4 of 63 (6 %), possible AE in 25 of 63 (40%), and Hashimoto encephalopathy in 1 of 63 (2%). At the disease nadir, median mRS score was 4 (range = 3–5). We applied CASE at maximum clinical severity (median CASE score = 9, range = 3–24). In our cohort, CASE significantly correlated with mRS (p < 0.0001, r = 0.5025; Fig), although the correlation strength was weaker than that reported by Lim et al. However, we encountered several issues when CASE was applied to our patients that need to be clarified. Severe clinical conditions, such as coma and status epilepticus, make other items hardly assessable (eg, gait instability, language). In such cases, it is not specified what score should be given to items that are not directly assessable (we gave the maximum score). Postictal state after seizures also interferes with the evaluation of most items. The authors should clarify how to apply the scale in these situations and whether this score is meant to be used only in clinically stable patients. Furthermore, when applying CASE to a pediatric population, some items, such as memory and language, were not easily assessable. We think that the scale is more suitable for adult patients. Moreover, it would be appropriate to develop a different scale for patients with altered consciousness. CASE is potentially a useful tool and addresses the unmet need of clinical scales to grade AE severity, as mRS is quite coarse in evaluating this condition. However, CASE needs further improvement and validation to be employed in clinical trials.

中文翻译:

回复“自身免疫性脑炎严重程度分级:进展与陷阱”

对自身免疫性脑炎的严重程度进行分级:进展和陷阱 Gregorio Spagni, MD 和 Raffaele Iorio, MD, PhD 1,2 我们饶有兴趣地阅读了 Lim 等人的研究,该研究旨在开发一个易于应用的量表来对脑炎的严重程度进行分级自身免疫性脑炎 (AE)。九项关键临床特征被确定并包含在自身免疫性脑炎临床评估量表 (CASE) 中。CASE 显示出良好的观察者间和观察者内的可靠性和内部一致性,以及与改良的 Rankin 量表 (mRS) 的高度相关性。我们对 2012 年至 2018 年间入住我们机构的 60 名 AE 患者进行了回顾性分析,并对另外 3 名患者进行了前瞻性研究。中位发病年龄为 55 岁(范围 = 3 个月至 88 岁),63 人中有 13 人(21%)在 18 岁之前,63 人中有 34 人(54%)是女性。所有患者均符合 AE 的临床诊断标准:63 名患者中有 16 名 (25%) 确诊为 AE,63 名患者中有 11 名 (17%) 确诊为抗 NMDA 受体脑炎,63 名患者中有 6 名确诊为自身免疫性边缘系统脑炎 (10%) ),63 例中有 4 例 (6%) 出现抗体阴性的可能 AE,63 例中有 25 例可能出现 AE (40%),63 例中有 1 例出现桥本脑病 (2%)。在疾病最低点,中位 mRS 评分为 4(范围 = 3-5)。我们在最大临床严重程度下应用 CASE(中位 CASE 评分 = 9,范围 = 3-24)。在我们的队列中,CASE 与 mRS 显着相关(p < 0.0001,r = 0.5025;图),尽管相关强度弱于 Lim 等人报道的强度。然而,当 CASE 应用于我们的患者时,我们遇到了几个需要澄清的问题。严重的临床情况,例如昏迷和癫痫持续状态,使其他项目难以评估(例如,步态不稳、语言)。在这种情况下,对于不能直接评估的项目应该给予什么分数(我们给出最高分)并没有具体说明。癫痫发作后的发作后状态也会干扰大多数项目的评估。作者应该阐明如何在这些情况下应用该量表,以及该评分是否仅用于临床稳定的患者。此外,当将 CASE 应用于儿科人群时,一些项目,如记忆和语言,不容易评估。我们认为该量表更适合成年患者。此外,为意识改变的患者制定不同的量表是合适的。CASE 可能是一个有用的工具,解决了临床量表对 AE 严重程度分级的未满足需求,因为 mRS 在评估这种情况时非常粗略。然而,CASE 需要进一步改进和验证才能用于临床试验。
更新日期:2019-05-20
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