Diagnosing autoimmune encephalitis in a real-world single-centre setting
Diagnosing autoimmune encephalitis in a real-world single-centre setting
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DOI：
10.1007/s00415-019-09607-3
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发表时间：
2020-02-01
影响因子：
6
通讯作者：
Martinelli, Vittorio
中科院分区：
文献类型：
--
作者：
Giordano, Antonino;Fazio, Raffaella;Martinelli, Vittorio
背景自身免疫性脑炎（AE）的早期识别和治疗对患者至关重要，但诊断通常困难且耗时。为此，格劳斯等人发表了一种基于综合征的诊断方法。（《柳叶刀·神经病学》15：391-404，2016），但在文献中很少有人知道它在临床实践中的应用。目的我们的目的是测试这种方法在现实世界的单中心设置的可行性，并分析最相关的因素，在标准fulfillment.Methods我们回顾性地应用这些标准，我们的队列患者出院诊断为自身免疫性脑炎结果所有受试者均符合可能AE（pAE）标准，EEG和MRI在诊断中起主要作用，而CSF主要用于排除其他疾病。3例患者符合可能的抗NMDA-R脑炎（pNMDA）标准。在4名检测到自身抗体的患者中诊断出明确的抗NMDAR脑炎，但令人惊讶的是，这些受试者均不符合pNMDA标准。18例患者被诊断为明确的边缘系统AE（15例抗体阳性，3例抗体阴性）。在抗体阴性边缘AE中需要MRI双侧受累限制诊断。一名患者符合标准可能抗体阴性AE，而10例患者仍然被归类为pAE.Conclusion从我们的回顾性分析，一些建议，一个更好的定义的标准可能会出现。对前瞻性队列进行更大规模的研究可能更有助于探讨可能的重要问题。
Background Early recognition and treatment of autoimmune encephalitis (AE) are crucial for patients, but diagnosis is often difficult and time-consuming. For this purpose, a syndrome-based diagnostic approach was published by Graus et al. (Lancet Neurol 15:391-404, 2016), but very little is known in the literature about its application in clinical practice.Aim Our aims are to test the feasibility of such approach in a real-world single-centre setting and to analyse the most relevant factors in criteria fulfilment.Methods We retrospectively applied these criteria to our cohort of patients discharged from our hospital with diagnosis of autoimmune encephalitis (n = 33, 58% antibody-positive).Results All the subjects fulfilled criteria for possible AE (pAE), with EEG and MRI playing a central role in diagnosis, while CSF was useful mainly to rule out other conditions. Three patients respected criteria for probable anti-NMDA-R encephalitis (pNMDA). Definite anti-NMDAR encephalitis was diagnosed in 4 patients with detection of the autoantibody but, surprisingly, none of these subjects had fulfilled criteria for pNMDA. 18 patients were diagnosed with definite limbic AE (15 patients were antibody-positive, three antibody-negative). Need for MRI bilateral involvement in antibody-negative limbic AE limited diagnosis. One patient fulfilled criteria for probable antibody-negative AE, while ten patients remained classified as pAE.Conclusion From our retrospective analysis, some suggestions for a better definition of the criteria may emerge. Larger studies on prospective cohorts may be more helpful to explore possible important issues.