Ganesh Aravind, Bartolini Luca, Wesley Sarah F
Department of Clinical Neurosciences (AG), University of Calgary, Canada; Centre for Prevention of Stroke and Dementia (AG), University of Oxford, United Kingdom; Clinical Epilepsy Section (LB), National Institutes of Health, Bethesda, MD; and Department of Neurology (SFW), Yale School of Medicine, New Haven, CT.
Neurol Clin Pract. 2020 Apr;10(2):140-148. doi: 10.1212/CPJ.0000000000000701.
To explore practice differences in the diagnosis and management of autoimmune encephalitis (AE), which is complicated by issues with sensitivity/specificity of antibody testing, nonspecific MRI/EEG/CSF findings, and competing differential diagnoses.
We used a worldwide electronic survey with practice-related demographic questions and clinical questions about 2 cases: (1) a 20-year-old woman with a neuropsychiatric presentation strongly suspicious of AE and (2) a 40-year-old man with new temporal lobe seizures and cognitive impairment. Responses among different groups were compared using multivariable logistic regression.
We received 1,333 responses from 94 countries; 12.0% identified as neuroimmunologists. Case 1: those treating >5 AE cases per year were more likely to send antibodies in both serum and CSF (adjusted odds ratio [aOR] vs 0 per year: 3.29, 95% CI 1.31-8.28, = 0.011), pursue empiric immunotherapy (aOR: 2.42, 95% CI 1.33-4.40, = 0.004), and continue immunotherapy despite no response and negative antibodies at 2 weeks (aOR: 1.65, 95% CI 1.02-2.69, = 0.043). Case 2: neuroimmunologists were more likely to send antibodies in both serum and CSF (aOR: 1.80, 95% CI 1.12-2.90, = 0.015). Those seeing >5 AE cases per year (aOR: 1.86, 95% CI 1.22-2.86, = 0.004) were more likely to start immunotherapy without waiting for antibody results.
Our results highlight the heterogeneous management of AE. Neuroimmunologists and those treating more AE cases generally take a more proactive approach to testing and immunotherapy than peers. Results highlight the need for higher-quality cohorts and trials to guide empiric immunotherapy, and evidence-based guidelines aimed at both experts and nonexperts. Because the average AE patient is unlikely to be first seen by a neuroimmunologist, ensuring greater uniformity in our approach to suspected cases is essential to ensure that patients are appropriately managed.
探讨自身免疫性脑炎（AE）诊断和管理中的实践差异，AE存在抗体检测的敏感性/特异性问题、非特异性的磁共振成像（MRI）/脑电图（EEG）/脑脊液（CSF）检查结果以及鉴别诊断相互竞争等问题。
我们开展了一项全球电子调查，包含与实践相关的人口统计学问题以及关于2个病例的临床问题：（1）一名20岁女性，有强烈怀疑为AE的神经精神症状表现；（2）一名40岁男性，有新发颞叶癫痫和认知障碍。使用多变量逻辑回归比较不同组之间的回答。
我们收到了来自94个国家的1333份回复；12.0%的受访者为神经免疫学家。病例1：每年治疗超过5例AE病例的医生更有可能同时送检血清和脑脊液中的抗体（与每年治疗0例相比，调整后的优势比[aOR]：3.29，95%置信区间[CI] 1.31 - 8.28，P = 0.011），采用经验性免疫治疗（aOR：2.42，95% CI 1.33 - 4.40，P = 0.004），并且即便在2周时无反应且抗体检测为阴性仍继续免疫治疗（aOR：1.65，95% CI 1.02 - 2.69，P = 0.043）。病例2：神经免疫学家更有可能同时送检血清和脑脊液中的抗体（aOR：1.80，95% CI 1.12 - 2.90，P = 0.015）。每年诊治超过5例AE病例的医生（aOR：1.86，95% CI 1.22 - 2.86，P = 0.004）更有可能在不等待抗体结果的情况下开始免疫治疗。
我们的结果凸显了AE管理的异质性。神经免疫学家以及诊治更多AE病例的医生通常比同行在检测和免疫治疗方面采取更积极主动的方法。结果凸显了需要更高质量的队列研究和试验来指导经验性免疫治疗，以及针对专家和非专家的循证指南。由于普通AE患者不太可能首先由神经免疫学家接诊，确保我们对疑似病例的处理方法更加统一对于确保患者得到恰当管理至关重要。
