Rehabilitation Unit, Pitié-Salpêtrière Hospital, AP-HP, Paris, France.
Clinical Investigation Centre, Institut du Cerveau, Pitié-Salpêtrière Hospital, Paris, France.
J Neurol. 2022 Jun;269(6):3109-3118. doi: 10.1007/s00415-021-10894-y. Epub 2021 Nov 23.
Seizures represent a core symptom of autoimmune encephalitides with specific therapeutic issues. To date, patients with new-onset seizures or established epilepsy are not systematically tested for autoimmune antibodies. We aimed to identify clinical and paraclinical criterion that could help to select patients requiring additional autoimmune antibodies serum and cerebrospinal fluid (CSF) detection.
In this retrospective single center study from the French Salpêtrière Hospital, data from 286 adult patients with epilepsy who received an autoantibody assay for the first time were analyzed. All patients were evaluated at our institution between January 2007 and December 2018 for assessment of new-onset epilepsy (n = 90) or established epilepsy (n = 196). We only analyzed patients that were screened for autoimmune antibodies. Demographic, clinical and neuroimaging measures were compared between patients with and without autoimmune encephalitis using Fisher's exact test for categorical variables and Welch's t test for continuous variables. Our primary goal was to identify significant factors that differentiated patients with and without autoimmune encephalitis.
We identified 27 patients with autoimmune epilepsy (9.4% of the patients who had been tested for autoantibodies). The significant factors differentiating patients with and without autoimmune encephalitis were: (i) the existence of a new-onset focal epilepsy + (e.g., newly diagnosed epilepsy < 6 months associated with additional symptoms, mainly cognitive or psychiatric symptoms), (ii) the presence of faciobrachial dystonic seizures very suggestive of anti- Leucine-rich glioma inactivated 1 (LGI1) encephalitis, and (iii) the presence of magnetic resonance imaging (MRI) abnormalities suggestive of encephalitis.
New-onset focal seizures combined with cognitive or psychiatric symptoms support the test for autoimmune antibodies. Further clinical already known red flags for an autoimmune origin are the presence of faciobrachial dystonic seizures and MRI signal changes consistent with encephalitis. On the other hand, isolated new-onset seizures and chronic epilepsy, even with associated symptoms, seem rarely linked to autoimmune encephalitis and should not lead to systematic testing.
癫痫发作是自身免疫性脑炎的核心症状之一，具有特定的治疗问题。迄今为止，新发癫痫发作或已确诊癫痫的患者并未系统地进行自身抗体检测。我们旨在确定有助于选择需要进一步检测血清和脑脊液（CSF）中自身抗体的患者的临床和辅助检查标准。
本研究为回顾性单中心研究，纳入了 286 名首次接受自身抗体检测的成年癫痫患者。所有患者于 2007 年 1 月至 2018 年 12 月在我院进行评估，以诊断新发癫痫（n=90）或已确诊癫痫（n=196）。仅分析了接受自身抗体筛查的患者。Fisher 确切检验用于分类变量，Welch's t 检验用于连续变量，比较了自身免疫性脑炎患者与非自身免疫性脑炎患者的人口统计学、临床和神经影像学指标。我们的主要目标是确定区分自身免疫性脑炎患者与非自身免疫性脑炎患者的显著因素。
我们发现了 27 例自身免疫性癫痫患者（接受自身抗体检测的患者中占 9.4%）。区分自身免疫性脑炎患者与非自身免疫性脑炎患者的显著因素包括：（i）新发局灶性癫痫的存在+（例如，新诊断的癫痫<6 个月，伴有其他症状，主要为认知或精神症状）；（ii）存在面臂肌张力障碍发作，强烈提示抗富亮氨酸胶质瘤失活 1 抗体（LGI1）脑炎；（iii）磁共振成像（MRI）存在提示脑炎的异常信号。
新发局灶性癫痫伴有认知或精神症状支持进行自身抗体检测。进一步提示自身免疫源性的临床已知“危险信号”是面臂肌张力障碍发作和与脑炎一致的 MRI 信号改变。另一方面，孤立性新发癫痫发作和慢性癫痫发作，即使伴有相关症状，似乎很少与自身免疫性脑炎相关，不应导致系统检测。
