Bhavani Madduluri, Shaik A Jabeen, Reshma S Shaik, Sireesha Yareeda, Suryaprabha Turaga, Madhur K Srivastava, Charan Neeradi
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引用次数: 0
Abstract
Background and objectives: Seronegative autoimmune encephalitis (SNAIE) is an enigmatic entity which lacks detectable autoantibodies, leading to a diagnostic delay. Our objective was to study the frequency of SNAIE and compare the clinical, electrophysiological, and imaging characteristics of patients with SNAIE to those in patients with seropositive autoimmune encephalitis (SPAIE).
Methods: This ambispective observational study was undertaken at a tertiary care center in South India for 7 years. The demographic data, clinical features, cerebrospinal fluid (CSF) analysis findings, electroencephalogram (EEG), and 3 T magnetic resonance imaging (MRI), and 18F fluoro-2-deoxy-d-glucose positron emission tomography computerized tomography (18F FDG PET-CT) findings were compared.
Results: Of the 64 subjects enrolled, SNAIE frequency was 62.5%, with a notable 3:1 male-to-female ratio. Seizure was the predominant clinical presentation in SNAIE compared to SPAIE (50% vs. 20%, P value 0.02). Refractory seizures (45% vs. 4.5%) and status epilepticus (37.5% vs. 0%) were more common in SNAIE ( P value 0.001), whereas involuntary movements and faciobrachial dystonic seizures were common in SPAIE (66% vs. 16%, P value 0.04). CSF pleocytosis was frequently seen in SPAIE (50% vs. 17%, P value 0.006). EEG findings in SNAIE included increased epileptiform discharges and built-up rhythms. FDG PET abnormalities were observed in both groups (91% SPAIE, 97% SNAIE), but the seropositive group exhibited more temporal lobe involvement (58% vs. 30%, P = 0.036) and SNAIE had more diffuse involvement.
Conclusions: SNAIE exhibited male predominance, primarily presenting with isolated seizure as the initial presentation. EEG and FDG PET findings distinguished the two groups, emphasizing the importance of these modalities as biomarkers in the early detection and management of SNAIE.
背景和目的:血清阴性自身免疫性脑炎(SNAIE)是一种神秘的实体,缺乏可检测的自身抗体,导致诊断延迟。我们的目的是研究SNAIE的发生频率,并比较SNAIE患者与血清阳性自身免疫性脑炎(SPAIE)患者的临床、电生理和影像学特征。方法:在南印度的一家三级保健中心进行了为期7年的双视角观察研究。比较人口学资料、临床特征、脑脊液(CSF)分析结果、脑电图(EEG)、3t磁共振成像(MRI)和18F氟-2-脱氧-d-葡萄糖正电子发射断层扫描计算机断层扫描(18F FDG PET-CT)结果。结果:入组的64名受试者中,SNAIE发生率为62.5%,男女比例为3:1。与SPAIE相比,癫痫发作是SNAIE的主要临床表现(50%对20%,P值0.02)。顽固性癫痫发作(45%对4.5%)和癫痫持续状态(37.5%对0%)在SNAIE中更为常见(P值0.001),而不自主运动和面肱肌张力障碍发作在SPAIE中更为常见(66%对16%,P值0.04)。脑脊液多细胞症在SPAIE中常见(50% vs. 17%, P值0.006)。SNAIE的脑电图结果包括癫痫样放电增加和累积节律。两组均有FDG PET异常(SPAIE组91%,SNAIE组97%),但血清阳性组表现出更多颞叶受累(58% vs. 30%, P = 0.036), SNAIE更弥漫性受累。结论:SNAIE表现为男性优势,主要以孤立性癫痫发作为首发表现。脑电图和FDG - PET结果区分了两组,强调了这些模式作为SNAIE早期检测和管理的生物标志物的重要性。
期刊介绍:
The journal has a clinical foundation and has been utilized most by clinical neurologists for improving the practice of neurology. While the focus is on neurology in India, the journal publishes manuscripts of high value from all parts of the world. Journal publishes reviews of various types, original articles, short communications, interesting images and case reports. The journal respects the scientific submission of its authors and believes in following an expeditious double-blind peer review process and endeavors to complete the review process within scheduled time frame. A significant effort from the author and the journal perhaps enables to strike an equilibrium to meet the professional expectations of the peers in the world of scientific publication. AIAN believes in safeguarding the privacy rights of human subjects. In order to comply with it, the journal instructs all authors when uploading the manuscript to also add the ethical clearance (human/animals)/ informed consent of subject in the manuscript. This applies to the study/case report that involves animal/human subjects/human specimens e.g. extracted tooth part/soft tissue for biopsy/in vitro analysis.