Department of Interventional Radiology, Shandong Qianfoshan Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.
Department of Neurology, Binzhou Medical University Hospital, Binzhou, China.
Int J Dev Neurosci. 2021 Oct;81(6):492-501. doi: 10.1002/jdn.10121. Epub 2021 May 22.
To improve the clinical understanding of anti-gamma-aminobutyric-acid B receptor encephalitis (anti-GABABR encephalitis) by analyzing 13 cases.
We retrospectively studied demographic and clinical features including clinical symptoms, serum/cerebrospinal fluid (CSF) laboratory findings (including antibody test), brain magnetic resonance imaging (MRI), electroencephalogram (EEG), treatment plan, and treatment effect for 13 patients with a definitive diagnosis of anti-GABABR encephalitis.
Seven patients (53.8%, 7/13) were complicated with lung cancer. Epileptic seizures were the most common symptoms at onset in 11 patients (84.6%, 11/13). All patients had seizures in the course of the disease. Abnormalities in craniocerebral MRI examination, including hippocampus, occipital lobe, insular lobe, were found in six of nine tested patients, and EEG abnormalities were found in seven out of nine tested patients. Elevated pro-gastrin releasing peptide (ProGRP) levels were found in 70% of patients with a median value of 490.10 pg/ml; and CSF oligoclonal bands were positive for 4 of 10 tested cases. However, there were no significant differences in modified Rankin Scale (mRS) between the ProGRP or CSF oligoclonal band positive and negative groups at admission and follow-up (p > .05). The value between SCLC and non-SCLC subgroup was significantly different (p < .05). Ten patients received immunotherapy (three patients refused treatment). After immunotherapy, the frequency of seizures was significantly reduced. There was a significant difference in mRS between admission and after treatment (p < .05). The average survival time after onset was 27.7 months.
Epilepsy is the most common clinical manifestation of Anti-GABABR encephalitis. The prognosis of anti-GABABR encephalitis is poor. Section of anti-GABABR encephalitis patients have higher level of serum ProGRP and positive GSF oligoclonal bands. Elevated ProGRP or positive CSF oligoclonal bands with classic clinical features can potentially help to improve early recognition of anti-GABABR encephalitis.
通过分析 13 例病例，提高对抗γ-氨基丁酸 B 型受体脑炎（抗 GABABR 脑炎）的临床认识。
我们回顾性研究了 13 例明确诊断为抗 GABABR 脑炎患者的人口统计学和临床特征，包括临床症状、血清/脑脊液（CSF）实验室检查（包括抗体检测）、脑磁共振成像（MRI）、脑电图（EEG）、治疗方案和治疗效果。
7 例（53.8%，7/13）患者合并肺癌。11 例（84.6%，11/13）患者首发症状为癫痫发作。所有患者在病程中均出现癫痫发作。9 例检测患者中，6 例存在颅脑 MRI 检查异常，包括海马、枕叶、岛叶，9 例检测患者中 7 例存在 EEG 异常。70%的患者（中位值为 490.10 pg/ml）的胃泌素释放肽前体（ProGRP）水平升高，10 例检测患者中有 4 例 CSF 寡克隆带阳性。然而，入院和随访时 ProGRP 或 CSF 寡克隆带阳性和阴性组的改良 Rankin 量表（mRS）评分无显著差异（p>.05）。小细胞肺癌和非小细胞肺癌亚组之间的差异有统计学意义（p<.05）。10 例患者接受免疫治疗（3 例拒绝治疗）。免疫治疗后，癫痫发作频率明显降低，入院和治疗后 mRS 评分有显著差异（p<.05）。发病后平均生存时间为 27.7 个月。
癫痫发作是抗 GABABR 脑炎最常见的临床表现。抗 GABABR 脑炎的预后较差。抗 GABABR 脑炎患者的血清 ProGRP 水平较高，CSF 寡克隆带阳性。具有典型临床特征的升高的 ProGRP 或阳性 CSF 寡克隆带可能有助于提高对抗 GABABR 脑炎的早期识别。
