Department of Neurology, Shandong Provincial Qianfoshan Hospital Affiliated to Shandong University, No.16766 Jingshi Road, Lixia District, Jinan, 250014, Shandong, China.
Department of Neurology, Zaozhuang Municopal Hospital, Zaozhuang, 277102, Shandong, China.
Neurol Sci. 2019 Apr;40(4):769-777. doi: 10.1007/s10072-018-3679-5. Epub 2019 Jan 22.
To explore the clinical, imaging, and electroencephalogram (EEG) findings, as well as the treatment and prognosis of five patients with anti-γ-aminobutyric acid B receptor (GABAR) encephalitis and review the current literature to gain a deeper understanding and improve the clinical diagnostic ability of the disease.
Clinical data such as blood examination, imaging, computed tomography (CT), EEG, and magnetic resonance imaging (MRI) findings from five patients with anti-GABAR encephalitis were retrospectively analyzed.
Based on the imaging data, autoimmune encephalitis with anti-GABAR antibodies displayed subacute onset of episodic memory loss, seizures, and confusion, in addition to signal changes in the medial temporal lobe and/or hippocampus. Anti-GABAR antibodies were found in blood and cerebrospinal fluid (CSF) in all five patients, although the CSF leukocyte count and the levels of protein, sugar, and chloride showed no obvious abnormalities. On MRI, only two patients presented with abnormal signals in the medial temporal lobe and/or hippocampus. The EEG showed a slow wave rhythm in all five patients. After treatment with methylprednisolone pulse therapy combined with antiepileptic treatment, all five patients recovered well, without any complications.
Autoimmune encephalitis with anti-GABAR antibodies may be a severe and refractory disease. Anti-GABAR antibodies tested in CSF and serum play a crucial role in the definitive diagnosis and treatment of autoimmune encephalitis. Early treatment is of vital importance to avoid serious complications and neurological sequelae.
探讨 5 例抗γ-氨基丁酸 B 型受体(GABAR)脑炎患者的临床、影像学和脑电图(EEG)表现,以及治疗和预后,并复习相关文献,加深对该病的认识,提高临床诊断能力。
回顾性分析 5 例抗 GABAR 脑炎患者的血液检查、影像学(包括 CT 和 MRI)、EEG 等临床资料。
根据影像学资料,抗 GABAR 抗体自身免疫性脑炎呈亚急性发作,表现为 episodic memory loss、癫痫发作和意识混乱,伴有内侧颞叶和/或海马信号改变。5 例患者血和脑脊液(CSF)中均检测到抗 GABAR 抗体,尽管 CSF 白细胞计数和蛋白、糖、氯水平无明显异常。MRI 仅 2 例患者存在内侧颞叶和/或海马异常信号。EEG 显示 5 例患者均呈慢波节律。经甲基强的松龙脉冲联合抗癫痫治疗后,5 例患者均恢复良好,无任何并发症。
抗 GABAR 抗体自身免疫性脑炎可能是一种严重且难治的疾病。CSF 和血清中抗 GABAR 抗体检测对自身免疫性脑炎的明确诊断和治疗具有重要意义。早期治疗对于避免严重并发症和神经后遗症至关重要。