Chen Xueping, Liu Fan, Li Jin-Mei, Xie Xiao-Qi, Wang Qiong, Zhou Dong, Shang Huifang
a Department of Neurology , West China Hospital, Sichuan University , Chengdu , China.
b Department of Nursing , West China Hospital of Stomatology, Sichuan University , Chengdu , China.
Neurol Res. 2017 Nov;39(11):973-980. doi: 10.1080/01616412.2017.1351062. Epub 2017 Oct 3.
Autoimmune encephalitis associated with antibodies against gamma-aminobutyric-acid B receptor (GABAR) has not been described in detail in Chinese patients.
Patients with anti-GABAR encephalitis treated between January 2013 and December 2015 were analyzed in terms of clinical characteristics, laboratory findings, tumor presence, autoantibody patterns, treatment response and outcomes.
Eleven patients were identified (male, N = 8; female, N = 3), with the median age of 51 years. All patients presented with seizures (N = 11; 100%), and they were given anti-epileptic drugs and first-line immunotherapy to address the disease. Seizures always accompanied by limbic manifestations (N = 10; 90.9%). Extralimbic manifestations were present in 4 patients (N = 4; 36.4%). MRI Brain abnormality with increased medio-temporal lobe T2/FLAIR signal were present in 2 patients (N = 2; 18.2%), and epileptiform epileptiform activity on electroencephalography were observed in 2 patients (N = 2; 18.2%). Small-cell lung cancer was histologically confirmed in 3 patients (N = 3; 27.3%). Seven patients showed good outcomes (mRS 1-2; N = 7; 63.6%), one patient showed poor neurological status with minimal changes (mRS 4; N = 1; 9.1%), and three patients died during follow-up (mRS = 6; N = 3; 27.3%). Outcomes were correlated with age-of-onset, and were worse among older patients (P = 0.0112).
Anti-GABAR encephalitis is a potentially treatable disorder involving seizures as the most predominant presentation at admission. It should be considered as a possible diagnosis in middle-aged and older patients with refractory new-onset epilepsy.
在中国患者中，尚未对与抗γ-氨基丁酸B受体（GABAR）抗体相关的自身免疫性脑炎进行详细描述。
对2013年1月至2015年12月期间接受治疗的抗GABAR脑炎患者的临床特征、实验室检查结果、肿瘤存在情况、自身抗体模式、治疗反应和结局进行分析。
共确定11例患者（男性8例，女性3例），中位年龄51岁。所有患者均出现癫痫发作（11例，100%），并给予抗癫痫药物和一线免疫治疗以治疗该疾病。癫痫发作常伴有边缘系统表现（10例，90.9%）。4例患者出现边缘叶外表现（4例，36.4%）。2例患者脑部MRI显示中颞叶T2/FLAIR信号增强（2例，18.2%），2例患者脑电图观察到癫痫样放电（2例，18.2%）。3例患者经组织学确诊为小细胞肺癌（3例，27.3%）。7例患者预后良好（改良Rankin量表评分1-2分；7例，63.6%），1例患者神经功能状态较差且变化极小（改良Rankin量表评分4分；1例，9.1%），3例患者在随访期间死亡（改良Rankin量表评分=6分；3例，27.3%）。结局与发病年龄相关，老年患者结局更差（P=0.0112）。
抗GABAR脑炎是一种潜在可治疗的疾病，入院时最主要的表现为癫痫发作。对于中年及老年新发难治性癫痫患者，应考虑将其作为一种可能的诊断。
