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Clinical characteristics and prognostic analysis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis in Northeast China.
BMC Neurology ( IF 2.2 ) Pub Date : 2020-01-03 , DOI: 10.1186/s12883-019-1585-y
Xinyue Zhang 1 , Yue Lang 1 , Lichao Sun 2 , Weiguanliu Zhang 1 , Weihong Lin 1 , Li Cui 1
Affiliation  

OBJECTIVE To investigate the clinical characteristics and prognosis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis. METHODS This retrospective study enrolled nineteen patients with anti-GABA-B receptor encephalitis. Clinical manifestations, radiological and electroencephalogram features, treatment and outcomes were collected and analyzed. The neurological function was evaluated according to the modified Rankin Scale (mRS). RESULTS There were eleven patients in the favorable-prognosis group (mRS ≤ 2) and eight patients in the poor-prognosis group (mRS > 2). In the favorable-prognosis group, clinical symptoms included memory deterioration (n = 10; 90.9%), epileptic seizures (n = 9; 81.8%), psychiatric disorders (n = 9; 81.8%), and conscious disturbance (n = 5; 45.5%); magnetic resonance imaging (MRI) indicated an involvement of the limbic system in three (27.3%) cases in this group. Lung cancer was detected in one patient (9.1%). After an average follow-up period of 11.7 months, four (36.4%) patients were cured, and seven (63.6%) patients showed significant improvements. In the poor-prognosis group, all patients presented with memory deterioration, epileptic seizures, psychiatric disorders, and conscious disturbance; five (62.5%) patients had convulsive status epilepticus, and five (62.5%) patients developed respiratory failure; MRI indicated an involvement of the limbic system in seven (87.5%) cases. Malignant tumors were detected in five (62.5%) patients. After an average follow-up period of 14.8 months, seven (87.5%) patients died and one (12.5%) patient remained dependent in daily life. CONCLUSIONS The clinical manifestations of anti-GABA-B receptor encephalitis include epileptic seizures, cognitive impairment and psychiatric disorders. Patients with convulsive status epilepticus or respiratory failure have poor outcomes. In anti-GABA-B receptor encephalitis, limbic system involvement is associated with a poor prognosis in and radiological examinations can reflect disease progression. Early diagnosis and appropriate treatment should be highlighted.

中文翻译:

东北地区抗γ-氨基丁酸-B(GABA-B)受体脑炎的临床特征和预后分析。

目的探讨抗γ-氨基丁酸-B(GABA-B)受体脑炎的临床特点及预后。方法这项回顾性研究招募了19例抗GABA-B受体脑炎的患者。收集并分析临床表现,影像学和脑电图特征,治疗和结局。根据改良的兰金量表(mRS)评估神经功能。结果预后良好组11例(mRS≤2),预后不良组8例(mRS> 2)。预后良好组的临床症状包括记忆力减退(n = 10; 90.9%),癫痫发作(n = 9; 81.8%),精神病(n = 9; 81.8%)和意识障碍(n = 5 ; 45.5%); 磁共振成像(MRI)显示该组中三例(27.3%)病例累及边缘系统。一名患者(9.1%)检测到肺癌。在平均11.7个月的随访期后,四名(36.4%)的患者治愈了,七名(63.6%)的患者表现出明显的改善。在预后不良组中,所有患者均出现记忆力减退,癫痫发作,精神病和意识障碍。5例(62.5%)的患者患有惊厥性癫痫持续状态,5例(62.5%)的患者出现呼吸衰竭。MRI提示边缘系统受累7例(87.5%)。在五名(62.5%)患者中检测到恶性肿瘤。在平均14.8个月的随访期后,有7名(87.5%)患者死亡,另有1名(12.5%)患者仍然依赖于日常生活。结论抗GABA-B受体脑炎的临床表现包括癫痫性癫痫发作,认知障碍和精神病。癫痫持续状态或呼吸衰竭的患者预后较差。在抗GABA-B受体脑炎中,边缘系统受累与预后不良有关,放射学检查可反映疾病进展。应强调早期诊断和适当治疗。
更新日期:2020-01-04
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