A case series of five patients with anti-γ-aminobutyric acid type B receptor encephalitis
10.3760/cma.j.cn113694-20191105-00683
- VernacularTitle:抗γ-氨基丁酸B型受体脑炎五例分析
- Author:
Lina LI
1
;
Ling LI
;
Liu TU
;
Qingyan YANG
;
Jing FAN
;
Jie WANG
;
Jinhao YE
;
Zhenze LU
;
Jifu CAI
;
Haibing XIAO
Author Information
1. 香港大学深圳医院神经内科，广东深圳 518053
- From:
Chinese Journal of Neurology
2020;53(4):298-304
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features, treatment and prognosis of anti-γ-aminobutyric acid type B receptor (GABA B R) encephalitis. Methods:Retrospective analysis of five patients of anti-GABA BR encephalitis from the Department of Neurology, the University of Hong Kong-Shenzhen Hospital from September 2017 to June 2019 was carried out. Clinical manifestations, auxiliary examination, and treatment were analyzed. The patients were followed up for 3.5-23.0 months to assess their prognosis. Results:Five cases of anti-GABA BR encephalitis (19-81 years old) presented acute onset, with refractory epilepsy as the main clinical manifestation. There were hyperintensive signals on T 2/fluid attenuated inversion recovery in four patients′ temporal lobe and hippocampus. Electroencephalogram showed slow wave or epileptic discharge; Lung mass was found in four patients, and all were small cell lung cancer. Five cases had poor response to first-line immunotherapy (intravenous use of pulse methylprednisolone, high dose immunoglobulin or plasma exchange), then three patients received second-line immunotherapy (rituximab, cyclophosphamide), two of whom with tumor also received tumor chemotherapy. Patients who received second-line treatment and tumor chemotherapy showed better outcome than those who only received first-line treatment. Conclusions:Anti-GABA BR encephalitis present with limbic encephalitis syndromes characterized by refractory epilepsy. For patients with poor response to first-line immunotherapy, initiating second-line immunotherapy as soon as possible can improve the prognosis significantly.
