Neuropsychiatric Disease and Treatment (Jan 2021)
A Retrospective Study of Patients with GABABR Encephalitis: Therapy, Disease Activity and Prognostic Factors
Abstract
Xiangchuan Wen,1 Baojie Wang,1 Chunjuan Wang,1,2 Chenglin Han,3 Shougang Guo1,2 1Department of Neurology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong 250021, People’s Republic of China; 2Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong 250021, People’s Republic of China; 3Department of Urology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong 250021, People’s Republic of ChinaCorrespondence: Shougang GuoDepartment of Neurology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jing Wu Road, Huaiyin District, Jinan, 250021 Shandong, People’s Republic of ChinaTel +86-13220585081Email [email protected]: To explore the effects of immunotherapy and tumour treatment on patients with GABABR encephalitis, evaluate the correlation between immune cell subsets and disease activity, and investigate effective prognostic factors.Patients and Methods: Twenty patients with γ-aminobutyric acid B receptor (GABABR) encephalitis were enrolled from December 2015 to April 2020. The clinical data, modified Rankin Scale (mRS) score, prognosis and percentage of serum lymphocytes were recorded.Results: All patients received first-line immunotherapy. The median mRS scores were 4 and 3 before and after first-line immunotherapy (P< 0.01). Seven patients received second-line immunotherapy and had median mRS scores of 3 and 2 before and after second-line immunotherapy (P=0.015). Small-cell lung cancer was detected in twelve patients. Among the patients who died because of tumours, patients who received tumour treatment lived longer than patients who did not receive tumour treatment (P=0.025). All four surviving patients who received tumour treatment had good outcomes (mRS≤ 2). The median serum CD19+B cell percentage in sixteen patients were 20.00% and 13.42% prior first-line immunotherapy and at the last follow-up (P< 0.01). After a maximum follow-up of 54 months (median: 12; range: 3– 54), eleven patients (55%) had a poor prognosis (mRS> 2). Predictors of a poor prognosis were older age (P=0.031), delayed initial improvement after immunotherapy (> 4 weeks) (P=0.038) and respiratory failure (P=0.038).Conclusion: Aggressive immunotherapy and tumour treatment contribute to improvements in neurological function and a better prognosis of patients with GABABR encephalitis. The serum CD19+B cell percentage may be an indicator of disease activity. Older age, delayed initial improvement after immunotherapy, and respiratory failure may be associated with poor outcomes.Keywords: GABABR encephalitis, immunotherapy, tumour treatment, CD19+B cell percentage, prognosis