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Abstract
Objective: To measure mortality, identify predictors of death and investigate causes of death in patients with anti-gamma-aminobutyric-acid B receptor (anti-GABABR) encephalitis.Methods: Prospective analysis of anti-GABABR encephalitis cases diagnosed between June 2013 and August 2018 in West China Hospital of Sichuan University, with assessment of factors associated with mortality.Results: A total of 28 patients (11 females) with anti-GABABR encephalitis were included in this study. After a maximum time of 52 months (median 11 months, range 2–52) of follow-up, 9 (32.1%) patients died, with a median survival time of 6.5 months. Five patients died of tumor progression, one patient died of convulsive status epilepticus, one patient died of septic shock, and two patients died of severe pneumonia. Predictors of death were older age at onset (P = 0.025), presence of a tumor (66.7 vs. 15.8%, P = 0.013), the number of complications (2.6 vs. 1.0, P = 0.009) and deep venous thrombosis (33.3% vs. 0, P = 0.026).Conclusion: Patients with GABABR encephalitis have a high mortality rate within 5 years. Older age at onset, presence a tumor, the number of complications, and deep venous thrombosis are associated with death.
Highlights
Anti-gamma-aminobutyric-acid B receptor encephalitis, which was first described by Lancaster et al (1), is clinically characterized by limbic encephalitis and other uncommon clinical syndromes (1)
The exclusion criteria were as follows: (1) patients with
We initially evaluated 31 patients with positive results of antiGABABR antibodies in CSF and/or serum, and three were excluded because two patients were lost to followup and one patient presented with Isaacs syndrome without encephalitis symptoms such as seizures, working memory deficits, or psychiatric disorders (8)
Summary
Anti-gamma-aminobutyric-acid B receptor (anti-GABABR) encephalitis, which was first described by Lancaster et al (1), is clinically characterized by limbic encephalitis (including seizures, cognitive disorders, behavioral changes) and other uncommon clinical syndromes (such as cerebellar ataxia and opsoclonus-myoclonus syndrome) (1). Previous studies have reported some patients had additional autoantibodies, such as antibodies targeting voltage-gated calcium channel (VGCC), glutamic acid decarboxylase 65 (GAD65), and sex determining region Y-box 1 (SOX1) (2, 3). Prognosis of Anti-GABAB Encephalitis that patients with anti-GABABR encephalitis have a poor prognosis, in those with tumors (1, 2). The prospective studies with respect to long-term prognosis of anti-GABABR encephalitis are lacking and predictors of death are still unknown. The primary objective of this prospective study was to assess long-term outcomes of patients with anti-GABABR encephalitis and analyze the possible predictors of death
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