PT  - JOURNAL ARTICLE
AU  - Tangna Sun
AU  - Daidi Zhao
AU  - Gejuan Zhang
AU  - Yue Huang
AU  - Jia Guo
AU  - Wen Jiang
AU  - Rui Jia
AU  - Maynur Maimaiti
AU  - Jianguo Liu
AU  - Ning Bu
AU  - Zunbo Li
AU  - Yaping Yan
AU  - Xiaoyan Zhang
AU  - Chenjing Sun
AU  - Cong Zhao
AU  - Xiaotao Jia
AU  - Baoyi Mao
AU  - Hui Tian
AU  - Yan Liu
AU  - Zheng Chen
AU  - Zilian Fan
AU  - Xiaoyan Guo
AU  - Jiarui Lu
AU  - Kaixi Ren
AU  - Hongzeng Li
AU  - Jun Guo
TI  - Late-Onset Anti-GABA<sub>B</sub> Receptor Encephalitis
AID  - 10.1212/NXI.0000000000200131
DP  - 2023 Jul 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e200131
VI  - 10
IP  - 4
4099  - http://nn.neurology.org/content/10/4/e200131.short
4100  - http://nn.neurology.org/content/10/4/e200131.full
SO  - Neurol Neuroimmunol Neuroinflamm2023 Jul 01; 10
AB  - Background and Objectives Existing evidence indicates anti-GABAB receptor encephalitis (GABABR-E) seems to occur more commonly later in life, yet the age-associated differences in clinical features and outcomes are not well determined. This study aims to explore the demographic, clinical characteristics, and prognostic differences between late-onset and early-onset GABABR-E and identify predictors of favorable long-term outcomes.Methods This is an observational retrospective study conducted in 19 centers from China. Data from 62 patients with GABABR-E were compared between late-onset (aged 50 years or older) and early-onset (younger than 50 years) groups and between groups with favorable outcomes (modified Rankin scale (mRS) ≤ 2) and poor outcomes (mRS >2). Logistic regression analyses were applied to identify factors affecting long-term outcomes.Results Forty-one (66.1%) patients experienced late-onset GABABR-E. A greater proportion of males, a higher mRS score at onset, higher frequencies of ICU admission and tumors, and a higher risk of death were demonstrated in the late-onset group than in the early-onset group. Compared with poor outcomes, patients with favorable outcomes had a younger onset age, a lower mRS score at onset, lower frequencies of ICU admission and tumors, and a greater proportion with immunotherapy maintenance for at least 6 months. On multivariate regression analysis, age at onset (OR, 0.849, 95% CI 0.739−0.974, p = 0.020) and the presence of underlying tumors (OR, 0.095, 95% CI 0.015−0.613, p = 0.013) were associated with poorer long-term outcomes, whereas immunotherapy maintenance for at least 6 months was associated with favorable outcomes (OR, 10.958, 95% CI 1.469−81.742, p = 0.020).Discussion These results demonstrate the importance of risk stratification of GABABR-E according to age at onset. More attention should be paid to older patients especially with underlying tumors, and immunotherapy maintenance for at least 6 months is recommended to achieve a favorable outcome.CBA=cell-based assay; GABAB=gamma aminobutyric acid–B; GABABR=gamma aminobutyric acid–B receptor; GAD65=glutamic acid decarboxylase 65; ICU=intensive care unit; IQR=interquartile range; IVIG=intravenous immunoglobulin; mRS=modified Rankin scale; NMDAR=N-methyl-d-aspartate receptor; OR=odds ratio; KCTD16=potassium channel tetramerization domain–containing 16; PKC-γ=protein kinase C gamma; SCLC=small cell lung cancer; SOX1=Sry-like high-mobility group box 1; TBA=tissue-based assay; Tr/DENR=delta/notch–like epidermal growth factor–related receptor; Zic4=zinc finger protein 4