Table 2.
#of patients (region, centers) | Demographic information | Common types | Clinical characteristics | Auxiliary examination | Treatment | Prognosis and outcome | |
---|---|---|---|---|---|---|---|
This study | N = 1,027 N = 778 (China, 23 centers) | 1–87 y.o. (median = 35); female 45.50% | Anti-NMDAR (61.35%), Anti-LGi1 (20.57%), Anti-GABAbR (10.67%), others (5.27%) | Psychosis (53.21%), seizures (61.95%), headache (32.59%), fever (33.20%), consciousness impairment (28.54%) | MRI: abnormal FLAIR signals (61.2%) mainly in the medial temporal lobe. EEG: slow activity (61.67%) and epileptiform discharges (6.88%) | First-line: steroid (85.48%) and IVIG (66.77%), mostly combined (57.1%); PE (2.5%) Second-line: RTX or CTX (4.5%) |
17.74% with relapse: anti-NMDAR (64.49%), anti-LGi1 (22.46%), anti-GABAR (10.11%), anti-Caspr2 (3.62%) |
Deng et al. | N = 86 (China, single-center) | Mean = 32.9 y.o.; female 44.19% | Anti-NMDAR (83.72%), Anti-LGi1 (4.65%), Anti-GABAbR (5.81%), anti-Caspr2 (3.49%), others (3.49%) | Psychiatric disturbance, epilepsy, autonomic dysfunction, sleep disorders, consciousness disorders | MRI: abnormal in 50%, mainly insular/hippocampal abnormality. EEG: abnormal in 71%; typically slow waves and sharp waves, mainly in the frontal area (48.8%) and area centralis (32.6%) | steroid or in combination with PE, IVIG, or immunosuppressive agents like cyclophosphamide | Immune therapy administered within 15 days from onset was associated with a higher rate of mRS score difference ≥ 2 |
Gu et al. | N = 189 (Southwest China, multi-center) | 1–70 y.o. (median = 16); female 61.38% | Anti-NMDAR (80.95%), Anti-LGi1 (4.76%), Anti-GABAbR (7.41%), anti-Caspr2 (2.65%), others (4.23%) | N/A in detail; 62.22% of adult and 10.10% of children admitted to ICU | N/A | N/A | 76.71% of male patients and 92.92% of female patients had a good prognosis |
Probasco et al. | N = 61 (Sweden, single-center) | Median = 54 y.o.; female 54% | Anti-NMDAR (13.11%), Anti-LGi1 (8.20%), Anti-VKGC (6.56%), anti-GAD65 (6.56%), other seropositive (18.03%) | Short-term memory impairment (75%), cerebellar signs (77%), focal weakness (61%), focal numbness (57%), movement disorder (64%), seizures (41%) | FDG-PET/CT: brain regional abnormal metabolism 85% | N/A | N/A |
Titulaer et al. | N = 577 (USA, multi-center) | <18 y.o.: 37%; >18 y.o.:63%; female 81% |
Anti-NMDAR specifically | Behavior problem (65%), movement disorder (50%), Seizures (50%); 77% admitted to ICU | MRI: abnormal in 33% EEG: abnormal in 90% | First-line immunotherapy: 92% Second-line immunotherapy: 27% Tumor removal y: 2% |
Favorable outcomes: 81% Relapse: 11% |
Mueller et al. | N = 150 (Germany, multicenter) | Anti-NMDAR: mean = 30.3 y.o. Anti-LGi1: mean = 62.7 y.o. |
Anti-NMDAR and anti-Lgi1 specifically | Anti-NMDAR: seizures (73%), psychiatric symptoms (92%), movement disorder (45%), autonomic dysfunction (39%) Anti-Lgi1: seizures (76%), psychiatric symptoms (57%), movement disorder (11%), autonomic dysfunction (12%) |
Anti-NMDAR: abnormal MRI in 57%, abnormal EEG in 75% Anti-LGi1: abnormal MRI in 74%, abnormal EEG in 78% | N/A | N/A |
Xu et al. (12) | N = 220 (China single-center) | <18 y.o.: 31.08%; >18 y.o.: 68.92%, female 81% |
Anti-NMDAR specifically | Psychosis 82.7%, seizures 80.9%, Fever 57.3%, decrease of consciousness 53.2%, 30.9% admitted to ICU | MRI: abnormal in 35.9% EEG: abnormal in 51.4% | First-line immunotherapy: 99.5% Second-line immunotherapy: 7.3% |
94.1% improvement 3.6% stable 2.3% died |
Guan et al. (13) | N = 531 (China single-center) | N/A. | Anti-NMDAR (79.7%), Anti-LGi1 (12.8%), Anti-GABAbR (5.6%), others (1.8%) | N/A. | N/A. | N/A. | Relapse: 23.5% |