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Objective:To analyze the relationship between serum antibody titers, clinical characteristics, and prognosis in patients with encephalitis induced by anti leucine-rich glioma inactivated 1 (LGI1) antibody.Methods:Clinical data of 20 patients diagnosed with encephalitis in the Department of Neurology, First Hospital of Shanxi Medical University from February 2015 to February 2021 were collected and retrospectively analyzed. Patients were divided into 2 groups based on their serum anti LGI1 antibody titers, namely the high titer group (1∶100, 1∶320) and the low titer group (1∶10, 1∶32). The clinical characteristics, laboratory test results, and prognosis of the 2 groups of patients were compared. Relusts The age of the 20 patients with anti LGI1 antibody encephalitis ranged from 27 to 69 (53.5±11.2) years, with a male to female ratio of 1∶1. There were 9 patients in the low titer group and 11 patients in the high titer group. There was no statistically significant difference in the types and quantities of clinical symptoms between the 2 groups. Patients in the high titer group were more prone to abnormal lesions on cranial magnetic resonance imaging (10/11 vs 3/9, P=0.014). There was no statistically significant difference between the 2 groups in the presence or absence of cerebrospinal fluid anti LGI1 antibodies (9/11 vs 4/9, P=0.160). During the follow-up, it was found that 1/20 patient died of pulmonary embolism, 7/20 of patients were able to recover to their predisease state, and 9/20 of patients had residual memory impairment. In the high titer group, 3/11 of patients experienced recurrence, while there was no recurrence in the low titer group. There was no statistically significant difference in the neurological prognosis between the 2 groups at 3 months of discharge and follow-up (the number of patients whose modified Rankin Scale score≤2: 10/10 vs 8/9, P=0.474). Conclusions:Patients with high serum anti LGI1 antibody titers are more likely to develop intracerebral lesions. Higher antibody titers may be associated with a higher risk of disease recurrence. There was no significant correlation between serum antibody titers and neurological outcomes.
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Objective To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in antileucine-rich glioma-inactivated 1 (LGI1) encephalitis by analyzing the clinical and immunologic data of patients treated with MMF in this prospective cohort of anti-LGI1 encephalitis.Methods Patients treated with MMF for more than one year in Peking Union Medical College Hospital were included in this study.MMF was given at a dosage of 1.5-3.0 g/d in the induction period (two to four months) and 0.75-2.00 g/d in the maintenance period.All the patients were followed up regularly.Modified Rankin Scale (mRS) score evaluation,serum IgG and peripheral CD19-positive B cells,CD4-positive T cells and CD8-positive T cells testing were performed every two months.Results Fifteen patients were included in this study who received first-line immunotherapy combined with MMF.No other second-line therapy including rituximab was used.Thirteen patients responded well to MMF combined with first-line immunotherapy (a decrease in mRS score of more than 1).All 15 patients had a good outcome (i.e.,a mRS score of 0-2),including nine patients without residual symptoms (a mRS score of 0).After 12 months of MMF treatment,CD19-positive B cells were significantly decreased (median 320 (227,628) × 106/L vs 152 (105,223) × 106/L;Z=-2.028,P=0.043),while serum IgG (9.07 (6.70,11.32) g/L vs 8.35 (6.63,10.69) g/L,P=0.144)),CD4-positive T cells (1 136 (736,1 432) × 106/L vs 1 055 (802,1 072) × 106/L,P =0.866) and CD8-positive T cells (627 (413,784) × 106/L vs 568 (393,743) × 106/L,P =0.735) were not significantly changed.Three patients relapsed and were treated with additional cycle of first-line immunotherapy and increased dosage of MMF (induction dosage) resulting in remission.CD19-positive B cells were tested to be increased during the patients' relapse.No serious adverse event was noted in all these patients.Conclusions MMF is safe and effective as a long-term immunotherapy in patients with anti-LGI1 encephalitis.MMF can be used as an add-on therapy to first-line immunotherapy for autoimmune encephalitis.CD19-positive B cell count should be monitored and used as a parameter to individualize dosage of MMF.