Long-term Outcome of LGI-1 Encephalitis in Chinese: a 2-year follow-up

Abstract

Background Antibodies directed to leucien-rich glioma-inactivated 1 (LGI-1) encephalitis is a rare autoimmune encephalitis,characterized by limbic encephalitis syndrome and faciobrachial dystonic seizures (FBDS) . Most of cases are sensitive to immunotherapy in acute phase. Our aim was to give a detailed description of the long-term outcome of the LGI-1 encephalitis in Chinese . Methods We enrolled 36 patients with LGI-1 antibodies in serum/CSF from September 2013 to December 2016 and of which 28 patients were performed a 2-year follow-up. Clinical data of all patients was recorded and clinical outcome was assessed at 2-year follow-up. Follow-up MRI was scanned in partial patients. Results 11(39.3%)patients(mRS =0)had complete recovery,7(25.0%)patients(mRS =1)had mild neurological dysfunction, 10(7.2%)patients had severe neurological dysfunction(mRS≥2)and 8 patients (28.6%) had relapses.The numbers of patients with residual psychiatric change and memory deficit was 5 (17.8%)and 15(53.6%) respectively. No patients had a residual seizures and FBDS. Follow-up MRI were available in 10 patients. Among 5 patients with normal MRI in acute phase, 1 patients showed bilateral hippocampus atrophy on follow-up MRI and among 5 patients with abnormal MRI in acute phase, 4 patients showed lesion partial remission, 1 patients showed lesion dissolve on follow-up MRI. Conclusion Our study showed that only one third of patients with IGI-1 encephalitis got complete recovery at 2-year follow-up and relapses are common. The major residual symptom is memory deficit.