Objectives: This study described the clinical characteristics of autoimmune neurological diseases with dual seropositive antibodies of CASPR2 and LGI1. Patients and Methods: Three patients, with antibodies targeting both CASPR2 and LGI1 (EUROIMMUN, FA 112d-1, Germany), hospitalized in Department of Neurology, Xuanwu Hospital, Capital Medical University from June 2016 to June 2019 were collected in this study. We summarized the clinical characteristics of patients with CASPR2 and LGI1 antibodies from a targeted literature review. Results: Three patients reported were all middle-aged and elderly male with diverse neurological symptoms, including seizures, psychological abnormalities, limb weakness and hyperhidrosis. Interestingly, three patients displayed three different clinical syndromes (isolated epilepsy, Morvan syndrome and limbic encephalitis, respectively). White blood cell and glucose in Cerebrospinal fluid (CSF) were normal and CSF for protein was slightly elevated. Electromyography (EMG) showed abnormal spontaneous firing in case 2. Brain magnetic resonance imaging (MRI) revealed bilateral hyper-intensity of the temporal lobe on T2 and FLAIR sequence in case 3. Cancer screening program of patient 2 showed thymoma. Cell based assay was positive in serum for both LGI1 and CASPR2 antibodies, while these antibodies were negative in CSF. They were treated with glucocorticoid or intravenous immunoglobulin (IVIG). Followed up for 6 months to 1 year, all patients got remission to different extent. Conclusion: Through the detailed analysis of three patients, the combination of both antibodies contributes to a broad spectrum of neurological symptoms in the central, peripheral, and autonomic nervous systems. The patients with same antibodies can have different clinical syndromes. Early tumor screening and immunotherapy will improve the prognosis of the disease. © 2020 Elsevier B.V.