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Objective: This study aims to retrospectively study clinical characteristics, diagnosis, treatment and follow-up visits in patients with anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) for prompting the early diagnosis and efficacious treatment of this disease. Methods: Four anti-LGI1 LE patients were hospitalized in the Department of Neurology at the First Hospital of Jilin University. Data from their clinical manifestations, diagnoses, treatments and follow-up visits were analyzed. Results: Four patients with anti-LGI1 LE were characterized by hypomnesia, mental and behavior disorder, faciobrachial dystonic seizures (FBDS), seizures accompanied by pathological changes in the limbic system, obstinate hyponatremia and positive detection of anti-LGI1 antibody. In this study, we found that 1 patient initially presented with an abnormal sensation and had a subsequent FBDS, inconsistent with normal cases of anti-LGI1 LE. This patient had abnormalities in the basal ganglia region identified with head MRI, unlike most cases, which report abnormalities in the limbic system by positron emission tomography/CT scan. Finally, 3 of our patients with FBDS were treated with antiepileptic drugs as well as immune modulatory treatment, and they responded well. Conclusions: We identified new characteristics in clinical manifestation, diagnosis and treatment of anti-LGI1 LE, which contribute to a better understanding of this disease and help improve its early diagnosis and efficacious treatment. [ABSTRACT FROM AUTHOR]