Clinical analysis of 17 cases of LGI1 limbic encephalitis / 中国神经精神疾病杂志

ABSTRACT

Objective The objective of this study was to study LGI1 limbic encephalitis (LE). Methods We performed a retrospective analysis on the clinical features,laboratory findings,imaging profiles and treatment outcomes of 17 patients with LGI1. Results The study included 14 male and 3 female cases. The median age was 61 years old. The clinical manifestations includes 14 cases with cognitive dysfunction, 11 cases with faciobrachial dystonic seizures (FBDS), 10 cases with focal seizures, 8 cases with generalizedtonic-clonic seizure and 9 cases with mental and behavioral disorder. Among 17 examined patients, there were 16 patients with positive and 1 with negative serum LGI1 antibody (but whose CSF LGI1 antibody was positive). Among 16 examined patients, there were 14 patients with positive cerebrospinal fluid LGI1 antibody. All patients had good responses to the first-line treatment and 2 patients experienced recurrence. During more than one-year follow-up, the recurrence rate was 33％ and no patient died. Conclusion LGI1 LE is an autoimmune encephalitis, which is mainly unidirectional progression and can relapse. FBDS and focal seizures usually are first symptoms, followed by cognitive dysfunction. Patients are responded to immunotherapy well and have good prognosis.