Clinical study on antibody-associated limbic encephalitis

Abstract

In recent years, the antibody-associated limbic encephalitis (LE) has attracted attentions of more and more clinicians. The associated antibodies mainly act on neuronal cell surface antigens, including the N-methyl-D-aspartate (NMDA) receptor, the α-amino-3 -hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor, the γ-aminobutyric acid B (GABA B ) receptor, leucine-rich glioma⁃ inactivated 1 (LGI1) and contactin-associated protein-like 2 (Caspr2) and so on. The clinical manifestation is primarily defined by the subacute onset of short ⁃ term memory loss, seizures, confusion and psychiatric symptoms suggesting the involvement of the limbic system. These severe and protracted disorders can affect children and young adults, occurring with or without tumor association. Routine detection of serum and cerebrospinal fluid (CSF) and imaging tests show no specificity, but associated antibodies can be detected in serum and (or) CSF. The patients respond well to tumor resection and immunotherapies, including corticosteroids, intravenous immunoglobulin (IVIg), plasma exchange or combination of them, but may relapse. This article aims to study the clinical features and treatment of antibody-associated limbic encephalitis and to improve the diagnosis and prognosis of these diseases.