An Unusual Case of LGI1 (Leucine-Rich Glioma-Inactivated Protein 1) Limbic Encephalitis With Anti-acetylcholine Receptor and Anti-striational Autoantibodies.

ABSTRACT

Autoimmune encephalitis (AE) results from immune-mediated damage to the central nervous system (CNS) with varying clinical manifestations depending on autoimmune antibodies present and the antigens they target. Leucine-rich glioma-inactivated protein 1 (LGI1) has been recognized as one of the leading causes of limbic encephalitis (LE), presenting with seizures, memory loss, and faciobrachial dystonic seizures. A better understanding of the unique presentations of these AE allows for quick and effective diagnosis and treatment. We are presenting a very unusual case of LGI1 autoimmune LE with two additional autoantibodies, anti-acetylcholine receptor (AChR) and anti-striational, in a patient with an underlying thymoma. We will discuss the pathophysiology and common clinical presentation of anti-LGI1 autoimmune LE.