Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies

Abstract

Several studies have certified that autoantibodies play an important role in the manifestation of neuromuscular diseases. Scientists have discovered specific neuronal tumor antibodies in patients with typical paraneoplastic neurological disorders. But in some clinical cases, it is not useful to cure this disease with common treatments unless the autoantibodies are addressed. In addition, recent studies have shown a close relationship between certain antibodies and neuronal surface proteins in some special cases. These antibodies, which act on the surface of neurons, mainly include voltage-gated calcium channel (VGKC) antibodies. VGKC antibodies are further divided into several types including antileucine-rich glioma inactivated 1 (LGI1), anti-contactin-associated protein-like 2 (Caspr2), anti-N-methyl-D-aspartate receptor (NMDAR), anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), anti-γ-aminobutyric acid receptor (GABAR), and glycine receptor. For the purpose of this review, cases of clinical studies of autoantibody-associated encephalitis were collected, the key points regarding the pathogenesis were summarized, the clinical manifestation was discussed, and all this information was organized as this review in order to introduce the relationship between autoantibodies and autoimmune encephalitis. Furthermore, it is hoped that it can effectively direct the development of diagnostic and therapeutic approach in the future.

Case 20: Autoimmune Encephalitis with Unusual Antibodies

“…First-line treatment of LE include steroids, intravenous immunoglobulin, or combination of both. Additionally, some patients require second-line treatments such as rituximab and/ or cyclophosphamide [5]. Therefore, LE is a treatable condition with total clinical recovery, but treatment is often delayed due to the lack of specific symptoms and the time it takes to obtain the result of the autoantibodies analysis.…”