Epilepsy and Autoantibodies

Abstract

For many years the central nervous system (CNS) has been considered an immune privileged site, since alloengraftment within the CNS induced a poor immune response. This weak response was explained by: (1) the presence of a blood-brain barrier (BBB); (2) constitutive lack or low expression of proteins of the immune system, such as major histocompatibility complex (MHC) class I and II, co-stimulatory and accessory molecules, on cells of the CNS (gUal cells, neurons, astrocytes); and (3) lack of lymphatic drainage [1] (see also Introduction to this volume). Nevertheless, the CNS is frequently the target of immune-mediated reactions which may have an autoimmune or an infectious aetiology. Through the years, it has become evident that autoantibodies against brain antigens exist and might lead to brain impairment and, in some disorders, are associated with seizures [2]. In this chapter, we will focus on the CNS disorders characterized by epilepsy and the presence of autoantibodies against neuronal antigens (Table 1). Table 1 Neurological diseases characterized by epilepsy and autoantibodies Antibody Target Immuno-therapy Rasmussen’s encephalitis (RE) Epilepsia partialis continua Drug-resistant luR3 Plasma exchange or protein A immunoadsorption Systemic lupus erythematosus (SLE) Primary generalized before SLE onset Phospholipid, Cardiolipin β2-glycoprotein I Not reported Therapy-resistant localization-related epilepsy Cardiolipin, nuclear, β2-glycoprotein I GADa Not reported Newly diagnosed seizure Cardiolipin, nuclear β2-glycoprotein I Not reported Generalized epilepsy syndromes Cardiolipin Not reported West’s syndrome Corticosteroids, intravenuous therapy Cryptogenic Lennox-Gastaut syndrome Haemocyanin Intravenous therapy Completely-controlled epilepsy GADa Not reported a The presence of anti-GAD antibodies in uncontrolled and completely-controlled epilepsy is disputed [56, 57] .