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Abstract

B cells and antibodies are increasingly recognized as an important feature in a broad variety of autoimmune and infectious central nervous system (CNS) diseases, such as multiple sclerosis, neuromyelitis optica, paraneoplastic diseases, acute demyelinating encephalomyelitis, Sydenham chorea, stiff-person syndrome and channelopathies. Autoantibodies might serve as a biomarker to identify the disease entity. Beyond this, autoantibodies can be a relevant pathogenic component. The present review focuses on strategies applied to identify the targets of autoantibodies and to test their pathogenetic relevance. The pathways of autoantibodies into and out of the CNS are summarized and the issue whether pathogenicity requires recognition of an extracellular target is discussed.