Significance of Autoantibodies

  • Christiane S. HampeEmail author
Part of the Contemporary Clinical Neuroscience book series (CCNE)


The field of autoimmune neurological disorders is rapidly expanding, and novel autoantibodies and their neuronal antigens continue to be discovered. Autoimmunity targeting brain proteins is enigmatic, because traditionally, the central nervous system (CNS) is viewed as immune-privileged. However, the discovery of the lymphatic and glymphatic circulation in the CNS demonstrates the interaction between the CNS and the immune response. Furthermore, the barriers protecting the brain from direct exposure to the immune response can be compromised by inflammations, infection, or injury. A compromised blood–brain barrier, or blood–cerebrospinal fluid barrier, will allow egress of neuronal antigens to regional and peripheral lymphoid organs and may lead to the initiation of an autoimmune response. Peripheral autoantibodies or intrathecally produced autoantibodies can reenter the CNS. Besides being useful diagnostic markers, these autoantibodies may be involved in the pathogenesis of the disease by mechanisms such as complement-dependent cytotoxicity (CDC), antibody-dependent cell-mediated cytotoxicity (ADCC), and modulation of receptor function. The effect of a neural autoantibody depends not only on the nature of its antigen but also on the antibody’s Ig isotype or IgG subclass. We will discuss different causes of neurological autoimmunity and pathogenic mechanisms involved in neurological autoimmune diseases. Finally, we will discuss naturally occurring IgM autoantibodies and IgG4 autoantibodies with protective and reparative functions and appropriate treatment options.


Autoantibodies Blood–brain barrier Blood–cerebrospinal fluid barrier Neurological disorders 



Acetylcholine receptor


Antibody-dependent cell-mediated cytotoxicity


Adipocyte enhancer-binding protein-1


α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic glutamate receptor




Autism spectrum disorder


Blood–brain barrier


Cerebellar ataxia caspr: contactin-associated protein 1


Complement-dependent cell death


Cerebellar degeneration-related protein 2-like


Chronic inflammatory demyelinating polyneuropathy


Central nervous system


Collapsing response mediator protein


Cerebrospinal fluid


Gamma-aminobutyric acid


65kda isoform of glutamate decarboxylase


Guillain–Barré syndrome


Glial fibrillary acidic protein


Heterogeneous nuclear ribonuclear protein-A1


Herpes simplex virus-1


HSV encephalitis


Lactate dehydrogenase


Lambert–Eaton myasthenic syndrome


Lipoprotein receptor-related protein 4


Membrane attack complex


Myelin-associated glycoprotein


Maternal autoantibody-related autism


Myelin basic protein


Myasthenia gravis


Myelin oligodendrocyte glycoprotein


Multiple sclerosis


Muscle-specific kinase


Myeloma overexpressed gene 2


N-Methyl-D-aspartate glutamate receptor


NMDAR encephalitis


Neuromuscular junction


Neuromyelitis optica


Paraneoplastic degeneration


Paraneoplastic encephalomyelitis


Paraneoplastic neurologic disorder


Proteasome non-ATPase regulatory subunit 4


Rasmussen’s encephalitis


Spinal cord injury


Small-cell lung cancer


Sulfoglucuronosyl paragloboside


Systemic lupus erythematosus


Stiff person syndrome


Stress-induced phosphoprotein 1


Small myelin protein-derived peptides


Traumatic brain injury




Voltage-gated calcium channel


Voltage-gated potassium channel


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of MedicineUniversity of Washington School of MedicineSeattleUSA

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