Autoimmune Astrocytopathy

  • Jorge CorrealeEmail author
  • María I. Gaitán
Part of the Contemporary Clinical Neuroscience book series (CCNE)


Astrocytes are the most abundant and heterogeneous type of glial cell in the Central Nervous System. In addition to their role maintaining physiological conditions stable in the CNS, they are recognized as early and highly active players in immune responses in the CNS, and their dysfunction is believed to contribute to neuroimmune disease.

Perhaps one of the most important discoveries in recent years has been the identification of IgG-NMO, a specific pathogenic antibody directed against water channel aquaporin-4 (AQP4). IgG-NMO has not only made neuromyelitis optica diagnosis easier but has allowed differential diagnoses to be established more clearly and lead to the design of better therapeutic alternatives. Likewise, a novel autoantibody directed against GFAP has been identified as biomarker of a relapsing autoimmune form of meningoencephalomyelitis, responsive to steroids, often associated with tumors. Similarly, in Rasmussen’s encephalitis, CD8+ T lymphocytes cause astrocyte apoptosis and loss in affected areas, altering normal neuron function. Reactive astrocytes also play an important role in different CNS infections, not only during acute phases of disease but also long term, and may condition the development of post-infectious sequelae. Finally, multiple mechanisms mediated by astrocytes are known to participate in both the genesis and the progression of MS and in processes of remyelination. Overall, these observations indicate astrocytes actively participate in both pathological and in repair mechanisms, observed in CNS neuroimmune diseases.


Anti-AQP4 Anti-GFAP Astrocytes Multiple sclerosis Neuromyelitis optica Rasmussen’s encephalitis 



α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid


Aquaporin 4


Adenosine triphosphate


4-Galactosyltransferase 5


B-cell activating factor


Blood–brain barrier


Complement component subunit 1q


Central Nervous System


Ciliary neurotrophic factor


Chondroitin sulfate proteoglycans




Danger-associated molecules patterns


Excitatory amino acid transporter 2


Experimental autoimmune encephalomyelitis


Extracellular matrix




Fas ligand


Fibroblast growth factor


Forkhead box P3




Glial fibrillary acidic protein


Glutamate/aspartate transporter


Glutamate transporter-1


Glutamate receptor 3


Granulocyte macrophage colony-stimulating factor


Glutamine synthetase


High-mobility box-1


Intercellular adhesion molecule 1




Inducible nitric oxide synthase


Interferon regulatory factor 1


Interferon-stimulated genes




Lymphocyte function-associated antigen


Leukemia inhibitory factor




Macrophage colony-stimulating factor


Matrix metalloproteinases


Nuclear factor kappa-light-chain-enhancer of activated B cells


Neuron-glial antigen 2




Neuromyelitis optica


Neuromyelitis optica spectrum disorders


Nitric oxide




Oligodendrocyte progenitor cells


Pathogen-associated molecular patterns


Pattern recognition receptors


Receptor for advanced glycation end products


Rasmussen’s encephalitis


Retinoic acid-inducible gene-like receptors


S100 calcium-binding protein


Transforming growth factor


T helper cell


T cell immunoglobulin and mucin domain 3


Tissue inhibitors of metalloproteinases


Toll-like receptor


Type 1 regulatory T cells


Vascular cell adhesion protein 1


Very late antigen 4



This work was supported by an unrestricted grant from FLENI.

The authors thank Dr. Ismael Calandri for preparation of some figures.


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of NeurologyFLENIBuenos AiresArgentina

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