Immunopathology: Autoimmune glial diseases and differentiation from multiple sclerosis

Bogdan F.G.H. Popescu, Claudia F. Lucchinetti

Research output: Chapter in Book/Report/Conference proceedingConference contribution

7 Scopus citations


While multiple sclerosis (MS) is often referred to as an autoimmune inflammatory demyelinating disease, neuromyelitis optica (NMO) is currently the only proven and well-characterized autoimmune disease affecting the glial cells. The target antigen is the water channel aquaporin-4 (AQP4), expressed on astrocytes, and antibodies against AQP4 (AQP4-IgG) are present in the serum of NMO patients. Clinical, serologic, cerebrospinal fluid, and neuroimaging criteria help differentiate NMO from other central nervous system inflammatory demyelinating disorders. Pathologically, the presence of dystrophic astrocytes, myelin vacuolation, granulocytic inflammatory infiltrates, vascular hyalinization, macrophages containing glial fibrillary acidic protein-positive debris and/or the absence of Creutzfeldt-Peters cells is more characteristic, but not specific, for NMO. These findings should prompt the neuropathologist to perform AQP4 immunohistochemistry, and recommend serologic testing for AQP4-IgG to exclude a diagnosis of NMO/NMO spectrum disorder (NMOSD). Loss of AQP4 on biopsied active demyelinating lesions and/or seropositivity for AQP4-IgG may confirm the diagnosis of NMO/NMOSD, which is important because treatments that are suitable for MS can aggravate NMO. Few other putative glial antigens have been postulated, but their pathogenic role remains to be demonstrated.

Original languageEnglish (US)
Title of host publicationAutoimmune Neurology, 2016
EditorsSean J. Pittock, Angela Vincent
Number of pages12
ISBN (Print)9780444634320
StatePublished - 2016

Publication series

NameHandbook of Clinical Neurology
ISSN (Print)0072-9752
ISSN (Electronic)2212-4152


  • Antibody
  • Aquaporin-4
  • Astrocyte
  • Demyelination
  • Microglia
  • Neuromyelitis optica
  • Oligodendrocyte

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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