TY - GEN
T1 - Immunopathology
T2 - Autoimmune glial diseases and differentiation from multiple sclerosis
AU - Popescu, Bogdan F.G.H.
AU - Lucchinetti, Claudia F.
PY - 2016
Y1 - 2016
N2 - 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.
AB - 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.
KW - Antibody
KW - Aquaporin-4
KW - Astrocyte
KW - Demyelination
KW - Microglia
KW - Neuromyelitis optica
KW - Oligodendrocyte
UR - http://www.scopus.com/inward/record.url?scp=84978387415&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84978387415&partnerID=8YFLogxK
U2 - 10.1016/B978-0-444-63432-0.00006-2
DO - 10.1016/B978-0-444-63432-0.00006-2
M3 - Conference contribution
C2 - 27112673
AN - SCOPUS:84978387415
SN - 9780444634320
T3 - Handbook of Clinical Neurology
SP - 95
EP - 106
BT - Autoimmune Neurology, 2016
A2 - Pittock, Sean J.
A2 - Vincent, Angela
PB - Elsevier
ER -