TY - JOUR
T1 - Mechanisms of disease
T2 - Aquaporin-4 antibodies in neuromyelitis optica
AU - Jarius, Sven
AU - Paul, Friedemann
AU - Franciotta, Diego
AU - Waters, Patrick
AU - Zipp, Frauke
AU - Hohlfeld, Reinhard
AU - Vincent, Angela
AU - Wildemann, Brigitte
PY - 2008/4
Y1 - 2008/4
N2 - Neuromyelitis optica (NMO) is a rare CNS inflammatory disorder that predominantly affects the optic nerves and spinal cord. Recent serological findings strongly suggest that NMO is a distinct disease rather than a subtype of multiple sclerosis. In NMO, serum antibodies, collectively known as NMO-IgG, characteristically bind to cerebral microvessels, pia mater and Virchow-Robin spaces. The main target antigen for this immunoreactivity has been identified as aquaporin-4 (AQP4). The antibodies are highly specific for NMO, and they are also found in patients with longitudinally extensive transverse myelitis without optic neuritis, which is thought to be a precursor to NMO in some cases. An antibody-mediated pathogenesis for NMO is supported by several observations, including the characteristics of the AQP4 antibodies, the distinct NMO pathology - which includes IgG and complement deposition and loss of AQP4 from spinal cord lesions - and emerging evidence of the beneficial effects of B-cell depletion and plasma exchange. Many aspects of the pathogenesis, however, remain unclear.
AB - Neuromyelitis optica (NMO) is a rare CNS inflammatory disorder that predominantly affects the optic nerves and spinal cord. Recent serological findings strongly suggest that NMO is a distinct disease rather than a subtype of multiple sclerosis. In NMO, serum antibodies, collectively known as NMO-IgG, characteristically bind to cerebral microvessels, pia mater and Virchow-Robin spaces. The main target antigen for this immunoreactivity has been identified as aquaporin-4 (AQP4). The antibodies are highly specific for NMO, and they are also found in patients with longitudinally extensive transverse myelitis without optic neuritis, which is thought to be a precursor to NMO in some cases. An antibody-mediated pathogenesis for NMO is supported by several observations, including the characteristics of the AQP4 antibodies, the distinct NMO pathology - which includes IgG and complement deposition and loss of AQP4 from spinal cord lesions - and emerging evidence of the beneficial effects of B-cell depletion and plasma exchange. Many aspects of the pathogenesis, however, remain unclear.
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U2 - 10.1038/ncpneuro0764
DO - 10.1038/ncpneuro0764
M3 - Article
C2 - 18334978
AN - SCOPUS:42049118701
VL - 4
SP - 202
EP - 214
JO - Nature Clinical Practice Neurology
JF - Nature Clinical Practice Neurology
SN - 1745-834X
IS - 4
ER -