Abstract
Neuromyelitis optica (NMO) is characterized by severe optic neuritis and longitudinally
extended, transverse myelitis. There have been long controversial whether NMO is a
variant of multiple sclerosis (MS) or a different disease. However, since the discovery
of an NMO-specific autoantibody to aquaporin 4 (AQP4), a dominant water channel in
the central nervous system densely expressed on foot processes of astrocytes, the
clinical distinction between NMO and MS has become clear, and now AQP4 antibody status
is critically important for neurologists in deciding on treatment strategy. Moreover,
pathological studies demonstrated an extensive loss of immunoreactivities to AQP4
and glial fibrillary acidic protein (GFAP) with relative preservation of the staining
of myelin basic protein in acute NMO lesions, which is not seen in MS. In fact, the
GFAP levels in the cerebrospinal fluid during acute exacerbation of NMO are remarkably
elevated, while the values in MS are not different from those in controls. In addition,
recent experimental studies conducted in vitro and in vivo have shown that AQP4 antibody
is pathogenic. These findings strongly suggest that AQP4 antibody has diagnostic,
therapeutic and pathogenetic implications, and that severe astrocytic damage mediated
by AQP4 antibody distinguishes NMO from MS.
Keywords
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Article info
Publication history
Published online: March 14, 2011
Accepted:
February 16,
2011
Received in revised form:
October 19,
2010
Received:
April 30,
2010
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
