Does MOG Ig-positive AQP4-seronegative opticospinal inflammatory disease justify a diagnosis of NMO spectrum disorder?
نویسندگان
چکیده
While neuromyelitis optica (NMO) immunoglobulin (Ig) G is considered the hallmark serologic marker of NMO, its association is not absolute, as NMO IgG is not detected in approximately one-fourth of the patients diagnosed with NMO spectrum disorder (NMOSD). Thus, the recent discovery that antibodies to myelin oligodendrocyte glycoprotein (MOG) are detected in some NMO IgG-seronegative patients manifesting clinical and neuroimaging signs of NMO or NMOSD has created tremendous excitement. However, it may be premature to classify this subgroup as NMOSD. NMO is considered an autoimmune astrocytopathy, and aquaporin-4 (AQP4), expressed on astrocytes, is recognized as the target autoantigen of NMO IgG. As its name denotes, MOG is produced by oligodendrocytes, CNS myelin-producing cells, and MOG is well-recognized as one of the candidate autoantigens in multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). Thus, is it possible that the clinical NMOSD-like phenotype associated with MOG-specific antibodies represents a variant of opticospinal MS or ADEM but not AQP4 autoimmunity or NMOSD? Whether this MOG-Ig positive AQP4-seronegative phenotype should be classified as NMOSD, opticospinal MS, or a unique entity is not simply a theoretical question but rather has practical implications for patients, their physicians, insurance carriers, and clinical investigators conducting NMO treatment trials.
منابع مشابه
MOG antibody-associated diseases
In the past few years several studies have consistently reported the presence of high-titer serum IgG antibodies to conformational epitopes of the myelin oligodendrocyte glycoprotein (MOG) in predominantly pediatric patients with acquired demyelinating diseases. Moreover, MOG antibodies seem not only to distinguish patients with clinically isolated syndromes or multiple sclerosis (MS) from mono...
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OBJECTIVE To evaluate clinical features among patients with neuromyelitis optica spectrum disorders (NMOSD) who have myelin oligodendrocyte glycoprotein (MOG) antibodies, aquaporin-4 (AQP4) antibodies, or seronegativity for both antibodies. METHODS Sera from patients diagnosed with NMOSD in 1 of 3 centers (2 sites in Brazil and 1 site in Japan) were tested for MOG and AQP4 antibodies using ce...
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Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system (CNS) characterized by severe optic neuritis (ON) and acute myelitis (AM). NMO differs in many respects from multiple sclerosis (MS). NMO-IgG is an autoantibody exclusively detected in the sera of NMO, and is directed against aquaporin-4 (AQP4), a water channel richly expressed on astrocytes in the CNS. In most ...
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Correspondence: Yara Dadalti Fragoso; Rua da Constituição, 374; 11015470 Santos SP; Brazil. E-mail: [email protected]. br Conflict of interest: There is no conflict of interest to declare. Received 13 April 2014 Accepted 28 April 2014 Neuromyelitis optica (NMO) is a relatively rare and particularly intriguing disease. NMO was first described by Devic and Gault at the end of the 19 century, but it ...
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