Neuromyelitis optica spectrum disorders(NMOSD)is a demyelinating disease mainly involving the optic nerve and spinal cord.It has recurrent and aggravating attacks and high disability rate.Most patients have a stepwise...Neuromyelitis optica spectrum disorders(NMOSD)is a demyelinating disease mainly involving the optic nerve and spinal cord.It has recurrent and aggravating attacks and high disability rate.Most patients have a stepwise progression,resulting in complete blindness or paraplegia.NMOSD lesions contain not only the optic nerve and spinal cord,but also other neurological and non-neurological symptoms,which has clinical heterogeneity.The discovery of aquaporin-4-immunoglobulin G(AQP4-IgG)attributed it to autoimmune ion-channel disease,and rituximab(RTX)has achieved good clinical efficacy in the treatment of NMOSD.Myelin oligodendrocyte glycoprotein(MOG)antibodies have been found in some AQP4-IgG-negative NMOSD patients,which have different clinical and immunological features,posing new challenges to the diagnosis and treatment of NMOSD,which may require re-design and testing of new immune-targeted drugs.展开更多
We characterized a unique group of patients with neuromyelitis optica spectrum disorder （NMOSD） who carded autoantibod- ies of aquaporin-4 （AQP4） and myelin-oligodendrocyte glycoprotein （MOG）. Among the 125 NMOS...We characterized a unique group of patients with neuromyelitis optica spectrum disorder （NMOSD） who carded autoantibod- ies of aquaporin-4 （AQP4） and myelin-oligodendrocyte glycoprotein （MOG）. Among the 125 NMOSD patients, 10 （8.0%） were AQP4- and MOG-ab double positive, and 14 （11.2%） were MOG-ab single positive. The double-positive patients had a multiphase disease course with a high annual relapse rate （P=0.0431）, and severe residual disability （P〈0.0001）. Of the dou- ble-positive patients, 70% had MS-like brain lesions, more severe edematous, multifocal regions on spinal magnetic resonance imaging （MRI）, pronounced decreases of retinal nerve fiber layer thickness and atrophy of optic nerves. In contrast, patients with only MOG-ab had a higher ratio of monophasic disease course and mild residual disability. Spinal cord MRI illustrated multifocal cord lesions with mild edema, and brain MRIs showed more lesions around lateral ventricles. NMOSD patients carrying both autoantibodies to AQP4 and MOG existed and exhibited combined features of prototypic NMO and relaps- ing-remitting form of MS, whereas NMOSD with antibodies to MOG only exhibited an ＂intermediate＂ phenotype between NMOSD and MS. Our study suggests that antibodies against MOG might be pathogenic in NMOSD patients and that determi- nation of anti-MOG antibodies maybe instructive for management of NMOSD patients.展开更多
文摘Neuromyelitis optica spectrum disorders(NMOSD)is a demyelinating disease mainly involving the optic nerve and spinal cord.It has recurrent and aggravating attacks and high disability rate.Most patients have a stepwise progression,resulting in complete blindness or paraplegia.NMOSD lesions contain not only the optic nerve and spinal cord,but also other neurological and non-neurological symptoms,which has clinical heterogeneity.The discovery of aquaporin-4-immunoglobulin G(AQP4-IgG)attributed it to autoimmune ion-channel disease,and rituximab(RTX)has achieved good clinical efficacy in the treatment of NMOSD.Myelin oligodendrocyte glycoprotein(MOG)antibodies have been found in some AQP4-IgG-negative NMOSD patients,which have different clinical and immunological features,posing new challenges to the diagnosis and treatment of NMOSD,which may require re-design and testing of new immune-targeted drugs.
基金supported by National Basic Research Program of China Grant (2013CB96690)the Natural Science Foundation of China Grants (81100888, 81230028, 81371372)+2 种基金the National Key Clinical Specialty Construction Program of ChinaUS National Institute of Health (R01AI083294)the American Heart Association (14GRNT18970031)
文摘We characterized a unique group of patients with neuromyelitis optica spectrum disorder （NMOSD） who carded autoantibod- ies of aquaporin-4 （AQP4） and myelin-oligodendrocyte glycoprotein （MOG）. Among the 125 NMOSD patients, 10 （8.0%） were AQP4- and MOG-ab double positive, and 14 （11.2%） were MOG-ab single positive. The double-positive patients had a multiphase disease course with a high annual relapse rate （P=0.0431）, and severe residual disability （P〈0.0001）. Of the dou- ble-positive patients, 70% had MS-like brain lesions, more severe edematous, multifocal regions on spinal magnetic resonance imaging （MRI）, pronounced decreases of retinal nerve fiber layer thickness and atrophy of optic nerves. In contrast, patients with only MOG-ab had a higher ratio of monophasic disease course and mild residual disability. Spinal cord MRI illustrated multifocal cord lesions with mild edema, and brain MRIs showed more lesions around lateral ventricles. NMOSD patients carrying both autoantibodies to AQP4 and MOG existed and exhibited combined features of prototypic NMO and relaps- ing-remitting form of MS, whereas NMOSD with antibodies to MOG only exhibited an ＂intermediate＂ phenotype between NMOSD and MS. Our study suggests that antibodies against MOG might be pathogenic in NMOSD patients and that determi- nation of anti-MOG antibodies maybe instructive for management of NMOSD patients.
