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Advances in research on neuromyelitis optica spectrum disorders and its clinical heterogeneity |
| Yi Bao*, Ming Jin, Qi Zhao, Lu Liu, Yanpeng Sun, Xiaoqin Peng, Lu Yang, Guangjian Liu |
| Department of Neurology, Taihe Hospital Affiliated to Hubei University of Medicine, Shiyan 442000, China |
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Abstract 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.
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neuromyelitis optica spectrum disorders (NMOSD)
aquaporin-4-immunoglobulin G (AQP4-IgG)
myelin oligodendrocyte glycoprotein (MOG)
rituximab (RTX)
paraneoplastic neurological syndrome
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Issue Date: 23 May 2020
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