视神经脊髓炎
医学
脊髓炎
视神经炎
多发性硬化
光谱紊乱
儿科
疾病
脱髓鞘病
免疫抑制
脊髓
呕吐
皮肤病科
水通道蛋白4
横贯性脊髓炎
重症监护医学
病理
免疫学
内科学
精神科
作者
Saif Huda,Dan Whittam,Maneesh Bhojak,Jayne L. Chamberlain,Carmel P. Noonan,Anu Jacob,Rachel Kneen
出处
期刊:Clinical Medicine
[Royal College of Physicians]
日期:2019-03-01
卷期号:19 (2): 169-176
被引量:109
标识
DOI:10.7861/clinmedicine.19-2-169
摘要
Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord inflammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Relapses are treated aggressively to prevent residual disability with high-dose steroids and often plasma exchange. Relapse prevention is crucial and achieved with long-term immunosuppression. In this article we review the pathogenesis, clinical features, diagnosis and management of NMOSD.
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