Updates in NMOSD and MOGAD Diagnosis and Treatment A Tale of Two Central Nervous System Autoimmune Inflammatory Disorders

被引:33
作者
Cacciaguerra, Laura [1 ]
Flanagan, Eoin P. [1 ,2 ,3 ]
机构
[1] Mayo Clin, Ctr Multiple Sclerosis & Autoimmune Neurol, Dept Neurol, Rochester, MN USA
[2] Mayo Clin, Lab Med & Pathol, Rochester, MN USA
[3] Dept Neurol, 200 1st St SW, Rochester, MN 55905 USA
关键词
AQP4-IgG; MOG-IgG; Diagnosis; Treatment; myelin oligodendrocyte glycoprotein; MOG; aquaporin-4; neuromyelitis optica spectrum disorder; MYELIN-OLIGODENDROCYTE GLYCOPROTEIN; OPTICA SPECTRUM DISORDERS; BRIGHT SPOTTY LESIONS; NEUROMYELITIS-OPTICA; MULTIPLE-SCLEROSIS; ANTIMYELIN ANTIBODIES; MYASTHENIA-GRAVIS; CORTICAL-LESIONS; CLINICAL-COURSE; BRAIN-LESIONS;
D O I
10.1016/j.ncl.2023.06.009
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The recent identification of AQP4+NMOSD and MOGAD as separate disorders has prompted the development of separate diagnostic criteria for MOGAD diagnosis. The stratification of patients based on the presence/absence of AQP4-IgG in NMOSD and stratification by antibody-titer in MOGAD represents the main differences between the two criteria. Using CBA in serum, AQP4-IgG and MOG-IgG are sensitive and highly specific but caution is needed with low-positive MOG-IgG, which is found in 1% to 2% of disease controls. In addition, specific MRI features of the diseases are described and included as supportive criteria in uncertain cases. NMOSD negative for AQP4-IgG and MOG-IgG likely represents a heterogenous group of disorders that should be a focus of research to potentially discover new antibodies that associate with demyelination. A number of highly effective attack-prevention treatments are now available for AQP4+NMOSD and should be started promptly at diagnosis. Empiric attack-prevention treatments in MOGAD are generally reserved for those with 2 or more attacks. However, level 1 evidence is lacking in MOGAD. The approach to developing treatments in AQP4+NMOSD will be a useful template for MOGAD and randomized clinical trials are now underway with the hope for a proven attackprevention treatment in the near future.
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页码:77 / 114
页数:38
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