Neuromyelitis Optica

Bruce A.C. Cree, MD, PhD; Douglas S. Goodin, MD; Stephen L. Hauser, MD


Semin Neurol. 2002;22(2) 

In This Article


Unfortunately, there are no proven effective therapies for NMO. Glucocorticoids are typically used to treat cases acutely and may be beneficial.[29,31,134] Some patients appear to become glucocorticoid dependent and experience relapses when the dosage of prednisone is lowered.[29] Plasma exchange may be tried in patients who do not respond to glucocorticoids.[135] In an uncontrolled case series, 6 of 10 patients with NMO treated with plasma exchange showed moderate or marked improvement.[136] Interferons and sometimes immunosuppressant drugs are used with the hope that further relapses will be prevented, but prospective data in support of their efficacy are lacking.[27,29] In one uncontrolled series seven patients with NMO were treated with long-term prednisone and azathioprine and were observed to improve after 6 months of therapy.[137] Because patients with NMO can improve spontaneously, it is not possible to determine from this uncontrolled series whether this regimen was of any benefit. One case report described a possible benefit of lymphocytaplasmapheresis in a 26-year-old pregnant patient with NMO.[138] Based on recent experimental[126] and pathological[132] evidence, it seems likely that immunoglobulins and complement deposition play a role in the pathogenesis of NMO. Consequently, therapies directed toward inhibiting complement (such as soluble Cr-1),[139] depletion of B-cells (anti-CD20),[140] or plasma exchange[136] should be investigated in randomized, controlled trials.


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