Efficacy of Therapeutic Plasma Exchange in Acute Attacks of Neuromyelitis Optica
Young-Min Lim, MD, Bong-Hui Kang, MD, Kwang-Kuk Kim, MD, PhD.
Department of Neurology, University of Ulsan College of Medicine, Seoul, Korea
Background: Acute attacks of neuromyelitis optica (NMO) are usually severe and frequently unresponsive to high-dose steroids. Identification of aquaporin-4 antibodies and the pathologic studies showing immunoglobulin and complement deposition in NMO lesions suggest that humoral immunity plays a major role in its pathogenesis and plasma exchange (PE) can be used to treat NMO.
The aim of this study is to evaluate the therapeutic efficacy of PE for acute severe attacks in NMO.
Methods: We included 7 NMO-IgG positive patients with NMO who had PE between July 2007 and June 2009. The outcome was evaluated based on decrement in the Expanded Disability Status Scale (EDSS) and NMO-IgG titers after PE.
Results: All patients were women, and the mean age was 35 years (9-52 years). PE was performed in 7 (50.0%) of total 14 attacks. The target relapses were myelitis (n=4, 57.1%), optic neuritis (n=2,28.6%), and extensive cerebral lesions (n=1, 14.3%). The mean interval between attacks and PE was 9.3 days (4-20 days). The median NMO-IgG titer at PE onset was 1:480 (1:120-1:960). Six (85.7%) became negative for NMO-IgG after PE and the titer was lowered by 50% in the remainder.
The mean EDSS ± SD at PE onset was 6.4±2.1 and the mean decrease of EDSS was 1.7±1.5. Six (85.7%) showed functional improvement one month after PE.
Conclusions: Our study suggests that PE is an effective therapy for steroid-refractory attacks in NMO to enhance neurological recovery and prevent cumulative disabilities.
Journal of Multiple Sclerosis 1(1):16-19, 2010
