Neuromyelitis optica (NMO) is an uncommon idiopathic demyelinating disease of the central nervous system and is sometimes unresponsive to steroid treatment as compared to multiple sclerosis (MS). There are only a few reports of plasma exchange (PE) as an effective rescue treatment when high-dose steroid therapy fails in exacerbations of NMO. Thus, we aimed to evaluate the efficacy of PE for acute attacks of NMO that failed to respond to high-dose steroid therapy. A retrospective review and clinical follow-up were conducted in two hospitals from January 2001 to January 2008. We recruited patients with NMO who had failed to respond to high-dose steroid treatment, and who then received PE during an acute relapse. We evaluated a global functional assessment of the change in the neurological condition, and the Expanded Disability Status Scale (EDSS) score. All nine patients were middle-aged women (mean age: 48.7 years old), five of whom tested positive for anti-aquaporin (AQP)-4 antibodies in the study. The patients were severely disabled at the initiation of PE (median EDSS score, 8.7; range, 8.5-9.0). Improvement occurred early in the course of PE. At the 2-month post-PE follow-up, eight of nine patients had improved to their pre-attack condition. This study highlights the potential role of PE as a rescue therapy in the management of steroid-unresponsive acute attacks of NMO, especially in patients with auto-antibodies against AQP-4.
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