The rescue effect of plasma exchange for neuromyelitis optica

Abstract

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.