Treatment of Steroid Unresponsive Relapse With Plasma Exchange in Aggressive Multiple Sclerosis Mario Habek, 1 Barbara Barun, 1 Zvonimir Puretic ´, 2 and Vesna V Brinar 1 1 Referral Center for Demyelinating Diseases of the Central Nervous System, University Department of Neurology, Zagreb School of Medicine and University Hospital Center, and 2 Department of Dialysis, University Hospital Center Zagreb, Zagreb, Croatia Abstract: The options for treating steroid unresponsive relapses in relapsing remitting multiple sclerosis (RRMS) are modest. We present a small series of patients with an aggressive course of RRMS whose steroid unresponsive relapses were treated with plasma exchange. In the period from January 2007 until February 2009 we identified four patients with steroid unresponsive relapses. All recorded relapses were treated with methylprednisolone, either with 500 mg for 5 days or 1000 mg for 3 days. If there was no improvement, patients were given five cycles of plasma exchange. If there was no recovery after the initial five cycles, five more were administered. Each patient’s clinical status was monitored using the extended disability status scale. The median time from symptom onset until starting plasma exchange was 30 days (23–45 days). For four relapses, five cycles of plasma exchange were given with marked recovery in one, moderate in two, and mild in one case. In one patient, after five cycles there was no recovery, so five more cycles were administered, after which a mod- erate recovery ensued. This study further supports the efficacy of plasma exchange in the treatment of steroid unresponsive relapses in aggressive RRMS. Key Words: Aggressive, Multiple sclerosis, Plasma exchange, Relapse. Aggressive or malignant relapsing–remitting mul- tiple sclerosis (RRMS) is a rare variant of RRMS characterized by: very frequent (4/year) and severe (extended disability status scale [EDSS]  6.0) relapses; a short duration of the total disease or of the aggressive disease (<1.5 years); and clearly docu- mented recent improvement periods (1). Treatment modalities in such cases are modest, and there is much debate in the literature as to whether plasma exchange has a role in the treatment of steroid unre- sponsive RRMS relapses.We present a small series of patients with an aggressive form of RRMS whose steroid unresponsive relapses were treated with plasma exchange. PATIENTS AND METHODS During the period from January 2007 until Febru- ary 2009 we identified four patients with steroid unre- sponsive relapses. All recorded relapses were treated with methylprednisolone, either with 500 mg for 5 days or 1000 mg for 3 days. A steroid unresponsive relapse is defined as a relapse without reduction of at least one point in the EDSS seven days after stopping corticosteroids. When this criterion was met, and if there were no contraindications, patients were given five cycles of plasma exchange. If there was no recov- ery after the initial five cycles, five more were admin- istered. Their clinical status was monitored using the EDSS. The plasma exchange characteristics were as follows: plasma filter TPE 2000 (Gambro, Bratislava, Slovakia) or MPS05 (Bellco, Mirandola, Italy); blood pump flow rate 60–100 mL/min; reinfusion flow rate 20–30 mL/min; filtrate flow rate 20–30 mL/min. All patients were given heparin as an initial bolus of 2500 U and an infusion of 1000 U/h, and were Received August 2009; revised September 2009. Address correspondence and reprint requests to Dr Mario Habek, MD, Klinika za neurologiju, Medicinskog fakulteta u Zagrebu, KBC Zagreb, Kišpatic ´eva 12, HR-10000 Zagreb, Croatia. Tel: +38598883323; Fax: +38512388045; Email: mhabek@mef.hr Therapeutic Apheresis and Dialysis 14(3):298–302 doi: 10.1111/j.1744-9987.2009.00794.x © 2010 The Authors Journal compilation © 2010 International Society for Apheresis 298