B cell depletion therapy for new-onset opsoclonus-myoclonus

✍ Scribed by Michael R. Pranzatelli; Elizabeth D. Tate; Jennifer A. Swan; Anna L. Travelstead; Jerry A. Colliver; Steven J. Verhulst; Carl J. Crosley; William D. Graf; Suja A. Joseph; Howard M. Kelfer; G. Praveen Raju

• Book ID: 102945478
• Publisher: John Wiley and Sons
• Year: 2010
• Tongue: English
• Weight: 553 KB
• Volume: 25
• Category: Article
• ISSN: 0885-3185
• DOI: 10.1002/mds.22941

✦ Synopsis

Abstract

Twelve immunotherapy‐naïve children with opsoclonus‐myoclonus syndrome and CSF B cell expansion received rituximab, adrenocorticotropic hormone (ACTH), and IVIg. Motor severity lessened 73% by 6 mo and 81% at 1 yr (P < 0.0001). Opsoclonus and action myoclonus disappeared rapidly, whereas gait ataxia and some other motor components improved more slowly. ACTH dose was tapered by 87%. Reduction in total CSF B cells was profound at 6 mo (‐93%). By study end, peripheral B cells returned to 53% of baseline and serum IgM levels to 63%. Overall clinical response trailed peripheral B cell and IgM depletion, but improvement continued after their levels recovered. All but one non‐ambulatory subject became ambulatory without additional chemotherapy; two relapsed and remitted; four had rituximab‐related or possibly related adverse events; and two had low‐titer human anti‐chimeric antibody. Combination of rituximab with conventional agents as initial therapy was effective and safe. A controlled trial with long‐term safety monitoring is indicated. © 2009 Movement Disorder Society