B cell depletion therapy for new-onset opsoclonus-myoclonus

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

Research output: Contribution to journalArticlepeer-review

50 Scopus citations


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 antichimeric antibody. Combination of rituximab with conventional agents as initial therapy was effective and safe. A controlled trial with long-term safety monitoring is indicated.

Original languageEnglish (US)
Pages (from-to)238-242
Number of pages5
JournalMovement Disorders
Issue number2
StatePublished - Jan 30 2010


  • ACTH
  • Anti-B cell agent
  • Dancing eyes
  • Kinsbourne syndrome
  • Neuroblastoma
  • Paraneoplastic syndrome
  • Rituximab

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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