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Insights on Chronic-Relapsing Opsoclonus-Myoclonus From a Pilot Study of Mycophenolate Mofetil

National Pediatric Myoclonus Center and Department of Neurology, mpranzatelli{at}siumed.edu

Elizabeth D. Tate, MN, C-FNP

National Pediatric Myoclonus Center and Department of Neurology

Anna L. Travelstead, BS

Flow Cytometry Facility Southern Illinois University School of Medicine, Springfield, Illinois

Christine A. Baumgardner, MD

OSF Medical Group-Galesburg Galesburg, Illinois

Narayana V. Gowda, MD

Department of Pediatric Hematology/Oncology St Mary's Hospital, West Palm Beach, Florida

Sri N. Halthore, MD

Department of Pediatrics University of Nevada School of Medicine, Las Vegas, Nevada

Peter Kerstan, MD

Medical and Surgical Associates Park Ridge, Illinois

Brian D. Kossak, MD

Department of Neurology Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire

Wendy G. Mitchell, MD

Children's Hospital Los Angeles/Neurology Division and Keck School of Medicine, University of Southern California, Los Angeles, California

Jeffrey W. Taub, MD

Department of Pediatrics Division of Pediatric Hematology/ Oncology, Children's Hospital of Michigan/Wayne State University, Detroit, Michigan

Opsoclonus-myoclonus syndrome is characterized by abnormal lymphocyte trafficking into brain. The authors hypothesized that mycophenolate mofetil, a lymphocyte proliferation inhibitor, might be therapeutic. The cerebrospinal fluid and blood immunophenotypes of 15 children with predominantly chronic-relapsing opsoclonus-myoclonus syndrome were compared before and after treatment by flow cytometry. Mycophenolate mofetil reduced the cerebrospinal fluid expansion of HLA-DR+ activated T cells (—40%); the frequency of other T-cell or natural killer cell subsets remained unchanged, but cerebrospinal fluid B cells increased significantly. Adrenocorticotropic hormone dose was lowered by 64% over an average of 1.5 years, yet 73% eventually relapsed despite therapeutic drug levels. Prior treatment with rituximab prevented relapse-associated increase in cerebrospinal fluid B cells, without hindering mycophenolate mofetil—induced reduction in T-cell activation. These data demonstrate resistant immunologic problems in chronic-relapsing opsoclonus-myoclonus syndrome. Mycophenolate mofetil did not prevent relapse. The novel effect of mycophenolate mofetil on chronically activated T cells may contribute to its efficacy in T-cell mediated neurological disorders.

Key Words: cerebrospinal fluid immunophenotyping • Kinsbourne syndrome • neuroblastoma • paraneoplastic syndrome

Journal of Child Neurology, Vol. 24, No. 3, 316-322 (2009)
DOI: 10.1177/0883073808324217


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