Insights on chronic-relapsing opsoclonus-myoclonus from a pilot study of mycophenolate mofetil.
Publication/Presentation Date
3-1-2009
Abstract
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.
Volume
24
Issue
3
First Page
316
Last Page
322
ISSN
1708-8283
Published In/Presented At
Pranzatelli, M. R., Tate, E. D., Travelstead, A. L., Baumgardner, C. A., Gowda, N. V., Halthore, S. N., Kerstan, P., Kossak, B. D., Mitchell, W. G., & Taub, J. W. (2009). Insights on chronic-relapsing opsoclonus-myoclonus from a pilot study of mycophenolate mofetil. Journal of child neurology, 24(3), 316–322. https://doi.org/10.1177/0883073808324217
Disciplines
Medicine and Health Sciences | Pediatrics
PubMedID
19258290
Department(s)
Department of Pediatrics
Document Type
Article