title: Another promising treatment option for neuroblastoma-associated opsoclonus-myoclonus syndrome by oral high-dose dexamethasone pulse: lymphocyte markers as disease activity.
abstract: A one-year-old boy with neuroblastoma (NBoma)-associated opsoclonus-myoclonus syndrome (OMS) was treated by oral high-dose dexamethasone (DEX) pulses (20 mg/m(2)/day of DEX for three consecutive days) every 28 days for 6 months after resection of the tumor. All OMS symptoms improved after the first course of DEX pulse therapy and disappeared after the last course. No adverse effects were observed. Minor deterioration of his developmental quotient was noted 33 months after the onset of the disease. NBoma remission has been maintained since treatment. Before DEX pulse therapy, frequency of T lymphocyte, in particular CD4-positive cell decreased markedly resulted in low CD4/8 ratio in the peripheral blood (PB). The frequency of B lymphocyte increased, especially in cerebrospinal fluid. These aberrant values in PB were reversed by DEX pulse therapy and correlated well with the neurological symptoms. A prospective study that assesses the efficacy of this promising and inexpensive treatment for OMS is warranted.
authors.affiliation: Department of Pediatrics, Jichi Medical University School of Medicine, Tochigi 329-0498, Japan.
authors.forename: Makiko
authors.initials: M
authors.lastname: Oguma
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
authors.forename: Akiko
authors.initials: A
authors.lastname: Takada
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Kashii
authors.forename: Tokiko
authors.initials: T
authors.lastname: Fukuda
authors.forename: Masato
authors.initials: M
authors.lastname: Mori
authors.forename: Takanori
authors.initials: T
authors.lastname: Yamagata
authors.forename: Hideo
authors.initials: H
authors.lastname: Sugie
authors.forename: Mariko Y
authors.initials: MY
authors.lastname: Momoi
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D001402:B-Lymphocytes / Q000276:immunology; D015415:Biomarkers / Q000097:blood*; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology; D002675:Child, Preschool; D003907:Dexamethasone / Q000008:administration & dosage*; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000276:immunology; D008297:Male; D009447:Neuroblastoma / Q000150:complications / Q000276:immunology; D053578:Opsoclonus-Myoclonus Syndrome / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology
chemical_list: D000893:Anti-Inflammatory Agents; D015415:Biomarkers; D003907:Dexamethasone