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/m2 /day of DEX for three consecutive days) every 28 days for 6 months after resection of the tumor. All OMS symptoms improved after th...

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Veröffentlicht in:Brain & development (Tokyo. 1979) 2012-03, Vol.34 (3), p.251-254
Hauptverfasser: Oguma, Makiko, Morimoto, Akira, Takada, Akiko, Kashii, Yoshifumi, Fukuda, Tokiko, Mori, Masato, Yamagata, Takanori, Sugie, Hideo, Momoi, Mariko Y
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container_end_page 254
container_issue 3
container_start_page 251
container_title Brain & development (Tokyo. 1979)
container_volume 34
creator Oguma, Makiko
Morimoto, Akira
Takada, Akiko
Kashii, Yoshifumi
Fukuda, Tokiko
Mori, Masato
Yamagata, Takanori
Sugie, Hideo
Momoi, Mariko Y
description 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/m2 /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.
doi_str_mv 10.1016/j.braindev.2011.04.005
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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.</description><identifier>ISSN: 0387-7604</identifier><identifier>EISSN: 1872-7131</identifier><identifier>DOI: 10.1016/j.braindev.2011.04.005</identifier><identifier>PMID: 21531096</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Anti-Inflammatory Agents - administration &amp; dosage ; B-Lymphocytes - immunology ; Biomarkers - blood ; CD4-Positive T-Lymphocytes - immunology ; CD4/CD8 ratio ; CD8-Positive T-Lymphocytes - immunology ; Child, Preschool ; Dexamethasone ; Dexamethasone - administration &amp; dosage ; Humans ; Kidney Neoplasms - complications ; Kidney Neoplasms - immunology ; Male ; Neuroblastoma ; Neuroblastoma - complications ; Neuroblastoma - immunology ; Neurology ; Opsoclonus-Myoclonus Syndrome - drug therapy ; Opsoclonus-Myoclonus Syndrome - etiology ; Opsoclonus-Myoclonus Syndrome - immunology ; Opsoclonus–myoclonus syndrome</subject><ispartof>Brain &amp; development (Tokyo. 1979), 2012-03, Vol.34 (3), p.251-254</ispartof><rights>The Japanese Society of Child Neurology</rights><rights>2011 The Japanese Society of Child Neurology</rights><rights>Copyright © 2011 The Japanese Society of Child Neurology. 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subjects Anti-Inflammatory Agents - administration & dosage
B-Lymphocytes - immunology
Biomarkers - blood
CD4-Positive T-Lymphocytes - immunology
CD4/CD8 ratio
CD8-Positive T-Lymphocytes - immunology
Child, Preschool
Dexamethasone
Dexamethasone - administration & dosage
Humans
Kidney Neoplasms - complications
Kidney Neoplasms - immunology
Male
Neuroblastoma
Neuroblastoma - complications
Neuroblastoma - immunology
Neurology
Opsoclonus-Myoclonus Syndrome - drug therapy
Opsoclonus-Myoclonus Syndrome - etiology
Opsoclonus-Myoclonus Syndrome - immunology
Opsoclonus–myoclonus syndrome
title Another promising treatment option for neuroblastoma-associated opsoclonus–myoclonus syndrome by oral high-dose dexamethasone pulse: Lymphocyte markers as disease activity
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