A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children

Japanese Society for Pediatric Immune-mediated Brain Diseases

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Background: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease characterized by opsoclonus, myoclonus, ataxia, and behavioral changes. The aim of our study was to investigate the epidemiological characteristics of OMS in Japan and to clarify the association between therapy and prognosis. Methods: We retrospectively collected the data from 626 Japanese medical institutions from 2005 to 2010, and analyzed the clinical features of pediatric patients with OMS based on the data. Results: In this survey, there were 23 patients (10 males and 13 females). The median ages at the disease onset and the time of study were 16.5. months (range: 11-152. months) and 54. months (range: 24-160. months), respectively. The principal symptoms were opsoclonus (23 patients, 100%), myoclonus (21 patients, 91.3%), and ataxia (23 patients, 100%). The related factors were neuroblastoma (10, 43.5%), infection (9, 39.1%), and immunization (2, 8.7%). The treatments for OMS were included intravenous immunoglobulin (17, 73.9%), methylprednisolone pulse (13, 56.5%), oral prednisolone (12 patients, 52.2%), and chemotherapy and/or operation for the underlying tumors (6, 26.1%), and rituximab (2, 8.7%). Complete remissions were obtained in 35.3%, 23.1%, 33.3%, 66.7%, and 100% of these treatments, respectively. At the latest follow-up period, 8 (34.8%) and 17 patients (73.9%) showed neurological sequelae of motor and intellectual functions, respectively. Patients whose treatment was started more than 30. weeks after the disease onset suffered from the severest neurological sequelae (OMS severity 4) more frequently than those less than 30. weeks (. p=. 0.022). Conclusion: The annual incidence of OMS was estimated to be 0.27-0.40 cases per million in Japanese children. More than 70% of OMS patients had neurological sequelae, especially intellectual function. Early effective treatments within 30. weeks after the onset may be required to prevent the serious neurological outcome.

Original languageEnglish
Pages (from-to)656-660
Number of pages5
JournalBrain and Development
Volume37
Issue number7
DOIs
Publication statusPublished - Aug 1 2015

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Opsoclonus-Myoclonus Syndrome
Ocular Motility Disorders
Therapeutics
Surveys and Questionnaires
Myoclonus
Time and Motion Studies
Intravenous Immunoglobulins
Methylprednisolone
Ataxia
Rare Diseases
Prednisolone
Neuroblastoma
Age of Onset
Immunization
Japan

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

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A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children. / Japanese Society for Pediatric Immune-mediated Brain Diseases.

In: Brain and Development, Vol. 37, No. 7, 01.08.2015, p. 656-660.

Research output: Contribution to journalArticle

Japanese Society for Pediatric Immune-mediated Brain Diseases 2015, 'A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children', Brain and Development, vol. 37, no. 7, pp. 656-660. https://doi.org/10.1016/j.braindev.2014.10.010
Japanese Society for Pediatric Immune-mediated Brain Diseases. / A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children. In: Brain and Development. 2015 ; Vol. 37, No. 7. pp. 656-660.
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abstract = "Background: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease characterized by opsoclonus, myoclonus, ataxia, and behavioral changes. The aim of our study was to investigate the epidemiological characteristics of OMS in Japan and to clarify the association between therapy and prognosis. Methods: We retrospectively collected the data from 626 Japanese medical institutions from 2005 to 2010, and analyzed the clinical features of pediatric patients with OMS based on the data. Results: In this survey, there were 23 patients (10 males and 13 females). The median ages at the disease onset and the time of study were 16.5. months (range: 11-152. months) and 54. months (range: 24-160. months), respectively. The principal symptoms were opsoclonus (23 patients, 100{\%}), myoclonus (21 patients, 91.3{\%}), and ataxia (23 patients, 100{\%}). The related factors were neuroblastoma (10, 43.5{\%}), infection (9, 39.1{\%}), and immunization (2, 8.7{\%}). The treatments for OMS were included intravenous immunoglobulin (17, 73.9{\%}), methylprednisolone pulse (13, 56.5{\%}), oral prednisolone (12 patients, 52.2{\%}), and chemotherapy and/or operation for the underlying tumors (6, 26.1{\%}), and rituximab (2, 8.7{\%}). Complete remissions were obtained in 35.3{\%}, 23.1{\%}, 33.3{\%}, 66.7{\%}, and 100{\%} of these treatments, respectively. At the latest follow-up period, 8 (34.8{\%}) and 17 patients (73.9{\%}) showed neurological sequelae of motor and intellectual functions, respectively. Patients whose treatment was started more than 30. weeks after the disease onset suffered from the severest neurological sequelae (OMS severity 4) more frequently than those less than 30. weeks (. p=. 0.022). Conclusion: The annual incidence of OMS was estimated to be 0.27-0.40 cases per million in Japanese children. More than 70{\%} of OMS patients had neurological sequelae, especially intellectual function. Early effective treatments within 30. weeks after the onset may be required to prevent the serious neurological outcome.",
author = "{Japanese Society for Pediatric Immune-mediated Brain Diseases} and Shunji Hasegawa and Takeshi Matsushige and Madoka Kajimoto and Hirofumi Inoue and Hiroshi Momonaka and Momoko Oka and Shouchi Ohga and Takashi Ichiyama and T. Naito and N. Azuma and T. Tsuru and A. Ohba and N. Sugiyama and H. Matsuura and T. Yamaue and K. Furuhashi and S. Yamashita and T. Shiihara and N. Ishikawa and H. Torisu and S. Suenobu and T. Yamagata and H. Kawawaki and K. Fujii and Y. Fukuyama and H. Aiba and A. Oka and T. Kishi and R. Kira and S. Kimura and M. Kubota and J. Takanashi and Y. Takahashi and M. Takayanagi and H. Tamai and J. Natsume and S. Hamano and T. Hara and S. Hirabayashi and Y. Maegaki and M. Matsuo and M. Mizuguchi and K. Minagawa and H. Yoshikawa",
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TY - JOUR

T1 - A nationwide survey of opsoclonus-myoclonus syndrome in Japanese children

AU - Japanese Society for Pediatric Immune-mediated Brain Diseases

AU - Hasegawa, Shunji

AU - Matsushige, Takeshi

AU - Kajimoto, Madoka

AU - Inoue, Hirofumi

AU - Momonaka, Hiroshi

AU - Oka, Momoko

AU - Ohga, Shouchi

AU - Ichiyama, Takashi

AU - Naito, T.

AU - Azuma, N.

AU - Tsuru, T.

AU - Ohba, A.

AU - Sugiyama, N.

AU - Matsuura, H.

AU - Yamaue, T.

AU - Furuhashi, K.

AU - Yamashita, S.

AU - Shiihara, T.

AU - Ishikawa, N.

AU - Torisu, H.

AU - Suenobu, S.

AU - Yamagata, T.

AU - Kawawaki, H.

AU - Fujii, K.

AU - Fukuyama, Y.

AU - Aiba, H.

AU - Oka, A.

AU - Kishi, T.

AU - Kira, R.

AU - Kimura, S.

AU - Kubota, M.

AU - Takanashi, J.

AU - Takahashi, Y.

AU - Takayanagi, M.

AU - Tamai, H.

AU - Natsume, J.

AU - Hamano, S.

AU - Hara, T.

AU - Hirabayashi, S.

AU - Maegaki, Y.

AU - Matsuo, M.

AU - Mizuguchi, M.

AU - Minagawa, K.

AU - Yoshikawa, H.

PY - 2015/8/1

Y1 - 2015/8/1

N2 - Background: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease characterized by opsoclonus, myoclonus, ataxia, and behavioral changes. The aim of our study was to investigate the epidemiological characteristics of OMS in Japan and to clarify the association between therapy and prognosis. Methods: We retrospectively collected the data from 626 Japanese medical institutions from 2005 to 2010, and analyzed the clinical features of pediatric patients with OMS based on the data. Results: In this survey, there were 23 patients (10 males and 13 females). The median ages at the disease onset and the time of study were 16.5. months (range: 11-152. months) and 54. months (range: 24-160. months), respectively. The principal symptoms were opsoclonus (23 patients, 100%), myoclonus (21 patients, 91.3%), and ataxia (23 patients, 100%). The related factors were neuroblastoma (10, 43.5%), infection (9, 39.1%), and immunization (2, 8.7%). The treatments for OMS were included intravenous immunoglobulin (17, 73.9%), methylprednisolone pulse (13, 56.5%), oral prednisolone (12 patients, 52.2%), and chemotherapy and/or operation for the underlying tumors (6, 26.1%), and rituximab (2, 8.7%). Complete remissions were obtained in 35.3%, 23.1%, 33.3%, 66.7%, and 100% of these treatments, respectively. At the latest follow-up period, 8 (34.8%) and 17 patients (73.9%) showed neurological sequelae of motor and intellectual functions, respectively. Patients whose treatment was started more than 30. weeks after the disease onset suffered from the severest neurological sequelae (OMS severity 4) more frequently than those less than 30. weeks (. p=. 0.022). Conclusion: The annual incidence of OMS was estimated to be 0.27-0.40 cases per million in Japanese children. More than 70% of OMS patients had neurological sequelae, especially intellectual function. Early effective treatments within 30. weeks after the onset may be required to prevent the serious neurological outcome.

AB - Background: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disease characterized by opsoclonus, myoclonus, ataxia, and behavioral changes. The aim of our study was to investigate the epidemiological characteristics of OMS in Japan and to clarify the association between therapy and prognosis. Methods: We retrospectively collected the data from 626 Japanese medical institutions from 2005 to 2010, and analyzed the clinical features of pediatric patients with OMS based on the data. Results: In this survey, there were 23 patients (10 males and 13 females). The median ages at the disease onset and the time of study were 16.5. months (range: 11-152. months) and 54. months (range: 24-160. months), respectively. The principal symptoms were opsoclonus (23 patients, 100%), myoclonus (21 patients, 91.3%), and ataxia (23 patients, 100%). The related factors were neuroblastoma (10, 43.5%), infection (9, 39.1%), and immunization (2, 8.7%). The treatments for OMS were included intravenous immunoglobulin (17, 73.9%), methylprednisolone pulse (13, 56.5%), oral prednisolone (12 patients, 52.2%), and chemotherapy and/or operation for the underlying tumors (6, 26.1%), and rituximab (2, 8.7%). Complete remissions were obtained in 35.3%, 23.1%, 33.3%, 66.7%, and 100% of these treatments, respectively. At the latest follow-up period, 8 (34.8%) and 17 patients (73.9%) showed neurological sequelae of motor and intellectual functions, respectively. Patients whose treatment was started more than 30. weeks after the disease onset suffered from the severest neurological sequelae (OMS severity 4) more frequently than those less than 30. weeks (. p=. 0.022). Conclusion: The annual incidence of OMS was estimated to be 0.27-0.40 cases per million in Japanese children. More than 70% of OMS patients had neurological sequelae, especially intellectual function. Early effective treatments within 30. weeks after the onset may be required to prevent the serious neurological outcome.

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