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.
N1 - Publisher Copyright:
© 2014 The Japanese Society of Child Neurology.
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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U2 - 10.1016/j.braindev.2014.10.010
DO - 10.1016/j.braindev.2014.10.010
M3 - Article
C2 - 25454391
AN - SCOPUS:84931082954
SN - 0387-7604
VL - 37
SP - 656
EP - 660
JO - Brain and Development
JF - Brain and Development
IS - 7
ER -