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KMID : 0391520160240040251
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Journal of the Korean Child Neurology Society
2016 Volume.24 No. 4 p.251 ~ p.256
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Opsoclonus-Myoclonus-Ataxia Syndrome in Children: Clinical Characteristics and Treatment Response
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Cho Jae-So
Woo Hye-Won
Kim Soo-Yeon
Choi Sun-Ah
Kim Hun-Min
Lim Byung-Chan
Hwang Hee
Choi Ji-Eun
Kim Ki-Joong
Chae Jong-Hee
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Abstract
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Purpose: Opsoclonus-Myoclonus-Ataxia syndrome (OMS) is a rare neurological disease associated with opsoclonus, myclonus, ataxia and behavioral changes. The purpose of this study was to investigate the clinical features of OMS and identify possible risk factors of unfavorable prognosis.
Methods: We retrospectively reviewed 15 patients who were diagnosed and treated as OMS at Seoul National University Children’s Hospital from May 2005-July 2015.
Results: 15 Patients were diagnosed and treated as OMS. The median ages at the disease onset were 20.9 months (range 13-28 months). The average time from symptom onset to treatment was 5 months (range 0-24 months). 4 patients (27%) were associated with neuroblastoma or ganglioneuroblastoma. The treatment for OMS included steroids, intravenous immunoglobulin and Rituximab and/or operation for underlying tumor. Ten patients received steroid for initial treatment and 2 patients (20%) showed complete response, 2 (20%) showed partial response and 6 (60%) showed no response. For final line treatment, Rituximab was used in 8 patients without side effects. Of the 8 patients, 6 (75%) showed complete response and 2 (25%) showed partial response. Patients who were treated 6 months after the symptom onset showed unfavorable response to the first line treatment (P<0.05). At the latest follow up, poor neurological outcomes were observed (P<0.05) in patients who started their first treatment 6 months after the symptom onset than those treated in early phase of the disease.
Conclusion: Early detection and treatment of OMS patient is crucial in favorable first line treatment response as well as neurological outcomes.
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KEYWORD
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Opsoclonus-myoclonus ataxia syndrome, OMS, Dancing eye syndrome, Rituximab, Prognostic factor, Treatment, Opsoclonus, Myoclonus, Ataxia, Neuroblastoma, Neurological sequelae
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