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Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India

BACKGROUND: Opsoclonus myoclonus syndrome (OMS) is a neuroinflammatory disorder. Indian literature on its clinical profile and outcome is sparse. OBJECTIVES: The objective of this study is to describe the clinical profile and analyze outcomes and prognostic predictors in a cohort of children with OM...

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Autores principales: Muthusamy, Karthik, Thomas, Maya, Yoganathan, Sangeetha, Sudhakar, Sniya Valsa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6613420/
https://www.ncbi.nlm.nih.gov/pubmed/31359941
http://dx.doi.org/10.4103/aian.AIAN_101_18
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author Muthusamy, Karthik
Thomas, Maya
Yoganathan, Sangeetha
Sudhakar, Sniya Valsa
author_facet Muthusamy, Karthik
Thomas, Maya
Yoganathan, Sangeetha
Sudhakar, Sniya Valsa
author_sort Muthusamy, Karthik
collection PubMed
description BACKGROUND: Opsoclonus myoclonus syndrome (OMS) is a neuroinflammatory disorder. Indian literature on its clinical profile and outcome is sparse. OBJECTIVES: The objective of this study is to describe the clinical profile and analyze outcomes and prognostic predictors in a cohort of children with OMS. MATERIALS AND METHODS: This was a retrospective study of children with OMS between 2007 and 2017. RESULTS: Twenty-two children were included in the study. The mean age at onset of symptom was 20.9 months (standard deviation [SD]: 7.5). The mean duration of delay in diagnosis was 8.4 months (SD 1.26) with acute cerebellitis being the most common misdiagnosis. Eleven children (50%) were diagnosed with tumor during evaluation and follow-up and 11 children (50%) belonged to idiopathic/postinfectious group. Magnetic resonance imaging brain was normal in all children except for one revealing cerebellar atrophy on follow-up. One child in the paraneoplastic group (neuroblastoma) had a positive PNMA2/Ta onconeural antibody. Children in the tumor group had an earlier age of onset (mean 15.5 vs. 26.3 months), shorter time to onset of opsoclonus from initial symptom (2.54 vs. 7.27 weeks), and higher severity score at presentation (13.7 vs. 11.3) compared to the nontumor group. Children in the nontumor group attained their first remission with treatment earlier (10.9 weeks, SD: 4.5) than the children with tumor (18.72 weeks, SD: 5.8). There was no significant difference in the outcome between the groups. Children with multiple relapses (>3) and late surgical intervention for tumor (>6 months after symptom onset) had a poor outcome. DISCUSSION: A high index of suspicion coupled with early diagnosis and periodic tumor surveillance (even in the initially negative cases) along with aggressive combined multimechanistic immunotherapies is the key in improving outcomes. CONCLUSION: A high index of suspicion in appropriate clinical circumstances and early aggressive immunomodulation might lead to a better outcome.
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spelling pubmed-66134202019-07-29 Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India Muthusamy, Karthik Thomas, Maya Yoganathan, Sangeetha Sudhakar, Sniya Valsa Ann Indian Acad Neurol Original Article BACKGROUND: Opsoclonus myoclonus syndrome (OMS) is a neuroinflammatory disorder. Indian literature on its clinical profile and outcome is sparse. OBJECTIVES: The objective of this study is to describe the clinical profile and analyze outcomes and prognostic predictors in a cohort of children with OMS. MATERIALS AND METHODS: This was a retrospective study of children with OMS between 2007 and 2017. RESULTS: Twenty-two children were included in the study. The mean age at onset of symptom was 20.9 months (standard deviation [SD]: 7.5). The mean duration of delay in diagnosis was 8.4 months (SD 1.26) with acute cerebellitis being the most common misdiagnosis. Eleven children (50%) were diagnosed with tumor during evaluation and follow-up and 11 children (50%) belonged to idiopathic/postinfectious group. Magnetic resonance imaging brain was normal in all children except for one revealing cerebellar atrophy on follow-up. One child in the paraneoplastic group (neuroblastoma) had a positive PNMA2/Ta onconeural antibody. Children in the tumor group had an earlier age of onset (mean 15.5 vs. 26.3 months), shorter time to onset of opsoclonus from initial symptom (2.54 vs. 7.27 weeks), and higher severity score at presentation (13.7 vs. 11.3) compared to the nontumor group. Children in the nontumor group attained their first remission with treatment earlier (10.9 weeks, SD: 4.5) than the children with tumor (18.72 weeks, SD: 5.8). There was no significant difference in the outcome between the groups. Children with multiple relapses (>3) and late surgical intervention for tumor (>6 months after symptom onset) had a poor outcome. DISCUSSION: A high index of suspicion coupled with early diagnosis and periodic tumor surveillance (even in the initially negative cases) along with aggressive combined multimechanistic immunotherapies is the key in improving outcomes. CONCLUSION: A high index of suspicion in appropriate clinical circumstances and early aggressive immunomodulation might lead to a better outcome. Medknow Publications & Media Pvt Ltd 2019 /pmc/articles/PMC6613420/ /pubmed/31359941 http://dx.doi.org/10.4103/aian.AIAN_101_18 Text en Copyright: © 2006 - 2019 Annals of Indian Academy of Neurology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Muthusamy, Karthik
Thomas, Maya
Yoganathan, Sangeetha
Sudhakar, Sniya Valsa
Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India
title Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India
title_full Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India
title_fullStr Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India
title_full_unstemmed Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India
title_short Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India
title_sort clinical profile, prognostic indicators, and therapeutic outcomes of pediatric opsoclonus-myoclonus-ataxia syndrome: a single-center experience from south india
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6613420/
https://www.ncbi.nlm.nih.gov/pubmed/31359941
http://dx.doi.org/10.4103/aian.AIAN_101_18
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