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The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review
Background and Objectives: The opsoclonus-myoclonus syndrome (OMS) is characterised by opsoclons, myoclons and impaired balance, often concomitant with sleep disorder and behavioural difficulties. The symptoms develop as a result of autoimmune response triggered by a neuroblastic tumour (NT). OMS ca...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7466285/ https://www.ncbi.nlm.nih.gov/pubmed/32823831 http://dx.doi.org/10.3390/medicina56080412 |
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author | Mizia-Malarz, Agnieszka Stolpa, Weronika Sobol-Milejska, Grażyna |
author_facet | Mizia-Malarz, Agnieszka Stolpa, Weronika Sobol-Milejska, Grażyna |
author_sort | Mizia-Malarz, Agnieszka |
collection | PubMed |
description | Background and Objectives: The opsoclonus-myoclonus syndrome (OMS) is characterised by opsoclons, myoclons and impaired balance, often concomitant with sleep disorder and behavioural difficulties. The symptoms develop as a result of autoimmune response triggered by a neuroblastic tumour (NT). OMS can also develop secondarily to a viral infection or as an immune response triggered by an unknown agent. This leads to the activation of B- and T-cells, which produce and release autoantibodies or cytokines directly within the central nervous system (CNS), thus damaging the neurons within the cerebellum and the brain stem. The available OMS treatments aim at decreasing lymphocyte, cytokine and autoantibody production or accelerating the utilisation of the latter. Another treatment option for OMS involves using cytostatic agents, which damage T- and B-cells causing their depletion and impaired function, which reduces their ability to produce antibodies and cytokines. Materials and Methods: We present a single-centre experience in treating OMS secondary to NT in 7 children. Results: The combined treatment with cyclophosphamide plus dexamethasone resulted in a complete resolution of OMS symptoms in 4 children, and a significant improvement in the 3 children. Two of them periodically present hyperactivity, and one girl requires an additional support at school due to special educational needs (SEN). Conclusions: NT resection does not resolve OMS in children with OMS secondary to NT. The combined treatment with dexamethasone plus cyclophosphamide seems to be an effective treatment of OMS. |
format | Online Article Text |
id | pubmed-7466285 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-74662852020-09-14 The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review Mizia-Malarz, Agnieszka Stolpa, Weronika Sobol-Milejska, Grażyna Medicina (Kaunas) Article Background and Objectives: The opsoclonus-myoclonus syndrome (OMS) is characterised by opsoclons, myoclons and impaired balance, often concomitant with sleep disorder and behavioural difficulties. The symptoms develop as a result of autoimmune response triggered by a neuroblastic tumour (NT). OMS can also develop secondarily to a viral infection or as an immune response triggered by an unknown agent. This leads to the activation of B- and T-cells, which produce and release autoantibodies or cytokines directly within the central nervous system (CNS), thus damaging the neurons within the cerebellum and the brain stem. The available OMS treatments aim at decreasing lymphocyte, cytokine and autoantibody production or accelerating the utilisation of the latter. Another treatment option for OMS involves using cytostatic agents, which damage T- and B-cells causing their depletion and impaired function, which reduces their ability to produce antibodies and cytokines. Materials and Methods: We present a single-centre experience in treating OMS secondary to NT in 7 children. Results: The combined treatment with cyclophosphamide plus dexamethasone resulted in a complete resolution of OMS symptoms in 4 children, and a significant improvement in the 3 children. Two of them periodically present hyperactivity, and one girl requires an additional support at school due to special educational needs (SEN). Conclusions: NT resection does not resolve OMS in children with OMS secondary to NT. The combined treatment with dexamethasone plus cyclophosphamide seems to be an effective treatment of OMS. MDPI 2020-08-14 /pmc/articles/PMC7466285/ /pubmed/32823831 http://dx.doi.org/10.3390/medicina56080412 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Mizia-Malarz, Agnieszka Stolpa, Weronika Sobol-Milejska, Grażyna The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review |
title | The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review |
title_full | The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review |
title_fullStr | The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review |
title_full_unstemmed | The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review |
title_short | The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours—Single-Centre Experience and Literature Review |
title_sort | treatment of opsoclonus-myoclonus syndrome secondary to neuroblastic tumours—single-centre experience and literature review |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7466285/ https://www.ncbi.nlm.nih.gov/pubmed/32823831 http://dx.doi.org/10.3390/medicina56080412 |
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