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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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Autores principales: Mizia-Malarz, Agnieszka, Stolpa, Weronika, Sobol-Milejska, Grażyna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
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.
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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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