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Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome

Cognitive and acquired neurodevelopmental deficits have been reported in children with opsoclonus–myoclonus syndrome (OMS) and are known to be associated with more severe and relapsing disease course. However, there is a paucity of data regarding cognitive dysfunction in children with stable neurolo...

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Autores principales: Goh, En Lin, Scarff, Kate, Satariano, Stephanie, Lim, Ming, Anand, Geetha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7552772/
https://www.ncbi.nlm.nih.gov/pubmed/32824925
http://dx.doi.org/10.3390/children7090103
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author Goh, En Lin
Scarff, Kate
Satariano, Stephanie
Lim, Ming
Anand, Geetha
author_facet Goh, En Lin
Scarff, Kate
Satariano, Stephanie
Lim, Ming
Anand, Geetha
author_sort Goh, En Lin
collection PubMed
description Cognitive and acquired neurodevelopmental deficits have been reported in children with opsoclonus–myoclonus syndrome (OMS) and are known to be associated with more severe and relapsing disease course. However, there is a paucity of data regarding cognitive dysfunction in children with stable neurological disease. We report three children with OMS and evolving cognitive dysfunction in the context of a mild disease course. The children’s ages at disease onset were between 17 and 35 months and they were followed up for 4–10 years. Neuroblastoma was identified in one child. OMS severity scores ranged between 8 and 12/15 at presentation. They underwent immunotherapy and all were in remission by 7 months (range 4–13 months), with treatment maintained for 1 year. One child remained relapse-free, while two others had one clinical relapse each and were immunotherapy-responsive again. In all cases, evolving cognitive dysfunction was reported despite being in remission and stable off treatment for a median of 20 months (range of 12–31 months; two OMS scores of 0/15 and one of 2/15). In children with OMS who have completed treatment and have made full or near full neurological recovery, concerns remain regarding long-term outcome in terms of future learning and cognitive development.
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spelling pubmed-75527722020-10-19 Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome Goh, En Lin Scarff, Kate Satariano, Stephanie Lim, Ming Anand, Geetha Children (Basel) Article Cognitive and acquired neurodevelopmental deficits have been reported in children with opsoclonus–myoclonus syndrome (OMS) and are known to be associated with more severe and relapsing disease course. However, there is a paucity of data regarding cognitive dysfunction in children with stable neurological disease. We report three children with OMS and evolving cognitive dysfunction in the context of a mild disease course. The children’s ages at disease onset were between 17 and 35 months and they were followed up for 4–10 years. Neuroblastoma was identified in one child. OMS severity scores ranged between 8 and 12/15 at presentation. They underwent immunotherapy and all were in remission by 7 months (range 4–13 months), with treatment maintained for 1 year. One child remained relapse-free, while two others had one clinical relapse each and were immunotherapy-responsive again. In all cases, evolving cognitive dysfunction was reported despite being in remission and stable off treatment for a median of 20 months (range of 12–31 months; two OMS scores of 0/15 and one of 2/15). In children with OMS who have completed treatment and have made full or near full neurological recovery, concerns remain regarding long-term outcome in terms of future learning and cognitive development. MDPI 2020-08-19 /pmc/articles/PMC7552772/ /pubmed/32824925 http://dx.doi.org/10.3390/children7090103 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
Goh, En Lin
Scarff, Kate
Satariano, Stephanie
Lim, Ming
Anand, Geetha
Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
title Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
title_full Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
title_fullStr Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
title_full_unstemmed Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
title_short Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
title_sort evolving cognitive dysfunction in children with neurologically stable opsoclonus–myoclonus syndrome
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7552772/
https://www.ncbi.nlm.nih.gov/pubmed/32824925
http://dx.doi.org/10.3390/children7090103
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