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QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA
OBJECTIVE: Cerebellar mutism syndrome (CMS) is a severe neurological complication of tumour surgery in the posterior fossa in childhood. The incidence is reported between 8 and 39%, where CMS sets in within days of surgery and resolves within months, yet long-term sequelae are seen in most patients....
Autores principales: | , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7715299/ http://dx.doi.org/10.1093/neuonc/noaa222.712 |
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author | Grønbæk, Jonathan Kjær Wibroe, Morten Toescu, Sebastian Grillner, Pernilla Mallucci, Conor Molinari, Emanuela Solem, Kristin Westerholm-Ormio, Mia Kiudeliene, Rosita Mudra, Katalin Hauser, Peter van Baarsen, Kirsten Hoving, Eelco Zipfel, Julian Nysom, Karsten Schmiegelow, Kjeld Sehested, Astrid Juhler, Marianne Mathiasen, René |
author_facet | Grønbæk, Jonathan Kjær Wibroe, Morten Toescu, Sebastian Grillner, Pernilla Mallucci, Conor Molinari, Emanuela Solem, Kristin Westerholm-Ormio, Mia Kiudeliene, Rosita Mudra, Katalin Hauser, Peter van Baarsen, Kirsten Hoving, Eelco Zipfel, Julian Nysom, Karsten Schmiegelow, Kjeld Sehested, Astrid Juhler, Marianne Mathiasen, René |
author_sort | Grønbæk, Jonathan Kjær |
collection | PubMed |
description | OBJECTIVE: Cerebellar mutism syndrome (CMS) is a severe neurological complication of tumour surgery in the posterior fossa in childhood. The incidence is reported between 8 and 39%, where CMS sets in within days of surgery and resolves within months, yet long-term sequelae are seen in most patients. This prospective cohort study investigates the course of CMS and the surgical cause of the syndrome. POPULATION AND METHODS: We included 500 children with a tumour in the posterior fossa with planned surgery or open biopsy. Enrolment was conducted between 2014 and 2020 in 26 centres in ten European countries. Speech, neurological symptoms and surgical procedure were registered in predefined standardized forms pre-operatively and at three post-operative follow-ups within one year. PRELIMINARY RESULTS: A total of 426 children underwent primary surgery and were eligible for analyses. CMS occurred in 56 patients (13.1%) one day (median; IQR: 0–2 days) after surgery and resolved within 38 days (median; IQR: 4–52 days). Another 58 patients (13.6%) had less severe speech impairment. Mutism was associated with lower age (OR: 0.91 [95%CI: 0.85;0.98, p=0.014]), medulloblastoma (OR: 2.5 [95%CI: 1.4;4.7, p=0.0036]) and ATRT (OR: 12.9 [95%CI: 3.4;51.9, p=0.00018]) and tumour location in the fourth ventricle (OR: 4.0 [95%CI: 2.3;7.2, p<0.0001]). Preliminary multivariate analyses revealed no significant association between mutism and surgical access. CONCLUSION: CMS is a common complication predominantly seen in younger children after tumour surgery for a medulloblastoma or ATRT in the fourth ventricle. The incidence is not related to the surgical access in this study population. |
format | Online Article Text |
id | pubmed-7715299 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-77152992020-12-09 QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA Grønbæk, Jonathan Kjær Wibroe, Morten Toescu, Sebastian Grillner, Pernilla Mallucci, Conor Molinari, Emanuela Solem, Kristin Westerholm-Ormio, Mia Kiudeliene, Rosita Mudra, Katalin Hauser, Peter van Baarsen, Kirsten Hoving, Eelco Zipfel, Julian Nysom, Karsten Schmiegelow, Kjeld Sehested, Astrid Juhler, Marianne Mathiasen, René Neuro Oncol Neuropsychology/Quality of Life OBJECTIVE: Cerebellar mutism syndrome (CMS) is a severe neurological complication of tumour surgery in the posterior fossa in childhood. The incidence is reported between 8 and 39%, where CMS sets in within days of surgery and resolves within months, yet long-term sequelae are seen in most patients. This prospective cohort study investigates the course of CMS and the surgical cause of the syndrome. POPULATION AND METHODS: We included 500 children with a tumour in the posterior fossa with planned surgery or open biopsy. Enrolment was conducted between 2014 and 2020 in 26 centres in ten European countries. Speech, neurological symptoms and surgical procedure were registered in predefined standardized forms pre-operatively and at three post-operative follow-ups within one year. PRELIMINARY RESULTS: A total of 426 children underwent primary surgery and were eligible for analyses. CMS occurred in 56 patients (13.1%) one day (median; IQR: 0–2 days) after surgery and resolved within 38 days (median; IQR: 4–52 days). Another 58 patients (13.6%) had less severe speech impairment. Mutism was associated with lower age (OR: 0.91 [95%CI: 0.85;0.98, p=0.014]), medulloblastoma (OR: 2.5 [95%CI: 1.4;4.7, p=0.0036]) and ATRT (OR: 12.9 [95%CI: 3.4;51.9, p=0.00018]) and tumour location in the fourth ventricle (OR: 4.0 [95%CI: 2.3;7.2, p<0.0001]). Preliminary multivariate analyses revealed no significant association between mutism and surgical access. CONCLUSION: CMS is a common complication predominantly seen in younger children after tumour surgery for a medulloblastoma or ATRT in the fourth ventricle. The incidence is not related to the surgical access in this study population. Oxford University Press 2020-12-04 /pmc/articles/PMC7715299/ http://dx.doi.org/10.1093/neuonc/noaa222.712 Text en © The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Neuropsychology/Quality of Life Grønbæk, Jonathan Kjær Wibroe, Morten Toescu, Sebastian Grillner, Pernilla Mallucci, Conor Molinari, Emanuela Solem, Kristin Westerholm-Ormio, Mia Kiudeliene, Rosita Mudra, Katalin Hauser, Peter van Baarsen, Kirsten Hoving, Eelco Zipfel, Julian Nysom, Karsten Schmiegelow, Kjeld Sehested, Astrid Juhler, Marianne Mathiasen, René QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA |
title | QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA |
title_full | QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA |
title_fullStr | QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA |
title_full_unstemmed | QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA |
title_short | QOL-59. CEREBELLAR MUTISM SYNDROME AND THE SURGICAL RISK FACTORS: A PROSPECTIVE MULTICENTRE STUDY OF 500 PATIENTS UNDERGOING TUMOUR SURGERY IN THE POSTERIOR FOSSA |
title_sort | qol-59. cerebellar mutism syndrome and the surgical risk factors: a prospective multicentre study of 500 patients undergoing tumour surgery in the posterior fossa |
topic | Neuropsychology/Quality of Life |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7715299/ http://dx.doi.org/10.1093/neuonc/noaa222.712 |
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