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The Cerebellar Mutism Syndrome : Risk Assessment, Prevention and Treatment

Grønbæk, Jonathan Kjær ; Boeg Thomsen, Ditte LU ; Persson, Karin LU orcid ; Mathiasen, René and Juhler, Marianne (2023) In Advances and technical standards in neurosurgery 46. p.65-94
Abstract

Cerebellar mutism syndrome (CMS) has received increasing attention over the last decades as a complication of posterior fossa tumour surgery in children. Risk factors, aetiological aspects, and treatment measures of the syndrome have been investigated, yet the incidence of CMS remains unchanged. Overall, we are currently able to identify patients at risk, but we are unable to prevent it from occurring.Once CMS sets in, several symptomatic pharmacological treatments have been suggested, but only in smaller case series and not in randomized controlled trials, and it is not clear whether the treatment or time itself had a helpful effect.Within weeks to months, most patients regain their ability to speak after a phase with mutism or... (More)

Cerebellar mutism syndrome (CMS) has received increasing attention over the last decades as a complication of posterior fossa tumour surgery in children. Risk factors, aetiological aspects, and treatment measures of the syndrome have been investigated, yet the incidence of CMS remains unchanged. Overall, we are currently able to identify patients at risk, but we are unable to prevent it from occurring.Once CMS sets in, several symptomatic pharmacological treatments have been suggested, but only in smaller case series and not in randomized controlled trials, and it is not clear whether the treatment or time itself had a helpful effect.Within weeks to months, most patients regain their ability to speak after a phase with mutism or severely reduced speech; however, many patients continue to have speech and language deficits. At this point, anti-cancer treatment with chemotherapy and radiotherapy may be of focus more than the prognosis of CMS; however, many patients continue to have speech and language problems for months and years to come, and they are at high risk of other neurocognitive sequelae as well.Without reliable measures to prevent or treat the syndrome, we may look towards improving the prognosis of speech and neurocognitive functioning in these patients. As speech and language impairment is the cardinal symptom and late effect of CMS, the effect of intense and early-onset speech and language therapy as a standard of care in these patients should be investigated in relation to its effect on regaining speech capacity.

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Please use this url to cite or link to this publication:
author
; ; ; and
organization
publishing date
type
Chapter in Book/Report/Conference proceeding
publication status
published
subject
keywords
Child, Humans, Mutism/diagnosis, Cerebellar Diseases/diagnosis, Brain Neoplasms/complications, Infratentorial Neoplasms/complications, Risk Assessment, Syndrome, Disease Progression, Postoperative Complications/diagnosis
host publication
Advances and Technical Standards in Neurosurgery
series title
Advances and technical standards in neurosurgery
volume
46
pages
65 - 94
external identifiers
  • pmid:37318570
ISSN
0095-4829
ISBN
978-3-031-28202-7
978-3-031-28204-1
DOI
10.1007/978-3-031-28202-7_4
language
English
LU publication?
yes
additional info
© 2023. The Author(s), under exclusive license to Springer Nature Switzerland AG.
id
b82ce43c-36e2-4fc2-9626-4a8ba0459f16
date added to LUP
2025-04-23 10:51:36
date last changed
2025-04-24 03:28:04
@inbook{b82ce43c-36e2-4fc2-9626-4a8ba0459f16,
  abstract     = {{<p>Cerebellar mutism syndrome (CMS) has received increasing attention over the last decades as a complication of posterior fossa tumour surgery in children. Risk factors, aetiological aspects, and treatment measures of the syndrome have been investigated, yet the incidence of CMS remains unchanged. Overall, we are currently able to identify patients at risk, but we are unable to prevent it from occurring.Once CMS sets in, several symptomatic pharmacological treatments have been suggested, but only in smaller case series and not in randomized controlled trials, and it is not clear whether the treatment or time itself had a helpful effect.Within weeks to months, most patients regain their ability to speak after a phase with mutism or severely reduced speech; however, many patients continue to have speech and language deficits. At this point, anti-cancer treatment with chemotherapy and radiotherapy may be of focus more than the prognosis of CMS; however, many patients continue to have speech and language problems for months and years to come, and they are at high risk of other neurocognitive sequelae as well.Without reliable measures to prevent or treat the syndrome, we may look towards improving the prognosis of speech and neurocognitive functioning in these patients. As speech and language impairment is the cardinal symptom and late effect of CMS, the effect of intense and early-onset speech and language therapy as a standard of care in these patients should be investigated in relation to its effect on regaining speech capacity.</p>}},
  author       = {{Grønbæk, Jonathan Kjær and Boeg Thomsen, Ditte and Persson, Karin and Mathiasen, René and Juhler, Marianne}},
  booktitle    = {{Advances and Technical Standards in Neurosurgery}},
  isbn         = {{978-3-031-28202-7}},
  issn         = {{0095-4829}},
  keywords     = {{Child; Humans; Mutism/diagnosis; Cerebellar Diseases/diagnosis; Brain Neoplasms/complications; Infratentorial Neoplasms/complications; Risk Assessment; Syndrome; Disease Progression; Postoperative Complications/diagnosis}},
  language     = {{eng}},
  pages        = {{65--94}},
  series       = {{Advances and technical standards in neurosurgery}},
  title        = {{The Cerebellar Mutism Syndrome : Risk Assessment, Prevention and Treatment}},
  url          = {{http://dx.doi.org/10.1007/978-3-031-28202-7_4}},
  doi          = {{10.1007/978-3-031-28202-7_4}},
  volume       = {{46}},
  year         = {{2023}},
}