Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: A prospective study by the Children's Oncology Group

Patricia L. Robertson, Karin M. Muraszko, Emiko J. Holmes, Richard Sposto, Roger J. Packer, Amar Gajjar, Mark S. Dias, Jeffrey C. Allen

Research output: Contribution to journalArticle

204 Citations (Scopus)

Abstract

Object. Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology. Methods. The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients. Conclusions. Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.

Original languageEnglish (US)
Pages (from-to)444-451
Number of pages8
JournalJournal of neurosurgery
Volume105 PEDIATRICS
Issue numberSUPPL. 6
StatePublished - Dec 1 2006

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Mutism
Cerebellar Diseases
Medulloblastoma
Prospective Studies
Incidence
Ataxia
Brain Stem
Neoplasms
Cerebellar Neoplasms
Infratentorial Neoplasms
Brain Stem Neoplasms
Pediatrics
Primitive Neuroectodermal Tumors
Muscle Hypotonia
Language
Clinical Trials
Morbidity

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Cite this

Robertson, P. L., Muraszko, K. M., Holmes, E. J., Sposto, R., Packer, R. J., Gajjar, A., ... Allen, J. C. (2006). Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: A prospective study by the Children's Oncology Group. Journal of neurosurgery, 105 PEDIATRICS(SUPPL. 6), 444-451.
Robertson, Patricia L. ; Muraszko, Karin M. ; Holmes, Emiko J. ; Sposto, Richard ; Packer, Roger J. ; Gajjar, Amar ; Dias, Mark S. ; Allen, Jeffrey C. / Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma : A prospective study by the Children's Oncology Group. In: Journal of neurosurgery. 2006 ; Vol. 105 PEDIATRICS, No. SUPPL. 6. pp. 444-451.
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title = "Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: A prospective study by the Children's Oncology Group",
abstract = "Object. Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology. Methods. The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24{\%}) of 450 children. Symptom intensity was judged to have been severe in 43{\%}, moderate in 49{\%}, and mild in 8{\%} of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients. Conclusions. Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92{\%} were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.",
author = "Robertson, {Patricia L.} and Muraszko, {Karin M.} and Holmes, {Emiko J.} and Richard Sposto and Packer, {Roger J.} and Amar Gajjar and Dias, {Mark S.} and Allen, {Jeffrey C.}",
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Robertson, PL, Muraszko, KM, Holmes, EJ, Sposto, R, Packer, RJ, Gajjar, A, Dias, MS & Allen, JC 2006, 'Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: A prospective study by the Children's Oncology Group', Journal of neurosurgery, vol. 105 PEDIATRICS, no. SUPPL. 6, pp. 444-451.

Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma : A prospective study by the Children's Oncology Group. / Robertson, Patricia L.; Muraszko, Karin M.; Holmes, Emiko J.; Sposto, Richard; Packer, Roger J.; Gajjar, Amar; Dias, Mark S.; Allen, Jeffrey C.

In: Journal of neurosurgery, Vol. 105 PEDIATRICS, No. SUPPL. 6, 01.12.2006, p. 444-451.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma

T2 - A prospective study by the Children's Oncology Group

AU - Robertson, Patricia L.

AU - Muraszko, Karin M.

AU - Holmes, Emiko J.

AU - Sposto, Richard

AU - Packer, Roger J.

AU - Gajjar, Amar

AU - Dias, Mark S.

AU - Allen, Jeffrey C.

PY - 2006/12/1

Y1 - 2006/12/1

N2 - Object. Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology. Methods. The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients. Conclusions. Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.

AB - Object. Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology. Methods. The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients. Conclusions. Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.

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Robertson PL, Muraszko KM, Holmes EJ, Sposto R, Packer RJ, Gajjar A et al. Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: A prospective study by the Children's Oncology Group. Journal of neurosurgery. 2006 Dec 1;105 PEDIATRICS(SUPPL. 6):444-451.