Skip to main content
Log in

Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study

  • Original Article
  • Published:
The Cerebellum Aims and scope Submit manuscript

Abstract

The aim of this study was to explore the association between sex and cerebellar mutism syndrome and to examine other potential risk factors. This ambispective cohort study examined 218 pediatric patients (132 boys) with a posterior fossa tumor who underwent tumor resection from July 2013 to March 2021. The patients’ demographics and tumor characteristics were examined and statistically analyzed to explore the associations among the variables. Multivariable and subgroup analyses were conducted to validate the independent risk factors for cerebellar mutism syndrome (CMS). The male and female patients did not differ significantly in terms of age, tumor size, tumor location, tumor consistency, VP shunt placement before resection, extent of resection, or surgeon, as well as with respect to the presence of hydrocephalus or paraventricular edema. The overall incidence of CMS was 32.6%. The incidence of CMS was significantly higher in male patients than that in female patients (41.7% vs. 18.6%; P = 0.001). In the multivariable analysis, male sex (adjusted odds ratio [OR], 3.27; P = 0.001), solid tumor consistency (adjusted OR, 5.61; P = 0.001), midline location (adjusted OR, 3.78; P = 0.004), and hydrocephalus (adjusted OR, 2.56; P = 0.047) were independent risk factors for the CMS. Chi-square analysis revealed that solid tumor consistency and midline location were associated with medulloblastoma (P < 0.001). Male patients had a higher risk of developing CMS after a posterior fossa tumor resection. Midline location, solid tumor consistency, and hydrocephalus were independent risk factors for CMS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Data Availability

Data is available at request.

References

  1. Gudrunardottir T, Morgan AT, Lux AL, Walker DA, Walsh KS, Wells EM, et al. Consensus paper on post-operative pediatric cerebellar mutism syndrome: the Iceland Delphi results. Childs Nerv Syst. 2016;32:1195–203.

    Article  PubMed  Google Scholar 

  2. Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K. Cerebellar mutism: review of the literature. Childs Nerv Syst. 2011;27:355–63.

    Article  PubMed  Google Scholar 

  3. Khan RB, Patay Z, Klimo P, Huang J, Kumar R, Boop FA, et al. Clinical features, neurologic recovery, and risk factors of postoperative posterior fossa syndrome and delayed recovery: a prospective study. Neuro Oncol. 2021;23:1586–96.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Morris EB, Phillips NS, Laningham FH, Patay Z, Gajjar A, Wallace D, et al. Proximal dentatothalamocortical tract involvement in posterior fossa syndrome. Brain. 2009;132:3087–95.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Grønbæk J, Molinari E, Avula S, Wibroe M, Oettingen G, Juhler M. The supplementary motor area syndrome and the cerebellar mutism syndrome: a pathoanatomical relationship? Childs Nerv Syst. 2020;36:1197–204.

    Article  PubMed  Google Scholar 

  6. Palesi F, Tournier JD, Calamante F, Muhlert N, Castellazzi G, Chard D, et al. Contralateral cerebello-thalamo-cortical pathways with prominent involvement of associative areas in humans in vivo. Brain Struct Funct. 2015;220:3369–84.

    Article  PubMed  Google Scholar 

  7. Palesi F, De Rinaldis A, Castellazzi G, Calamante F, Muhlert N, Chard D, et al. Contralateral cortico-ponto-cerebellar pathways reconstruction in humans in vivo: implications for reciprocal cerebro-cerebellar structural connectivity in motor and non-motor areas. Sci Rep. 2017;7:12841.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Marien P, Manto M. Cerebellum as a master-piece for linguistic predictability. Cerebellum. 2018;17:101–3.

    Article  CAS  PubMed  Google Scholar 

  9. Guell X, Gabrieli JDE, Schmahmann JD. Triple representation of language, working memory, social and emotion processing in the cerebellum: convergent evidence from task and seed-based resting-state fmri analyses in a single large cohort. Neuroimage. 2018;172:437–49.

    Article  PubMed  Google Scholar 

  10. 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. J Neurosurg. 2006;105:444.

    PubMed  Google Scholar 

  11. Kupeli S, Yalcin B, Bilginer B, Akalan N, Haksal P, Buyukpamukcu M. Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatr Blood Cancer. 2011;56:206–10.

    Article  PubMed  Google Scholar 

  12. Toescu SM, Hales PW, Aquilina K, Clark CA. Quantitative MRI in post-operative paediatric cerebellar mutism syndrome. Eur J Radiol. 2018;108:43–51.

    Article  PubMed  Google Scholar 

  13. Bakhshi SK, Mitha R, Mushtaq N, Shamim MS. Cerebellar mutism syndrome after surgical resection of posterior fossa neoplastic lesions. J Pak Med Assoc. 2020;70:1667–8.

    PubMed  Google Scholar 

  14. Ashida R, Nazar N, Edwards R, Teo M. Cerebellar mutism syndrome: an overview of the pathophysiology in relation to the cerebrocerebellar anatomy, risk factors, potential treatments, and outcomes. World Neurosurg. 2021;153:63–74.

    Article  PubMed  Google Scholar 

  15. Renne B, Radic J, Agrawal D, Albrecht B, Bonfield CM, Cohrs G, et al. Cerebellar mutism after posterior fossa tumor resection in children: a multicenter international retrospective study to determine possible modifiable factors. Childs Nerv Syst. 2020;36:1159–69.

    Article  PubMed  Google Scholar 

  16. Wibroe M, Cappelen J, Castor C, Clausen N, Grillner P, Gudrunardottir T, et al. Cerebellar mutism syndrome in children with brain tumours of the posterior fossa. BMC Cancer. 2017;17:439.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Catsman-Berrevoets CE. Cerebellar mutism syndrome: cause and rehabilitation. Curr Opin Neurol. 2017;30:133–9.

    Article  PubMed  Google Scholar 

  18. Cámara S, Fournier MC, Cordero P, Melero J, Robles F, Esteso B, et al. Neuropsychological profile in children with posterior fossa tumors with or without postoperative cerebellar mutism syndrome (cms). Cerebellum. 2020;19:78–88.

    Article  PubMed  Google Scholar 

  19. Jabarkheel R, Amayiri N, Yecies D, Huang Y, Toescu S, Nobre L, et al. Molecular correlates of cerebellar mutism syndrome in medulloblastoma. Neuro Oncol. 2020;22:290–7.

    PubMed  Google Scholar 

  20. Gora NK, Gupta A, Sinha VD. Cerebellar mutism syndrome following midline posterior fossa tumor resection in children: an institutional experience. J Pediatr Neurosci. 2017;12:313–9.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Tomasi D, Volkow ND. Laterality patterns of brain functional connectivity: gender effects. Cereb Cortex. 2012;22:1455–62.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Dinga S, Wu D, Huang S, Wu C, Wang X, Shi J, et al. Neuromagnetic correlates of audiovisual word processing in the developing brain. Int J Psychophysiol. 2018;128:7–21.

    Article  PubMed  Google Scholar 

  23. Yu VY, MacDonald MJ, Oh A, Hua GN, De Nil LF, Pang EW. Age-related sex differences in language lateralization: a magnetoencephalography study in children. Dev Psychol. 2014;50:2276–84.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Adani S, Cepanec M. Sex differences in early communication development: behavioral and neurobiological indicators of more vulnerable communication system development in boys. Croat Med J. 2019;60:141–9.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Narzisi A, Posada M, Barbieri F, Chericoni N, Ciuffolini D, Pinzino M, et al. Prevalence of autism spectrum disorder in a large Italian catchment area: a school-based population study within the ASDEU project. Epidemiol Psychiatr Sci. 2018;29:e5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Kogan MD, Vladutiu CJ, Schieve LA, Ghandour RM, Blumberg SJ, Zablotsky B, et al. The prevalence of parent-reported autism spectrum disorder among us children. Pediatrics. 2018;142,6:e20174161.

  27. Pettersson SD, Kitlinski M, Miekisiak G, Ali S, Krakowiak M, Szmuda T. Risk factors for postoperative cerebellar mutism syndrome in pediatric patients: a systematic review and meta-analysis. J Neurosurg Pediatr. 2022;29:467–75.

    Article  PubMed  Google Scholar 

  28. Lange BP, Euler HA, Zaretsky E. Sex differences in language competence of 3- to 6-year-old children. Appl Psycholinguist. 2016;37:1417–38.

    Article  Google Scholar 

  29. Ozcaliskan S, Goldin-Meadow S. Sex differences in language first appear in gesture. Dev Sci. 2010;13:752–60.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Pettersson SD, Kitlinski M, Miękisiak G, Ali S, Krakowiak M, Szmuda T. Risk factors for postoperative cerebellar mutism syndrome in pediatric patients: a systematic review and meta-analysis. J Neurosurg: Pediatr. 2021;5:1–9.

    Google Scholar 

  31. Gronbaek JK, Wibroe M, Toescu S, Fric R, Thomsen BL, Moller LN, et al. Postoperative speech impairment and surgical approach to posterior fossa tumours in children: a prospective european multicentre cohort study. Lancet Child Adolesc Health. 2021;5:814–24.

    Article  PubMed  Google Scholar 

  32. Cao F, Brennan C, Booth JR. The brain adapts to orthography with experience: evidence from English and Chinese. Dev Sci. 2015;18:785–98.

    Article  PubMed  Google Scholar 

  33. Ip KI, Marks RA, Hsu LS, Desai N, Kuan JL, Tardif T, et al. Morphological processing in Chinese engages left temporal regions. Brain Lang. 2019;199:104696.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Lou Y, Zhao L, Yu S, Sun B, Hou Z, Zhang Z, et al. Brain asymmetry differences between Chinese and Caucasian populations: a surface-based morphometric comparison study. Brain Imaging Behav. 2020;14:2323–32.

    Article  PubMed  Google Scholar 

  35. Doxey D, Bruce D, Sklar F, Swift D, Shapiro K. Posterior fossa syndrome: identifiable risk factors and irreversible complications. Pediatr Neurosurg. 1999;31:131–6.

    Article  CAS  PubMed  Google Scholar 

  36. Law N, Greenberg M, Bouffet E, Taylor MD, Laughlin S, Strother D, et al. Clinical and neuroanatomical predictors of cerebellar mutism syndrome. Neuro Oncol. 2012;14:1294–303.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Pols S, van Veelen M, Aarsen FK, Gonzalez CA, Catsman-Berrevoets CE. Risk factors for development of postoperative cerebellar mutism syndrome in children after medulloblastoma surgery. J Neurosurg Pediatr. 2017;20:35–41.

    Article  PubMed  Google Scholar 

  38. Tamburrini G, Frassanito P, Chieffo D, Massimi L, Caldarelli M, Di Rocco C. Cerebellar mutism. Childs Nerv Syst. 2015;31:1841–51.

    Article  CAS  PubMed  Google Scholar 

  39. Toescu SM, Samarth G, Layard HH, Issitt R, Margetts B, Phipps KP, et al. Fourth ventricle tumors in children: complications and influence of surgical approach. J Neurosurg Pediatr. 2020;27:52–61.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Professor Yaguang Peng, who guided us in the statistical analysis.

Funding

This study was funded by Beijing Hospital’s Authority Clinical Medicine Development of Special Funding (code: XMLX202144).

Author information

Authors and Affiliations

Authors

Contributions

Conceptualization: Wei Yang, Ming Ge

Methodology: Wei Yang, Kaiyi Zhu

Formal analysis and investigation: Jiashu Chen, Ping Yang, Yingjie Cai, Xiaojiao Peng, Jia Wang

Writing–original draft preparation: Wei Yang

Writing–review and editing: Wei Yang, Kaiyi Zhu

Funding acquisition: Ming Ge

Supervision: Ming Ge

Resources: Hailang Sun, Yuanqi Ji, Fengmao Zhao, Hong Zhang

Corresponding author

Correspondence to Ming Ge.

Ethics declarations

Conflict of Interest

The authors declare no competing interests.

Ethics Approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of Beijing Children’s Hospital (Beijing, China, grant number: [2021]-E-024-Y).

Consent to Participate

Informed consent was obtained from all individual participants included in the study.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Yang, W., Ge, M., Zhu, K. et al. Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study. Cerebellum 22, 730–738 (2023). https://doi.org/10.1007/s12311-022-01449-6

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12311-022-01449-6

Keywords

Navigation