Cerebellar mutism risk factors



Proven risk factors include brain stem invasion, diagnosis of medulloblastoma, midline localization, tumor size, invasion of the fourth ventricle, invasion of the superior cerebellar peduncle, left-handedness, and incision of the vermis 1)


For Grønbæk et al. data do not support the hypothesis that handedness should be of clinical relevance in the risk assessment of CMS 2).


Findings enhance an already hypothesized role of cerebellar language lateralization 3).


Risk factors for post-op cerebellar mutism following surgery for medulloblastoma in children: brainstem involvement & midline location 4).

Postoperative cerebellar mutism has been observed in ≈ 1% of adults following posterior fossa surgery 5)


Male sex and cerebro-cerebellar circuit damage are independent risk factors for pCMS. The cerebro-cerebellar circuit score indicates the duration of mutism 6)

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 7).


Chua et al., performed a clinical study that evaluated possible risk factors for permanent PFS in paediatric medulloblastoma patients. Analysis of collated results found that post-operative DWI restriction in bilateral regions within the surgical cavity demonstrated statistical significance as a predictor of PFS permanence-a novel finding in the current literatura 8).


Children who receive treatment for medulloblastoma have a high survival rate, but also a high likelihood of developing posterior fossa syndrome.

Diffusion abnormalities were identified in 10 cases, 7 of which involved the proximal efferent cerebellar pathway (pECP). A retrospective evaluation revealed evidence of PFS in 6 cases. There was a significant association between abnormalities involving pECP structures (P = .001) and the development of PFS. Bilateral involvement of pECP (P = .006) was a highly specific risk factor for predicting the development of PFS. Diffusion abnormality of the inferior vermis was significantly associated with PFS (P = .001) but may not represent a risk factor in isolation 9).


Infratentorial glioblastoma recurrence is a severe recurrence type in GBM patients. Its symptoms are neurologically unspecific and can be overlooked or misdiagnosed as side effects of treatments. Carefully checking the infratentorial region, especially around the fourth ventricle, is essential during the GBM patient follow-up. The primary symptoms of ITR were gait disturbance (100%, n = 6), dizziness (50.0%, n = 3), nausea (33.3%, n = 2), and cerebellar mutism (16.7%, n = 1). In four cases (66.7%), symptoms were presented before ITR development 10)

1)
Fabozzi F, Margoni S, Andreozzi B, Musci MS, Del Baldo G, Boccuto L, Mastronuzzi A, Carai A. Cerebellar mutism syndrome: From pathophysiology to rehabilitation. Front Cell Dev Biol. 2022 Dec 2;10:1082947. doi: 10.3389/fcell.2022.1082947. PMID: 36531947; PMCID: PMC9755514.
2)
Grønbæk JK, Laustsen AF, Toescu S, Pizer B, Mallucci C, Aquilina K, Molinari E, Hjort MA, Gumbeleviciene L, Hauser P, Pálmafy B, van Baarsen K, Hoving E, Zipfel J, Ehrstedt C, Grillner P, Callesen MT, Frič R, Wibroe M, Nysom K, Schmiegelow K, Sehested A, Mathiasen R, Juhler M; CMS study group. Left-handedness should not be overrated as a risk factor for postoperative speech impairment in children after posterior fossa tumour surgery: a prospective European multicentre study. Childs Nerv Syst. 2022 Aug;38(8):1479-1485. doi: 10.1007/s00381-022-05567-8. Epub 2022 Jun 27. PMID: 35759029.
3)
de Laurentis C, Cristaldi PMF, Rebora P, Valsecchi MG, Biassoni V, Schiavello E, Carrabba GG, Trezza A, DiMeco F, Ferroli P, Cinalli G, Locatelli M, Cenzato M, Talamonti G, Fontanella MM, Spena G, Stefini R, Bernucci C, Bellocchi S, Locatelli D, Massimino M, Giussani C. Posterior fossa syndrome in a population of children and young adults with medulloblastoma: a retrospective, multicenter Italian study on incidence and pathophysiology in a histologically homogeneous and consecutive series of 136 patients. J Neurooncol. 2022 Sep;159(2):377-387. doi: 10.1007/s11060-022-04072-x. Epub 2022 Jun 29. PMID: 35767101.
4)
Pitsika M, Tsitouras V. Cerebellar mutism. J Neurosurg Pediatr. 2013; 12:604–614
5)
Dubey A, Sung WS, Shaya M, et al. Complications of posterior cranial fossa surgery–an institutional experience of 500 patients. Surg Neurol. 2009; 72: 369–375
6)
Yang W, Zhang H, Cai Y, Peng X, Sun H, Chen J, Ying Z, Zhu K, Peng Y, Ge M. Postoperative MRI features of cerebellar mutism syndrome: a retrospective cohort study. J Neurosurg Pediatr. 2022 Oct 7;30(6):567-577. doi: 10.3171/2022.8.PEDS22294. PMID: 36208442.
7)
Yang W, Ge M, Zhu K, Chen J, Yang P, Cai Y, Peng X, Wang J, Sun H, Ji Y, Zhao F, Zhang H. Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study. Cerebellum. 2022 Jul 23. doi: 10.1007/s12311-022-01449-6. Epub ahead of print. PMID: 35870083.
8)
Chua FH, Thien A, Ng LP, Seow WT, Low DC, Chang KT, Lian DW, Loh E, Low SY. Post-operative diffusion weighted imaging as a predictor of posterior fossa syndrome permanence in paediatric medulloblastoma. Childs Nerv Syst. 2017 Feb 11. doi: 10.1007/s00381-017-3356-7. [Epub ahead of print] PubMed PMID: 28190209.
9)
Avula S, Kumar R, Pizer B, Pettorini B, Abernethy L, Garlick D, Mallucci C. Diffusion abnormalities on intraoperative magnetic resonance imaging as an early predictor for the risk of posterior fossa syndrome. Neuro Oncol. 2015 Apr;17(4):614-22. doi: 10.1093/neuonc/nou299. Epub 2014 Oct 15. PubMed PMID: 25319997.
10)
Kawauchi D, Ohno M, Honda-Kitahara M, Miyakita Y, Takahashi M, Yanagisawa S, Tamura Y, Kikuchi M, Ichimura K, Narita Y. Clinical characteristics and prognosis of Glioblastoma patients with infratentorial recurrence. BMC Neurol. 2023 Jan 7;23(1):9. doi: 10.1186/s12883-022-03047-9. PMID: 36609238; PMCID: PMC9824996.