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Radiology of post-operative paediatric cerebellar mutism syndrome

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Abstract

Background

Post-operative paediatric cerebellar mutism syndrome is a well-recognized complication following posterior fossa tumour resection in children. Over the past few decades, imaging has played an important role in understanding this disorder.

Aim

This review article aims to focus on the disorder from a radiological perspective, summarizing the salient radiological evidence related to the anatomical structures, pathophysiology, and risk factors related to this disorder.

Conclusion

Radiological studies have been integral to the improved understanding of this condition. Future large multicentre studies and quantitative analysis techniques will be vital in further refinement of our understanding of this complex condition.

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References

  1. Albazron F BJ, Jones R, Yock T, Pulsifer M, Abrams A, Sato M, Boes A (2018) Lesion localization in posterior fossa syndrome. Paper presented at the 18th International Symposium on Pediatric Neuro-oncology (ISPNO 2018) Denver, Colorado, USA, 22 June 2018

  2. Avula S, Kumar R, Pizer B, Pettorini B, Abernethy L, Garlick D, Mallucci C (2015) Diffusion abnormalities on intraoperative magnetic resonance imaging as an early predictor for the risk of posterior fossa syndrome. Neuro-oncology 17:614–622. https://doi.org/10.1093/neuonc/nou299

    Article  PubMed  Google Scholar 

  3. Avula S, Mallucci C, Kumar R, Pizer B (2015) Posterior fossa syndrome following brain tumour resection: review of pathophysiology and a new hypothesis on its pathogenesis. Childs Nerv Syst 31:1859–1867. https://doi.org/10.1007/s00381-015-2797-0

    Article  PubMed  Google Scholar 

  4. Avula S SM, Guillemaut J, Windridge D, Kumar R, Pizer B, Mallucci C, Lewis E (2018) Postoperative paediatric cerebellar mutism syndrome-identifying quantitative biomarkers on longitudinal follow-up MRI. Paper presented at the International Symposium on Pediatric Neuro-Oncology. Denver, USA, June 2018

  5. Avula S, Spiteri M, Kumar R, Lewis E, Harave S, Windridge D, Ong C, Pizer B (2016) Post-operative pediatric cerebellar mutism syndrome and its association with hypertrophic olivary degeneration. Quant Imaging Med Surg 6:535–544. https://doi.org/10.21037/qims.2016.10.11

    Article  PubMed  PubMed Central  Google Scholar 

  6. Bazille C, Megarbane B, Bensimhon D, Lavergne-Slove A, Baglin AC, Loirat P, Woimant F, Mikol J, Gray F (2005) Brain damage after heat stroke. J Neuropathol Exp Neurol 64:970–975

    Article  PubMed  Google Scholar 

  7. Catsman-Berrevoets CE, Van Dongen HR, Mulder PG, Paz y Geuze D, Paquier PF, Lequin MH (1999) Tumour type and size are high risk factors for the syndrome of “cerebellar” mutism and subsequent dysarthria. J Neurol Neurosurg Psychiatry 67:755–757

    Article  CAS  PubMed  Google Scholar 

  8. Chua FHZ, Thien A, Ng LP, Seow WT, Low DCY, Chang KTE, Lian DWQ, Loh E, Low SYY (2017) Post-operative diffusion weighted imaging as a predictor of posterior fossa syndrome permanence in paediatric medulloblastoma. Childs Nerv Syst 33:457–465. https://doi.org/10.1007/s00381-017-3356-7

    Article  PubMed  Google Scholar 

  9. Flamm ES, Ransohoff J, Wuchinich D, Broadwin A (1978) Preliminary experience with ultrasonic aspiration in neurosurgery. Neurosurgery 2:240–245

    Article  CAS  PubMed  Google Scholar 

  10. Fujioka Y, Yasui K, Hasegawa Y, Takahashi A, Sobue G (2009) An acute severe heat stroke patient showing abnormal diffuse high intensity of the cerebellar cortex in diffusion weighted image: a case report. Rinsho shinkeigaku = Clinical neurology 49:634–640

    Article  PubMed  Google Scholar 

  11. Fushimi Y, Taki H, Kawai H, Togashi K (2012) Abnormal hyperintensity in cerebellar efferent pathways on diffusion-weighted imaging in a patient with heat stroke. Clin Radiol 67:389–392. https://doi.org/10.1016/j.crad.2011.09.009

    Article  CAS  PubMed  Google Scholar 

  12. Germano A, Baldari S, Caruso G, Caffo M, Montemagno G, Cardia E, Tomasello F (1998) Reversible cerebral perfusion alterations in children with transient mutism after posterior fossa surgery. Childs Nerv Syst 14:114–119. https://doi.org/10.1007/s003810050191

    Article  CAS  PubMed  Google Scholar 

  13. Goyal M, Versnick E, Tuite P, Cyr JS, Kucharczyk W, Montanera W, Willinsky R, Mikulis D (2000) Hypertrophic olivary degeneration: metaanalysis of the temporal evolution of MR findings. AJNR Am J Neuroradiol 21:1073–1077

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Gudrunardottir T, Morgan AT, Lux AL, Walker DA, Walsh KS, Wells EM, Wisoff JH, Juhler M, Schmahmann JD, Keating RF, Catsman-Berrevoets C (2016) Consensus paper on post-operative pediatric cerebellar mutism syndrome: the Iceland Delphi results. Childs Nerv Syst. https://doi.org/10.1007/s00381-016-3093-3

    Article  PubMed  Google Scholar 

  15. Gudrunardottir T, Sehested A, Juhler M, Schmiegelow K (2011) Cerebellar mutism: review of the literature. Childs Nerv Syst 27:355–363. https://doi.org/10.1007/s00381-010-1328-2

    Article  PubMed  Google Scholar 

  16. Hirsch JF, Renier D, Czernichow P, Benveniste L, Pierre-Kahn A (1979) Medulloblastoma in childhood. Survival and functional results. Acta Neurochir 48:1–15

    Article  CAS  PubMed  Google Scholar 

  17. Jabre A, Symon L, Richards PG, Redmond S (1985) Mean hemispheral cerebral blood flow changes after craniotomy. Significance and prognostic value. Acta Neurochir 78:13–20

    Article  CAS  PubMed  Google Scholar 

  18. Kim J, Lee SK, Lee JD, Kim YW, Kim DI (2005) Decreased fractional anisotropy of middle cerebellar peduncle in crossed cerebellar diaschisis: diffusion-tensor imaging-positron-emission tomography correlation study. AJNR Am J Neuroradiol 26:2224–2228

    PubMed  PubMed Central  Google Scholar 

  19. Korah MP, Esiashvili N, Mazewski CM, Hudgins RJ, Tighiouart M, Janss AJ, Schwaibold FP, Crocker IR, Curran WJ Jr, Marcus RB Jr (2010) Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol Biol Phys 77:106–112. https://doi.org/10.1016/j.ijrobp.2009.04.058

    Article  PubMed  Google Scholar 

  20. Kotil K, Eras M, Akcetin M, Bilge T (2008) Cerebellar mutism following posterior fossa tumor resection in children. Turk neurosurg 18:89–94

    PubMed  Google Scholar 

  21. Kusano Y, Tanaka Y, Takasuna H, Wada N, Tada T, Kakizawa Y, Hongo K (2006) Transient cerebellar mutism caused by bilateral damage to the dentate nuclei after the second posterior fossa surgery. Case report. J Neurosurg 104:329–331. https://doi.org/10.3171/jns.2006.104.2.329

    Article  PubMed  Google Scholar 

  22. Lal V, Sardana V, Thussu A, Sawhney IM, Prabhakar S (1997) Cerebellar degeneration following neuroleptic malignant syndrome. Postgrad Med J 73:735–736

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Law N, Greenberg M, Bouffet E, Taylor MD, Laughlin S, Strother D, Fryer C, McConnell D, Hukin J, Kaise C, Wang F, Mabbott DJ (2012) Clinical and neuroanatomical predictors of cerebellar mutism syndrome. Neuro-oncology 14:1294–1303. https://doi.org/10.1093/neuonc/nos160

    Article  PubMed  PubMed Central  Google Scholar 

  24. Lee JS, Choi JC, Kang SY, Kang JH, Park JK (2009) Heat stroke: increased signal intensity in the bilateral cerebellar dentate nuclei and splenium on diffusion-weighted MR imaging. AJNR Am J Neuroradiol 30:E58. https://doi.org/10.3174/ajnr.A1432

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Lee S, Merriam A, Kim TS, Liebling M, Dickson DW, Moore GR (1989) Cerebellar degeneration in neuroleptic malignant syndrome: neuropathologic findings and review of the literature concerning heat-related nervous system injury. J Neurol Neurosurg Psychiatry 52:387–391

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Liu JF, Dineen RA, Avula S, Chambers T, Dutta M, Jaspan T, MacArthur DC, Howarth S, Soria D, Quinlan P, Harave S, Ong CC, Mallucci CL, Kumar R, Pizer B, Walker DA (2018) Development of a pre-operative scoring system for predicting risk of post-operative paediatric cerebellar mutism syndrome. Br J Neurosurg 32:18–27. https://doi.org/10.1080/02688697.2018.1431204

    Article  PubMed  Google Scholar 

  27. McEvoy SD, Lee A, Poliakov A, Friedman S, Shaw D, Browd SR, Ellenbogen RG, Ojemann JG, Mac Donald CL (2016) Longitudinal cerebellar diffusion tensor imaging changes in posterior fossa syndrome. NeuroImage Clinical 12:582–590. https://doi.org/10.1016/j.nicl.2016.09.007

    Article  PubMed  PubMed Central  Google Scholar 

  28. McMillan HJ, Keene DL, Matzinger MA, Vassilyadi M, Nzau M, Ventureyra EC (2009) Brainstem compression: a predictor of postoperative cerebellar mutism. Childs Nerv Syst 25:677–681. https://doi.org/10.1007/s00381-008-0777-3

    Article  CAS  PubMed  Google Scholar 

  29. Miller NG, Reddick WE, Kocak M, Glass JO, Lobel U, Morris B, Gajjar A, Patay Z (2010) Cerebellocerebral diaschisis is the likely mechanism of postsurgical posterior fossa syndrome in pediatric patients with midline cerebellar tumors. AJNR Am J Neuroradiol 31:288–294. https://doi.org/10.3174/ajnr.A1821

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Morris EB, Phillips NS, Laningham FH, Patay Z, Gajjar A, Wallace D, Boop F, Sanford R, Ness KK, Ogg RJ (2009) Proximal dentatothalamocortical tract involvement in posterior fossa syndrome. Brain: a journal of neurology 132:3087–3095. https://doi.org/10.1093/brain/awp241

    Article  Google Scholar 

  31. Ojemann JG, Partridge SC, Poliakov AV, Niazi TN, Shaw DW, Ishak GE, Lee A, Browd SR, Geyer JR, Ellenbogen RG (2013) Diffusion tensor imaging of the superior cerebellar peduncle identifies patients with posterior fossa syndrome. Childs Nerv Syst 29:2071–2077. https://doi.org/10.1007/s00381-013-2205-6

    Article  PubMed  Google Scholar 

  32. Ookura R, Shiro Y, Takai T, Okamoto M, Ogata M (2009) Diffusion-weighted magnetic resonance imaging of a severe heat stroke patient complicated with severe cerebellar ataxia. Intern Med 48:1105–1108

    Article  PubMed  Google Scholar 

  33. Patay Z (2015) Postoperative posterior fossa syndrome: unraveling the etiology and underlying pathophysiology by using magnetic resonance imaging. Childs Nerv Syst 31:1853–1858. https://doi.org/10.1007/s00381-015-2796-1

    Article  PubMed  Google Scholar 

  34. Patay Z, Enterkin J, Harreld JH, Yuan Y, Lobel U, Rumboldt Z, Khan R, Boop F (2014) MR imaging evaluation of inferior olivary nuclei: comparison of postoperative subjects with and without posterior fossa syndrome. AJNR Am J Neuroradiol 35:797–802. https://doi.org/10.3174/ajnr.A3762

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Pollack IF, Polinko P, Albright AL, Towbin R, Fitz C (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. Neurosurgery 37:885–893

    Article  CAS  PubMed  Google Scholar 

  36. Puget S, Boddaert N, Viguier D, Kieffer V, Bulteau C, Garnett M, Callu D, Sainte-Rose C, Kalifa C, Dellatolas G, Grill J (2009) Injuries to inferior vermis and dentate nuclei predict poor neurological and neuropsychological outcome in children with malignant posterior fossa tumors. Cancer 115:1338–1347. https://doi.org/10.1002/cncr.24150

    Article  PubMed  Google Scholar 

  37. Ray S, Sharma S, Maheshwari A, Aneja S, Kumar A (2013) Heat stroke in an infant with hypohidrotic ectodermal dysplasia: brain magnetic resonance imaging findings. J Child Neurol 28:538–540. https://doi.org/10.1177/0883073812474097

    Article  PubMed  Google Scholar 

  38. 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. J Neurosurg 105:444–451. https://doi.org/10.3171/ped.2006.105.6.444

    Article  PubMed  Google Scholar 

  39. Sagiuchi T, Ishii K, Aoki Y, Kan S, Utsuki S, Tanaka R, Fujii K, Hayakawa K (2001) Bilateral crossed cerebello-cerebral diaschisis and mutism after surgery for cerebellar medulloblastoma. Ann Nucl Med 15:157–160

    Article  CAS  PubMed  Google Scholar 

  40. Sergeant A, Kameda-Smith MM, Manoranjan B, Karmur B, Duckworth J, Petrelli T, Savage K, Ajani O, Yarascavitch B, Samaan MC, Scheinemann K, Alyman C, Almenawer S, Farrokhyar F, Fleming AJ, Singh SK, Stein N (2017) Analysis of surgical and MRI factors associated with cerebellar mutism. J Neuro-Oncol 133:539–552. https://doi.org/10.1007/s11060-017-2462-4

    Article  Google Scholar 

  41. Sherchan P, Kim CH, Zhang JH (2013) Surgical brain injury and edema prevention. Acta Neurochir Suppl 118:129–133. https://doi.org/10.1007/978-3-7091-1434-6_23

    Article  PubMed  Google Scholar 

  42. Siu A WE, Defreitas T, Myseros J et al. (2013) Multi-variate analysis of risk factors involved with a significant reduction in cerebellar mutism after resection of posterior fossa medulloblastoma Paper presented at the International society for Pediatric Neurosurgery. Mainz, Germany

  43. Soelva V, Hernaiz Driever P, Abbushi A, Rueckriegel S, Bruhn H, Eisner W, Thomale UW (2013) Fronto-cerebellar fiber tractography in pediatric patients following posterior fossa tumor surgery. Childs Nerv Syst 29:597–607. https://doi.org/10.1007/s00381-012-1973-8

    Article  PubMed  Google Scholar 

  44. Stoodley CJ, Schmahmann JD (2009) Functional topography in the human cerebellum: a meta-analysis of neuroimaging studies. NeuroImage 44:489–501. https://doi.org/10.1016/j.neuroimage.2008.08.039

    Article  PubMed  Google Scholar 

  45. Stoodley CJ, Valera EM, Schmahmann JD (2012) Functional topography of the cerebellum for motor and cognitive tasks: an fMRI study. NeuroImage 59:1560–1570. https://doi.org/10.1016/j.neuroimage.2011.08.065

    Article  PubMed  Google Scholar 

  46. Szathmari A, Thiesse P, Galand-desme S, Mottolese C, Bret P, Jouanneau E, Guyotat J, Lion-Francois L, Frappaz D (2010) Correlation between pre- or postoperative MRI findings and cerebellar sequelae in patients with medulloblastomas. Pediatr Blood Cancer 55:1310–1316. https://doi.org/10.1002/pbc.22802

    Article  PubMed  Google Scholar 

  47. Toescu SM, Hettige S, Phipps K, Smith RJP, Haffenden V, Clark C, Hayward R, Mankad K, Aquilina K (2018) Post-operative paediatric cerebellar mutism syndrome: time to move beyond structural MRI. Childs Nerv Syst. https://doi.org/10.1007/s00381-018-3867-x

    Article  PubMed  PubMed Central  Google Scholar 

  48. Turgut M (1998) Transient “cerebellar” mutism. Childs Nerv Syst 14:161–166

    Article  CAS  PubMed  Google Scholar 

  49. van Dongen HR, Catsman-Berrevoets CE, van Mourik M (1994) The syndrome of ‘cerebellar’ mutism and subsequent dysarthria. Neurology 44:2040–2046

    Article  PubMed  Google Scholar 

  50. Wells EM, Khademian ZP, Walsh KS, Vezina G, Sposto R, Keating RF, Packer RJ (2010) Postoperative cerebellar mutism syndrome following treatment of medulloblastoma: neuroradiographic features and origin. J Neurosurg Pediatr 5:329–334. https://doi.org/10.3171/2009.11.peds09131

    Article  PubMed  Google Scholar 

  51. Wibroe M, Cappelen J, Castor C, Clausen N, Grillner P, Gudrunardottir T, Gupta R, Gustavsson B, Heyman M, Holm S, Karppinen A, Klausen C, Lonnqvist T, Mathiasen R, Nilsson P, Nysom K, Persson K, Rask O, Schmiegelow K, Sehested A, Thomassen H, Tonning-Olsson I, Zetterqvist B, Juhler M (2017) Cerebellar mutism syndrome in children with brain tumours of the posterior fossa. BMC Cancer 17:439. https://doi.org/10.1186/s12885-017-3416-0

    Article  PubMed  PubMed Central  Google Scholar 

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  1. Alder Hey Children’s NHS Foundation Trust, East Prescot Road, Liverpool, L14 5AB, UK

    Shivaram Avula

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Avula, S. Radiology of post-operative paediatric cerebellar mutism syndrome. Childs Nerv Syst 36, 1187–1195 (2020). https://doi.org/10.1007/s00381-019-04224-x

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