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Open Access 01-11-2018 | Original Paper

Post-operative paediatric cerebellar mutism syndrome: time to move beyond structural MRI

Authors: Sebastian M. Toescu, Samantha Hettige, Kim Phipps, RJ Paul Smith, Verity Haffenden, Chris Clark, Richard Hayward, Kshitij Mankad, Kristian Aquilina

Published in: Child's Nervous System | Issue 11/2018

Abstract

Purpose

To determine the value of structural magnetic resonance imaging (MRI) in predicting post-operative paediatric cerebellar mutism syndrome (pCMS) in children undergoing surgical treatment for medulloblastoma.

Methods

Retrospective cohort study design. Electronic/paper case note review of all children with medulloblastoma presenting to Great Ormond Street Hospital between 2003 and 2013. The diagnosis of pCMS was established through a scoring system incorporating mutism, ataxia, behavioural disturbance and cranial nerve deficits. MRI scans performed at three time points were assessed by neuroradiologists blinded to the diagnosis of pCMS.

Results

Of 56 children included, 12 (21.4%) developed pCMS as judged by a core symptom of mutism. pCMS was more common in those aged 5 or younger. There was no statistically significant difference in pre-operative distortion or signal change of the dentate or red nuclei or superior cerebellar peduncles (SCPs) between those who did and did not develop pCMS. In both early (median 5 days) and late (median 31 months) post-operative scans, T2-weighted signal change in SCPs was more common in the pCMS group (p = 0.040 and 0.046 respectively). Late scans also showed statistically significant signal change in the dentate nuclei (p = 0.024).

Conclusions

The development of pCMS could not be linked to any observable changes on pre-operative structural MRI scans. Post-operative T2-weighted signal change in the SCPs and dentate nuclei underlines the role of cerebellar efferent injury in pCMS. Further research using advanced quantitative MRI sequences is warranted given the inability of conventional pre-surgical MRI to predict pCMS.

Daly DD, Love JG (1958) Akinetic mutism. Neurology 8:238–242CrossRef

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

Wisoff JH, Epstein FJ (1984) Pseudobulbar palsy after posterior fossa operation in children. Neurosurgery 15:707–709. https://doi.org/10.1227/00006123-198411000-00014 CrossRefPubMed

Rekate HL, Grubb RL, Aram DM, Hahn JF, Ratcheson RA (1985) Muteness of cerebellar origin. Arch Neurol 42:697–698CrossRef

Kubota T, Suzuki T, Kitase Y, Kidokoro H, Miyajima Y, Ogawa A, Natsume J, Okumura A (2011) Chronological diffusion-weighted imaging changes and mutism in the course of rotavirus-associated acute cerebellitis/cerebellopathy concurrent with encephalitis/encephalopathy. Brain Dev 33:21–27. https://doi.org/10.1016/J.BRAINDEV.2010.04.007 CrossRefPubMed

Takanashi J, Miyamoto T, Ando N, Kubota T, Oka M, Kato Z, Hamano S, Hirabayashi S, Kikuchi M, Barkovich AJ (2010) Clinical and radiological features of rotavirus cerebellitis. AJNR Am J Neuroradiol 31:1591–1595. https://doi.org/10.3174/ajnr.A2131 CrossRefPubMed

Erşahin Y (2005) Mutism after evacuation of acute subdural hematoma. Childs Nerv Syst 21:1016–1016. https://doi.org/10.1007/s00381-005-1259-5 CrossRefPubMed

Fujisawa H, Yonaha H, Okumoto K, Uehara H, Ie T, Nagata Y, Suehiro E, Suzuki M (2005) Mutism after evacuation of acute subdural hematoma of the posterior fossa. Childs Nerv Syst 21:234–236. https://doi.org/10.1007/s00381-004-0999-y CrossRefPubMed

Baillieux H, Weyns F, Paquier P, de Deyn PP, Mariën P (2007) Posterior fossa syndrome after a Vermian stroke: a new case and review of the literature. Pediatr Neurosurg 43:386–395. https://doi.org/10.1159/000106388 CrossRefPubMed

10.

Coplin WM, Kim DK, Kliot M, Bird TD (1997) Mutism in an adult following hypertensive cerebellar hemorrhage: nosological discussion and illustrative case. Brain Lang 59:473–493. https://doi.org/10.1006/brln.1997.1790 CrossRefPubMed

11.

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 CrossRefPubMed

12.

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 Pediatr 105:444–451. https://doi.org/10.3171/ped.2006.105.6.444 CrossRef

13.

Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121(Pt 4):561–579CrossRef

14.

Gudrunardottir T, Morgan AT, Lux AL et al (2016) Consensus paper on post-operative pediatric cerebellar mutism syndrome: the Iceland Delphi results. Childs Nerv Syst 32:1195–1203. https://doi.org/10.1007/s00381-016-3093-3 CrossRefPubMed

15.

Steinbok P, Cochrane DD, Perrin R, Price A (2003) Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatr Neurosurg 39:179–183CrossRef

16.

De Smet HJ, Baillieux H, Wackenier P et al (2009) Long-term cognitive deficits following posterior fossa tumor resection: a neuropsychological and functional neuroimaging follow-up study. Neuropsychology 23:694–704. https://doi.org/10.1037/a0016106 CrossRefPubMed

17.

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 132:3087–3095. https://doi.org/10.1093/brain/awp241 CrossRefPubMedPubMedCentral

18.

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 CrossRefPubMed

19.

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 CrossRefPubMedPubMedCentral

20.

Soelva V, Hernáiz 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 CrossRefPubMed

21.

Van Baarsen KM, Grotenhuis JA (2014) The anatomical substrate of cerebellar mutism. Med Hypotheses 82:774–780. https://doi.org/10.1016/j.mehy.2014.03.023 CrossRefPubMed

22.

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

23.

Mcmillan HJ, Keene DL, Matzinger MA et al Brainstem compression: a predictor of postoperative cerebellar mutism. https://doi.org/10.1007/s00381-008-0777-3 CrossRef

24.

Doxey D, Bruce D, Sklar F, Swift D, Shapiro K (1999) Posterior fossa syndrome: identifiable risk factors and irreversible complications. Pediatr Neurosurg 31:131–136CrossRef

25.

Pols SYCV, van Veelen MLC, Aarsen FK, Gonzalez Candel A, Catsman-Berrevoets CE (2017) Risk factors for development of postoperative cerebellar mutism syndrome in children after medulloblastoma surgery. J Neurosurg Pediatr 20:35–41. https://doi.org/10.3171/2017.2.PEDS16605 CrossRefPubMed

26.

Catsman-Berrevoets CE, Van Dongen HR, H Mulder PG et al (1999) Tumour type and size are high risk factors for the syndrome of cerebellar mutism and subsequent dysarthria. J Neurol Neurosurg Psychiatry 67:755–757CrossRef

27.

Turgut M (1998) Transient “cerebellar” mutism. Childs Nerv Syst 4:161–166CrossRef

28.

Pollack IF, Polinko P, Albright LA, Towbin R, Fitz C (1995) Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children. Neurosurgery 37:885–892. https://doi.org/10.1227/00006123-199511000-00006 CrossRefPubMed

29.

Van Calenbergh F, Van De Laar A, Plets C et al (1995) Transient cerebellar mutism after posterior fossa surgery in children. Neurosurgery 37:894–897. https://doi.org/10.1227/00006123-199511000-00007 CrossRefPubMed

30.

Catsman-Berrevoets CE, Aarsen FK (2010) The spectrum of neurobehavioural deficits in the posterior fossa syndrome in children after cerebellar tumour surgery. Cortex 46:933–946. https://doi.org/10.1016/j.cortex.2009.10.007 CrossRefPubMed

31.

Siffert J, Young Poussaint T, Goumnerova LC, Scott RM, LaValley B, Tarbell NJ, Pomeroy SL (2000) Neurological dysfunction associated with postoperative cerebellar mutism. J Neuro-Oncol 48:75–81. https://doi.org/10.1023/A:1006483531811 CrossRef

32.

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 77:106–112. https://doi.org/10.1016/j.ijrobp.2009.04.058 CrossRef

33.

Erşahin Y, Yararbas U, Duman Y, Mutluer S (2002) Single photon emission tomography following posterior fossa surgery in patients with and without mutism. Childs Nerv Syst 18:318–325. https://doi.org/10.1007/s00381-002-0614-z CrossRefPubMed

34.

Liu J-F, 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 32:1–10. https://doi.org/10.1080/02688697.2018.1431204 CrossRef

35.

Di Rocco C, Chieffo D, Frassanito P et al (2011) Heralding cerebellar mutism: evidence for pre-surgical language impairment as primary risk factor in posterior Fossa surgery. Cerebellum 10:551–562. https://doi.org/10.1007/s12311-011-0273-2 CrossRefPubMed

36.

Tamburrini G, Frassanito P, Chieffo D, Massimi L, Caldarelli M, di Rocco C (2015) Cerebellar mutism. Childs Nerv Syst 31:1841–1851. https://doi.org/10.1007/s00381-015-2803-6 CrossRefPubMed

37.

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 CrossRefPubMed

38.

Fraioli B, Guidetti (1975) Effects of stereotactic lesions of the dentate nucleus of the cerebellum in man. Appl Neurophysiol 38:81–90PubMed

39.

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. Am J Neuroradiol 35:797–802. https://doi.org/10.3174/ajnr.A3762 CrossRefPubMed

40.

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 CrossRefPubMed

41.

Basser PJ, Mattiello J, LeBihan D (1994) MR diffusion tensor spectroscopy and imaging. Biophys J 66:259–267. https://doi.org/10.1016/S0006-3495(94)80775-1 CrossRefPubMedPubMedCentral

42.

Abhinav K, Yeh FC, Mansouri A, Zadeh G, Fernandez-Miranda JC (2015) High-definition fiber tractography for the evaluation of perilesional white matter tracts in high-grade glioma surgery. Neuro-Oncology 17:1199–1209. https://doi.org/10.1093/neuonc/nov113 CrossRefPubMedPubMedCentral

43.

Thomale U-W, Driever PH (2013) Inconsistent terminology for cerebellar mutism. Childs Nerv Syst 29:717–718. https://doi.org/10.1007/s00381-013-2074-z CrossRefPubMed

Title: Post-operative paediatric cerebellar mutism syndrome: time to move beyond structural MRI
Authors: Sebastian M. Toescu
Samantha Hettige
Kim Phipps
RJ Paul Smith
Verity Haffenden
Chris Clark
Richard Hayward
Kshitij Mankad
Kristian Aquilina
Publication date: 01-11-2018
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 11/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-018-3867-x

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Post-operative paediatric cerebellar mutism syndrome: time to move beyond structural MRI

Abstract

Purpose

Methods

Results

Conclusions

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

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