Neurosurgical Review

, Volume 33, Issue 4, pp 401–408 | Cite as

Postoperative cerebellar mutism in adult patients with Lhermitte-Duclos disease

  • Ali Afshar-Oromieh
  • Heinz Linhart
  • Dino Podlesek
  • Wiebke Schrempf
  • Gabriele Schackert
  • Dietmar KrexEmail author


Cerebellar mutism (CM) is a rare and severe form of speech and language impairment, mostly diagnosed in children and adolescents and rarely reported in adults. We here review the literature and summarize all anatomical structures related to the pathogenesis of this rare syndrome. We also report two illustrative cases of CM following surgical treatment of Lhermitte-Duclos disease (LDD; dysplastic gangliocytoma) in two adult patients. LDD is a rare benign cerebellar tumor. Surgical excision appears to be the only effective treatment. However, surgery is hampered by the difficulty to distinguish between tumor and healthy cerebellar tissue, which may result in extensive resection and cause neurological deficits such as CM. A review of the literature and our two cases suggest that lesions or functional impairment of paravermian structures including dentate nuclei, vermis, lateral hemispheres, and cerebellocortical pathways contribute to the development of CM. However, there is no single anatomical structure identified to be associated with CM. It is unknown whether some diseases such as LDD carry a higher risk of postoperative CM than others. As illustrated by our two cases, although there are no special means, optimal preoperative diagnosis might contribute to the prevention of this syndrome. Despite the severity, CM carries a favorable prognosis and generally resolves within a few months.

To conclude, we review the clinical signs and particularly the pathophysiological observations and anatomical structures affected in the development of postoperative CM and contribute two cases illustrating the pathogenesis, prognosis, and possible prevention of this syndrome, to focus that CM might also occur in adults even in association with rare tumors.


Cerebellar mutism Posterior fossa Lhermitte-Duclos disease Dysplastic cerebellar gangliocytoma 



  1. 1.
    Ackermann H, Mathiak K, Ivry RB (2004) Temporal organization of “internal speech” as a basis for cerebellar modulation of cognitive functions. Behav Cognit Neurosci Rev 3:14–22CrossRefGoogle Scholar
  2. 2.
    Ackermann H, Mathiak K, Riecker A (2007) The contribution of the cerebellum to speech production and speech perception: clinical and functional imaging data. Cerebellum 6:202–213CrossRefPubMedGoogle Scholar
  3. 3.
    Aguiar PH, Plese JP, Ciquini O, Marino R (1995) Transient mutism following a posterior fossa approach to cerebellar tumors in children: a critical review of the literature. Childs Nerv Syst 11:306–310CrossRefPubMedGoogle Scholar
  4. 4.
    Akhaddar A, Belhachmi A, Elasri A, Boulahroud O, Okacha N, Elmostarshid B, Boucetta M (2008) Cerebellar mutism after removal of a vermian medulloblastoma in an adult. Neurochirurgie 54:548–550CrossRefPubMedGoogle Scholar
  5. 5.
    Ammirati M, Mirzai S, Samii M (1989) Transient mutism following removal of a cerebellar tumor. A case report and review of the literature. Childs Nerv Syst 5:12–14CrossRefPubMedGoogle Scholar
  6. 6.
    Baddeley AD, Andrade J (2000) Working memory and the vividness of imagery. J Exp Psychol Gen 129:126–145CrossRefPubMedGoogle Scholar
  7. 7.
    Bhatoe HS (1997) Mutism, oropharyngeal apraxia and dysarthria after posterior fossa tumour excision. Br J Neurosurg 11:341–343CrossRefPubMedGoogle Scholar
  8. 8.
    Buhl R, Barth H, Hugo HH, Straube T, Mehdorn HM (2003) Dysplastic gangliocytoma of the cerebellum: rare differential diagnosis in space occupying lesions of the posterior fossa. Acta Neurochir (Wien ) 145:509–512Google Scholar
  9. 9.
    Cakir Y, Karakisi D, Kocanaogullari O (1994) Cerebellar mutism in an adult: case report. Surg Neurol 41:342–344CrossRefPubMedGoogle Scholar
  10. 10.
    Caner H, Altinors N, Benli S, Calisaneller T, Albayrak A (1999) Akinetic mutism after fourth ventricle choroid plexus papilloma: treatment with a dopamine agonist. Surg Neurol 51:181–184CrossRefPubMedGoogle Scholar
  11. 11.
    Crutchfield JS, Sawaya R, Meyers CA, Moore BD III (1994) Postoperative mutism in neurosurgery. Report of two cases. J Neurosurg 81:115–121CrossRefPubMedGoogle Scholar
  12. 12.
    D’Avanzo R, Scuotto A, Natale M, Scotto P, Cioffi FA (1993) Transient “cerebellar” mutism in lesions of the mesencephalic-cerebellar region. Acta Neurol (Napoli) 15:289–296Google Scholar
  13. 13.
    Dailey AT, McKhann GM, Berger MS (1995) The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children. J Neurosurg 83:467–475CrossRefPubMedGoogle Scholar
  14. 14.
    de Ribaupierre S, Ryser C, Villemure JG, Clarke S (2008) Cerebellar lesions: is there a lateralisation effect on memory deficits? Acta Neurochir (Wien ) 150:545–550CrossRefGoogle Scholar
  15. 15.
    Decety J, Sjoholm H, Ryding E, Stenberg G, Ingvar DH (1990) The cerebellum participates in mental activity: tomographic measurements of regional cerebral blood flow. Brain Res 535:313–317CrossRefPubMedGoogle Scholar
  16. 16.
    Dietze DD Jr, Mickle JP (1990) Cerebellar mutism after posterior fossa surgery. Pediatr Neurosurg 16:25–31CrossRefPubMedGoogle Scholar
  17. 17.
    Dubey A, Sung WS, Shaya M, Patwardhan R, Willis B, Smith D, Nanda A (2009) Complications of posterior cranial fossa surgery—an institutional experience of 500 patients. Surg Neurol 72:369–375CrossRefPubMedGoogle Scholar
  18. 18.
    Dunwoody GW, Alsagoff ZS, Yuan SY (1997) Cerebellar mutism with subsequent dysarthria in an adult: case report. Br J Neurosurg 11:161–163CrossRefPubMedGoogle Scholar
  19. 19.
    Ersahin Y (1998) Is splitting of the vermis responsible for cerebellar mutism? Pediatr Neurosurg 28:328CrossRefPubMedGoogle Scholar
  20. 20.
    Ersahin Y (1998) SPECT in cerebellar mutism. Childs Nerv Syst 14:611–613CrossRefPubMedGoogle Scholar
  21. 21.
    Ersahin Y, Mutluer S, Cagli S, Duman Y (1996) Cerebellar mutism: report of seven cases and review of the literature. Neurosurgery 38:60–65CrossRefPubMedGoogle Scholar
  22. 22.
    Faillot T, Sichez JP, Brault JL, Capelle L, Kujas M, Bordi L, Boukobza M (1990) Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). Report of a case and review of the literature. Acta Neurochir (Wien ) 105:44–49CrossRefGoogle Scholar
  23. 23.
    Fiez JA, Petersen SE, Cheney MK, Raichle ME (1992) Impaired non-motor learning and error detection associated with cerebellar damage. A single case study. Brain 115(Pt 1):155–178CrossRefPubMedGoogle Scholar
  24. 24.
    Frim DM, Ogilvy CS (1995) Mutism and cerebellar dysarthria after brain stem surgery: case report. Neurosurgery 36:854–857CrossRefPubMedGoogle Scholar
  25. 25.
    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 21:234–236CrossRefPubMedGoogle Scholar
  26. 26.
    Gebhart AL, Petersen SE, Thach WT (2002) Role of the posterolateral cerebellum in language. Ann NY Acad Sci 978:318–333CrossRefPubMedGoogle Scholar
  27. 27.
    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 14:114–119CrossRefPubMedGoogle Scholar
  28. 28.
    Hirsch JF, Renier D, Czernichow P, Benveniste L, Pierre-Kahn A (1979) Medulloblastoma in childhood. Survival and functional results. Acta Neurochir (Wien ) 48:1–15CrossRefGoogle Scholar
  29. 29.
    Ildan F, Tuna M, Erman T, Gocer AI, Zeren M, Cetinalp E (2002) The evaluation and comparison of cerebellar mutism in children and adults after posterior fossa surgery: report of two adult cases and review of the literature. Acta Neurochir (Wien ) 144:463–473CrossRefGoogle Scholar
  30. 30.
    Ivry RB, Spencer RM, Zelaznik HN, Diedrichsen J (2002) The cerebellum and event timing. Ann NY Acad Sci 978:302–317CrossRefPubMedGoogle Scholar
  31. 31.
    Kai Y, Kuratsu J, Suginohara K, Marubayashi T, Ushio Y (1997) Cerebellar mutism after posterior fossa surgery—two case reports. Neurol Med Chir (Tokyo) 37:929–933CrossRefGoogle Scholar
  32. 32.
    Klisch J, Juengling F, Spreer J, Koch D, Thiel T, Buchert M, Arnold S, Feuerhake F, Schumacher M (2001) Lhermitte-Duclos disease: assessment with MR imaging, positron emission tomography, single-photon emission CT, and MR spectroscopy. AJNR Am J Neuroradiol 22:824–830PubMedGoogle Scholar
  33. 33.
    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 104:329–331CrossRefPubMedGoogle Scholar
  34. 34.
    Lotze M, Montoya P, Erb M, Hulsmann E, Flor H, Klose U, Birbaumer N, Grodd W (1999) Activation of cortical and cerebellar motor areas during executed and imagined hand movements: an fMRI study. J Cogn Neurosci 11:491–501CrossRefPubMedGoogle Scholar
  35. 35.
    McMillan HJ, Keene DL, Matzinger MA, Vassilyadi M, Nzau M, Ventureyra EC (2009) Brainstem compression: a predictor of postoperative cerebellar mutism. Childs Nerv Syst 25:677–681CrossRefPubMedGoogle Scholar
  36. 36.
    Moonis G, Ibrahim M, Melhem ER (2004) Diffusion-weighted MRI in Lhermitte-Duclos disease: report of two cases. Neuroradiology 46:351–354CrossRefPubMedGoogle Scholar
  37. 37.
    Nagatani K, Waga S, Nakagawa Y (1991) Mutism after removal of a vermian medulloblastoma: cerebellar mutism. Surg Neurol 36:307–309CrossRefPubMedGoogle Scholar
  38. 38.
    Nandagopal R, Krishnamoorthy SG (2004) Transient mutism due to posterior circulation infarction. Neurol India 52:510–511PubMedGoogle Scholar
  39. 39.
    Nowak DA, Trost HA (2002) Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand 105:137–145CrossRefPubMedGoogle Scholar
  40. 40.
    Nowak DA, Trost HA, Porr A, Stolzle A, Lumenta CB (2001) Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). Clin Neurol Neurosurg 103:105–110CrossRefPubMedGoogle Scholar
  41. 41.
    Ozgur BM, Berberian J, Aryan HE, Meltzer HS, Levy ML (2006) The pathophysiologic mechanism of cerebellar mutism. Surg Neurol 66:18–25CrossRefPubMedGoogle Scholar
  42. 42.
    Ozimek A, Richter S, Hein-Kropp C, Schoch B, Gorissen B, Kaiser O, Gizewski E, Ziegler W, Timmann D (2004) Cerebellar mutism—report of four cases. J Neurol 251:963–972CrossRefPubMedGoogle Scholar
  43. 43.
    Papavasiliou AS, Kotsalis C, Trakadas S (2004) Transient cerebellar mutism in the course of acute cerebellitis. Pediatr Neurol 30:71–74CrossRefPubMedGoogle Scholar
  44. 44.
    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 37:885–893CrossRefPubMedGoogle Scholar
  45. 45.
    Rekate HL, Grubb RL, Aram DM, Hahn JF, Ratcheson RA (1985) Muteness of cerebellar origin. Arch Neurol 42:697–698PubMedGoogle Scholar
  46. 46.
    Riva D, Giorgi C (2000) The cerebellum contributes to higher functions during development: evidence from a series of children surgically treated for posterior fossa tumours. Brain 123(Pt 5):1051–1061CrossRefPubMedGoogle Scholar
  47. 47.
    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 105:444–451PubMedGoogle Scholar
  48. 48.
    Ryding E, Decety J, Sjoholm H, Stenberg G, Ingvar DH (1993) Motor imagery activates the cerebellum regionally. A SPECT rCBF study with 99mTc-HMPAO. Brain Res Cogn Brain Res 1:94–99CrossRefPubMedGoogle Scholar
  49. 49.
    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 15:157–160CrossRefPubMedGoogle Scholar
  50. 50.
    Salvati M, Missori P, Lunardi P, Orlando ER (1991) Transient cerebellar mutism after posterior cranial fossa surgery in an adult. Case report and review of the literature. Clin Neurol Neurosurg 93:313–316CrossRefPubMedGoogle Scholar
  51. 51.
    Sherman JH, Sheehan JP, Elias WJ, Jane JA Sr (2005) Cerebellar mutism in adults after posterior fossa surgery: a report of 2 cases. Surg Neurol 63:476–479CrossRefPubMedGoogle Scholar
  52. 52.
    Siffert J, Poussaint TY, Goumnerova LC, Scott RM, LaValley B, Tarbell NJ, Pomeroy SL (2000) Neurological dysfunction associated with postoperative cerebellar mutism. J Neurooncol 48:75–81CrossRefPubMedGoogle Scholar
  53. 53.
    Sinha AK, Rajender Y, Dinakar I (1998) Transient cerebellar mutism after evacuation of a spontaneous vermian haematoma. Childs Nerv Syst 14:460–462CrossRefPubMedGoogle Scholar
  54. 54.
    Spencer RM, Ivry RB, Zelaznik HN (2005) Role of the cerebellum in movements: control of timing or movement transitions? Exp Brain Res 161:383–396CrossRefPubMedGoogle Scholar
  55. 55.
    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 39:179–183CrossRefPubMedGoogle Scholar
  56. 56.
    Tanriover N, Ulm AJ, Rhoton AL Jr, Yasuda A (2004) Comparison of the transvermian and telovelar approaches to the fourth ventricle. J Neurosurg 101:484–498CrossRefPubMedGoogle Scholar
  57. 57.
    Thomas B, Krishnamoorthy T, Radhakrishnan VV, Kesavadas C (2007) Advanced MR imaging in Lhermitte-Duclos disease: moving closer to pathology and pathophysiology. Neuroradiology 49:733–738CrossRefPubMedGoogle Scholar
  58. 58.
    Urban PP, Marx J, Hunsche S, Gawehn J, Vucurevic G, Wicht S, Massinger C, Stoeter P, Hopf HC (2003) Cerebellar speech representation: lesion topography in dysarthria as derived from cerebellar ischemia and functional magnetic resonance imaging. Arch Neurol 60:965–972CrossRefPubMedGoogle Scholar
  59. 59.
    Van Calenbergh F, Van de LA, Plets C, Goffin J, Casaer P (1995) Transient cerebellar mutism after posterior fossa surgery in children. Neurosurgery 37:894–898CrossRefPubMedGoogle Scholar
  60. 60.
    Van Dongen HR, Catsman-Berrevoets CE, van Mourik M (1994) The syndrome of ‘cerebellar’ mutism and subsequent dysarthria. Neurology 44:2040–2046PubMedGoogle Scholar
  61. 61.
    van Mourik M, Catsman-Berrevoets CE, Van Dongen HR, Neville BG (1997) Complex orofacial movements and the disappearance of cerebellar mutism: report of five cases. Dev Med Child Neurol 39:686–690CrossRefPubMedGoogle Scholar
  62. 62.
    Vandeinse D, Hornyak JE (1997) Linguistic and cognitive deficits associated with cerebellar mutism. Pediatr Rehabil 1:41–44PubMedGoogle Scholar
  63. 63.
    Wells EM, Walsh KS, Khademian ZP, Keating RF, Packer RJ (2008) The cerebellar mutism syndrome and its relation to cerebellar cognitive function and the cerebellar cognitive affective disorder. Dev Disabil Res Rev 14:221–228CrossRefPubMedGoogle Scholar
  64. 64.
    Wise RJ, Greene J, Buchel C, Scott SK (1999) Brain regions involved in articulation. Lancet 353:1057–1061CrossRefPubMedGoogle Scholar
  65. 65.
    Wu CH, Chai JW, Lee CH, Chen WH, Lee T, Chen CC (2006) Assessment with magnetic resonance imaging and spectroscopy in Lhermitte-Duclos disease. J Chin Med Assoc 69:338–342CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Ali Afshar-Oromieh
    • 1
  • Heinz Linhart
    • 2
  • Dino Podlesek
    • 1
  • Wiebke Schrempf
    • 3
  • Gabriele Schackert
    • 1
  • Dietmar Krex
    • 1
    Email author
  1. 1.Department of NeurosurgeryUniversity Hospital Carl Gustav Carus, Technical University of DresdenDresdenGermany
  2. 2.German Cancer Research Center (DKFZ) and National Center for Tumor Diseases (NCT)HeidelbergGermany
  3. 3.Department of NeurologyCarl-Gustav-Carus University Hospital of DresdenDresdenGermany

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