Skip to main content
SpringerLink
Log in

A comparison study assessing neuropsychological outcome of patients with post-operative pediatric cerebellar mutism syndrome and matched controls after proton radiation therapy

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Post-operative pediatric cerebellar mutism syndrome (CMS), characterized by mutism, ataxia/hypotonia, and emotional lability, can result in long-term deficits following resection of posterior fossa (PF) tumors. This longitudinal study compared neuropsychological outcomes of pediatric patients with post-operative CMS to a matched control patient group without CMS.

Methods

Fifty-eight PF tumor patients received post-surgical proton radiation therapy (PRT) and testing at baseline and at ≥ 1-year post-PRT over a 10-year period. Of these, 18 (31%) had post-operative CMS with baseline and follow-up neuropsychological test data. Those participants were matched to 18 controls by tumor location, age, gender, and handedness; no significant group differences were found at baseline for clinical/demographic variables. Total mean age at baseline was 7.26 years (SD = 4.42); mean follow-up interval was 3.26 years (SD = 2.24). Areas assessed: overall intelligence, expressive and receptive vocabulary, visuomotor integration, fine motor speed, inhibition, emotional control, depression, and anxiety.

Results

Patients were 52% male; 86% medulloblastoma/14% ependymoma; 86% craniospinal irradiation/14% focal radiation; and 86% chemotherapy. No group differences were found between most mean baseline scores; expressive vocabulary and fine motor speed were significantly lower in the post-operative CMS group (p < 0.05). Mean change scores revealed no significant differences for the sample; scores were within the normal range except fine motor skills were impaired for both groups.

Conclusions

Longitudinal neuropsychological outcomes for post-operative pediatric CMS patients did not differ significantly from matched controls without this condition. Patients were in the normal range in all areas except fine motor speed, which was impaired for both groups independent of CMS diagnosis.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Bayley N (1993) Bayley scales of infant development, Second Edition: manual. The Psychological Corporation, San Antonio, TX

  2. Beery KE, Buktenica NA, Beery NA (2006) Beery-Buktenica developmental test of visual-motor integration test (ed 5). NCS Pearson, San Antonio, TX

    Google Scholar 

  3. Beery KE, Buktenica NA, Beery NA (2010) Beery-Buktenica developmental test of visual-motor integration test (ed 6). NCS Pearson, San Antonio, TX

    Google Scholar 

  4. Catsman-Berrevoets CE, van Dongen HR, Zwetsloot CP (1992) Transient loss of speech followed by dysarthria after removal of posterior fossa tumour. Dev Med Child Neurol 34:1102–1109

    Article  CAS  Google Scholar 

  5. De Smet HJ, Baillieux H, Catsman-Berrevoets C, De Deyn PP, Marien P, Paquier PF (2007) Postoperative motor speech production in children with the syndrome of ‘cerebellar’ mutism and subsequent dysarthria: a critical review of the literature. Eur J Paediatr Neuro 11(4):193–207

    Article  Google Scholar 

  6. Dietze DD Jr, Mickle JP (1990) Cerebellar mutism after posterior fossa surgery. Pediatr Neurosurg 16:25–31

    Article  Google Scholar 

  7. Dunn L, Dunn D (2007) Peabody picture vocabulary test: manual (ed 4). Pearson, Minneapolis, Minnesota

    Google Scholar 

  8. Gioia GA, Isquith PK, Guy SC, Kenworthy L (2000) Behavior rating inventory of executive function: professional manual. Psychological Assessment Resources, Odessa, FL

    Google Scholar 

  9. Greenberger BA, Pulsifer MB, Egg DH, MacDonald SM, Jones RM, Butler WE, Huang MS, Marcus KJ, Oberg JA, Tarbell NJ, Yock TI (2014) Clinical outcomes and late endocrine, neurocognitive and visual profiles of proton radiation for pediatric low-grade gliomas. Int J Radiat Oncol Biol Phys 89:1060–1068

    Article  Google Scholar 

  10. 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 32(7):1195–1203

    Article  Google Scholar 

  11. Huber JF, Bradley K, Spiegler B, Dennis M (2007) Long-term neuromotor speech deficits in survivors of childhood posterior fossa tumors: effects of tumor type, radiation, age at diagnosis and survival years. J Child Neurol 22(7):848–854

    Article  Google Scholar 

  12. Kingma A, Mooij JJ, Metzemaekers JD, Leeuw JA (1994) Transient mutism and speech disorders after posterior fossa surgery in children with brain tumours. Acta Neurochir 131:74–79

    Article  CAS  Google Scholar 

  13. Korah MP, Esiashvili N, Mazewski CM, Hudgins RJ, Tighiouart M, Janss AJ, Schwaibold FP, Crocker IR, Curran WJ, Marcus RB (2010) Incidence, risks, and sequelae of posterior fossa syndrome in pediatric medulloblastoma. Int J Radiat Oncol Biol Phys 77(1):106–112

    Article  Google Scholar 

  14. Kupeli S, Yalcin B, Bilginer B, Akalan N, Haksal P, Buyukpamukcu M (2011) Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatr Blood Cancer 56(2):206–210

    Article  Google Scholar 

  15. Lafayette Instrument (2015) Purdue pegboard: manual. Lafayette Instrument Company, Lafayette, IN

    Google Scholar 

  16. Lanier JC, Abrams AN (2017) Posterior fossa syndrome: review of the behavioral and emotional aspects in pediatric cancer patients. Cancer. 123(4):551–559

    Article  Google Scholar 

  17. MacDonald SM, DeLaney TF, Loeffler JS (2006) Proton beam radiation therapy. Cancer Investig 24:199–208

    Article  Google Scholar 

  18. Martin NA, Brownell R (2011) Expressive one-word picture vocabulary test: manual (ed 4). ATP Assessments, Novato, California

    Google Scholar 

  19. Paganetti H, Niemierko A, Ancukiewicz M, Gerwek LD, Goitein M, Loeffler JS, Suit HD (2002) Relative biological effectiveness (RBE) values for proton beam therapy. Int J Radiat Oncol Biol Phys 53:407–421

    Article  Google Scholar 

  20. Palmer SL, Hassall T, Evankovich K, Mabbott DJ, Bonner DJ, Bonner M, Deluca C, Cohn R, Fisher MJ, Morris B, Broniscer A, Gajjar A (2010) Neurocognitive outcome 12 months following cerebellar mutism syndrome in pediatric patients with medulloblastoma. Neuro-Oncology. 12:1311–1317

    PubMed  PubMed Central  Google Scholar 

  21. Pollack IF (1997) Posterior fossa syndrome. Int Rev Neurobiol 41:411–432

    Article  CAS  Google Scholar 

  22. Pulsifer MB, Sethi RV, Kuhlthau KA, MacDonald SM, Tarbell NJ, Yock TI (2015) Early cognitive outcomes following proton radiation in pediatric patients with brain and central nervous system tumors. Int J Radiat Oncol Biol Phys 93:400–407

    Article  Google Scholar 

  23. Pulsifer MB, Duncanson H, Grieco J, Evans C, Tseretopoulous ID, MacDonald S, Tarbell NJ, Yock TI (2018) Cognitive and adaptive outcomes after proton radiation for pediatric patients with brain tumors. Int J Radiat Oncol Biol Phys 102:391–398

    Article  Google Scholar 

  24. Rekate HL, Grub RL, Aram DM et al (1985) Muteness of cerebellar origin. Arch Neurol 42:697–698

    Article  CAS  Google Scholar 

  25. Reynolds CR, Kamphaus RW (2004) Behavior assessment system for children: manual (ed 2). NCS Pearson, San Antonio, TX

    Google Scholar 

  26. Sadeh M, Cohen I (2001) Transient loss of speech after removal of posterior fossa tumors- one aspect of a larger neuropsychological entity: the cerebellar cognitive affective syndrome. Pediatr Hematol Oncol 18:423–426

    Article  CAS  Google Scholar 

  27. Schmahmann JD, Sherman JC (1997) Cerebellar cognitive affective syndrome. Int Rev Neurobiol 41:433–440

    Article  CAS  Google Scholar 

  28. Schrieber JE, Palmer SL, Conklin HM, Mabbott DJ, Swain MA, Bonner MJ, Chapieski ML, Huang L, Zhang H, Gajjar A (2017) Posterior fossa syndrome and long-term neuropsychological outcomes among children treated for medulloblastoma on a multi-institutional prospective study. Neuro-Oncology 19(12):1673–1682

    Article  Google Scholar 

  29. 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 39:179–183

    Article  Google Scholar 

  30. Tamburrini G, Frassanito P, Chieffo D, Massimi L, Caldarelli M, DiRocco C (2015) Cerebellar mutism. Child Nerv Syst 31:1841–1851

    Article  CAS  Google Scholar 

  31. United States Census Bureau (2012) American community survey, median income. From the 2008–2012 American community survey 5-year estimates. Available at http://factfinder2.census.gov/faces/nav/jsf/pages/community_facts.xhtml. Accessed 9 June 2014

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

    Article  Google Scholar 

  33. Wechsler D (1997) Wechsler adult intelligence scale (ed 3). A Harcourt Assessment Company, San Antonio, Texas

    Google Scholar 

  34. Wechsler D (2003) Wechsler intelligence scale for children (ed 4). A Harcourt Assessment Company, San Antonio, Texas

    Google Scholar 

  35. Wechsler D (2003) Wechsler preschool and primary scale of intelligence (ed 3). Psychological Corporation, A Harcourt Assessment Company, San Antonio, Texas

    Google Scholar 

  36. Wechsler D (2008) Wechsler adult intelligence scale (ed 4). A Harcourt Assessment Company, San Antonio, Texas

    Google Scholar 

  37. Wechsler D (2014) Wechsler intelligence scale for children (ed 5). A Harcourt Assessment Company, San Antonio, Texas

    Google Scholar 

  38. Wisoff JH, Epstein FJ (1984) Pseudobulbar palsy after posterior fossa operation n children. Neurosurgery. 15:707–709

    Article  CAS  Google Scholar 

  39. Wolfe-Christensen C, Mullins LL, Scott JG, McNail-Knapp RY (2007) Persistent psychosocial problems in children who develop posterior fossa syndrome after medulloblastoma resection. Pediatr Blood Cancer 49(5):723–726

    Article  Google Scholar 

  40. Yock T, Schneider R, Friedmann A, Adams J, Fullerton B, Tarbell N (2005) Proton radiotherapy for orbital rhabdomyosarcoma: clinical outcome and a dosimetric comparison with photons. Int J Radiat Oncol Biol Phys 63(4):1161–1168

    Article  Google Scholar 

Download references

Acknowledgments

The authors thank the MGH Francis Burr Proton Center pediatric patients and their families for participation in this study.

Funding

The project described was supported by Award Number P01CA021239 from the National Cancer Institute. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Cancer Institute or the National Institutes of Health. The project was supported by the Federal Share of program income earned by the Massachusetts General Hospital (MGH) on C06 CA059267 Proton Therapy Research and Treatment Center.

Author information

Authors and Affiliations

  1. Department of Psychiatry, Massachusetts General Hospital, One Bowdoin Square, 7th Floor, Boston, MA, 02114, USA

    Julie A. Grieco, Casey L. Evans & Margaret B. Pulsifer

  2. Harvard Medical School, Boston, MA, USA

    Julie A. Grieco, Annah N. Abrams, Torunn I. Yock & Margaret B. Pulsifer

  3. Department of Psychiatry, Massachusetts General Hospital, 55 Fruit Street, Boston, MA, 02114, USA

    Annah N. Abrams

  4. Department of Radiation Oncology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA, 02114, USA

    Torunn I. Yock

Authors
  1. Julie A. Grieco
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Annah N. Abrams
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Casey L. Evans
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Torunn I. Yock
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Margaret B. Pulsifer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Margaret B. Pulsifer.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

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

Grieco, J.A., Abrams, A.N., Evans, C.L. et al. A comparison study assessing neuropsychological outcome of patients with post-operative pediatric cerebellar mutism syndrome and matched controls after proton radiation therapy. Childs Nerv Syst 36, 305–313 (2020). https://doi.org/10.1007/s00381-019-04299-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-019-04299-6

Keywords

Search

Navigation