Skip to main content

Advertisement

SpringerLink
Log in

Sudden benzodiazepine-induced resolution of post-operative pediatric cerebellar mutism syndrome: a clinical-SPECT study

  • Case Report - Brain Tumors
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Post-operative pediatric cerebellar mutism syndrome (PPCMS) is a clinical syndrome arising from cerebellar injury and characterized by absence of speech and other possible symptoms and signs. Rare reports described some benefit after administration of dopamine agonist therapy, but no treatment has proven efficacy. In this paper, we report on the dramatic, sudden resolution of PPCMS induced by midazolam administration in a boy who underwent posterior fossa surgery for choroid plexus papilloma of the fourth ventricle. In addition to clinical improvement, post-midazolam single-photon emission computed tomography also demonstrated amelioration of brain perfusion.

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
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Adachi J, Nishikawa R, Hirose T, Matsutani M (2005) Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: case report. Surg Neurol 63:375–379

    Article  PubMed  Google Scholar 

  2. Caner H, Altinörs N, Benli S, Calişaneller T, Albayrak A (1999) Akinetic mutism after fourth ventricle choroid plexus papilloma: treatment with a dopamine agonist. Surg Neurol 51:181–184

    Article  CAS  PubMed  Google Scholar 

  3. 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  PubMed  Google Scholar 

  4. Clerico A, Sordi A, Ragni G, Festa A, Cappelli C, Maini CL (2002) Brief report: transient mutism following posterior fossa surgery studied by single photon emission computed tomography (SPECT). Med Pediatr Oncol 38:445–448

    Article  PubMed  Google Scholar 

  5. Dailey AT, McKhann GM 2nd, Berger MS (1995) The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children. J Neurosurg 83:467–475

    Article  CAS  PubMed  Google Scholar 

  6. Echiverri HC, Tatum WO, Merens TA, Coker SB (1988) Akinetic mutism: pharmacologic probe of the dopaminergic mesencephalofrontal activating system. Pediatr Neurol 4:228–230

    Article  CAS  PubMed  Google Scholar 

  7. Gudrunardottir T, Morgan AT, Lux AL, Walker DA, Walsh KS, Wells EM, Wisoff JH, Juhler M, Schmahmann JD, Keating RF, Catsman-Berrevoets C, Iceland Delphi Group (2016) Consensus paper on post-operative pediatric cerebellar mutism syndrome: the Iceland Delphi results. Childs Nerv Syst 32(7):1195–1203

    Article  PubMed  Google Scholar 

  8. Infeld B, Binns D, Lichtenstein M, Hopper JL, Davis SM (1997) Volumetric analysis of cerebral hypoperfusion on SPECT: validation and reliability. J Nucl Med 38(9):1447–1453

    CAS  PubMed  Google Scholar 

  9. Küper M, Timmann D (2013) Cerebellar mutism. Brain Lang 127(3):327–333

    Article  PubMed  Google Scholar 

  10. Liberatore M, Morreale M, Megna V, Collorone S, Chondrogiannis S, Drudi FM, Anagnostou C, Civitelli L, Francia A, Maffione AM, Marzola MC, Rubello D (2015) Role of brain perfusion SPECT with 99mTc HMPAO in the assessment of response to drug therapy in patients with autoimmune vasculitis: a prospective study. N Am J Med Sci 7:135–142

    Article  Google Scholar 

  11. Mapelli L, Pagani M, Garrido JA, D’Angelo E (2015) Integrated plasticity at inhibitory and excitatory synapses in the cerebellar circuit. Front Cell Neurosci 9:169

    Article  PubMed  PubMed Central  Google Scholar 

  12. Pitsika M, Tsitouras V (2013) Cerebellar mutism. J Neurosurg Pediatr 12(6):604–614

    Article  PubMed  Google Scholar 

  13. Shyu C, Burke K, Souweidane MM, Dunkel IJ, Gilheeney SW, Gershon T, Khakoo Y (2011) Novel use of zolpidem in cerebellar mutism syndrome. J Pediatr Hematol Oncol 33:148–149

    Article  CAS  PubMed  Google Scholar 

  14. van Baarsen KM, Grotenhuis JA (2014) The anatomical substrate of cerebellar mutism. Med Hypotheses 82(6):774–780

    Article  PubMed  Google Scholar 

  15. 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 

Download references

Acknowledgments

Prof. Ferdinando Nicoletti (Department of Physiology and Pharmacology, University Sapienza of Rome, Italy) is heartily acknowledged for his precious contribution and suggestions. Dr. Marina Manzo is fully acknowledged for performing neuropsychological tests.

Author information

Authors and Affiliations

  1. Child Neurology Division, Department of Pediatrics and Child Neuropsychiatry, Umberto I Hospital, Sapienza University of Rome, Viale Regina Elena 324, 00161, Roma, Italy

    Francesco Nicita & Alberto Spalice

  2. Pediatric Oncology Division, Department of Pediatrics and Child Neuropsychiatry, Umberto I Hospital, Sapienza University of Rome, Rome, Italy

    Milena Paiano, Anna Clerico & Amalia Schiavetti

  3. Section of Nuclear Medicine, Department of Radiological, Oncological and Anatomo-Pathological Sciences, Umberto I Hospital, Sapienza University of Rome, Rome, Italy

    Mauro Liberatore & Mariacristina Ullo

  4. Pediatric Intensive Care Unit, Department of Pediatrics and Child Neuropsychiatry, Umberto I Hospital, Sapienza University of Rome, Rome, Italy

    Paola Papoff

  5. Department of Neurosurgery, Umberto I Hospital, Sapienza University of Rome, Rome, Italy

    Manolo Piccirilli

Authors
  1. Francesco Nicita
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Milena Paiano
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mauro Liberatore
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alberto Spalice
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Paola Papoff
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Mariacristina Ullo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Manolo Piccirilli
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Anna Clerico
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Amalia Schiavetti
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Francesco Nicita.

Ethics declarations

Conflict of interest

None.

Informed patient consent

The patient has consented to submission of this case report to the journal.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Table S1

Mean and standard deviation (SD) of percentage of maximum count rate registered on the encephalon and calculated for all the selected ROI. Comparison between the values obtained during the different SPECT shows progressive improvement of perfusion on the left parietal and occipital lobes and of basal ganglia (values in bold). Abbreviation list: CNl = left caudate nucleus left; CNr = right caudate nucleus; FLl = left frontal lobe; FLr = right frontal lobe; OLl = left occipital lobe; OLr = right occipital lobe; PLl = left parietal lobe; PLr = right parietal lobe; TLl = left temporal lobe; TLr = right temporal lobe (DOC 38 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Nicita, F., Paiano, M., Liberatore, M. et al. Sudden benzodiazepine-induced resolution of post-operative pediatric cerebellar mutism syndrome: a clinical-SPECT study. Acta Neurochir 159, 475–479 (2017). https://doi.org/10.1007/s00701-016-3059-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-016-3059-y

Keywords

Search

Navigation