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Living with the cerebellar mutism syndrome: long-term challenges of the diagnosis

  • Original Article - Tumor - Other
  • Published:
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Abstract

Background

After posterior fossa tumour surgery, up to 39% of children experience postoperative cerebellar mutism syndrome (CMS) characterized by mutism and other motor and cognitive impairments. There is a lack of knowledge on the patient-reported challenges and long-term needs. Consequently, no specific recommendations exist for rehabilitative and supportive interventions for patients with CMS. The aims of this study were to explore the patients’ experiences related to the sequelae of CMS, to identify challenges and needs regarding support and rehabilitation in the period of growing from child to adult and to add perspectives for future developments of supportive care and rehabilitative guidelines.

Methods

Ten semi-structured interviews were conducted with young adults diagnosed with CMS as children. A thematic analysis identified four themes describing challenges impacting aspects of the participants’ lives.

Results

Four main themes were identified and highlight the rehabilitative need for focus on verbal and non-verbal communication skills in addition to the physical impairments. We found that brain tumour survivors with CMS can benefit from social and educational rehabilitation, straightforward and truthful information, support in structuring their everyday lives and increased public knowledge of CMS.

Conclusion

Children with CMS face a variety of challenges affecting many aspects of their everyday lives. They should be entitled to the elements of a current rehabilitation initiative for childhood cancer to support patients’ social disability and educational decline. Finally, we identified a need for an official information publication.

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Data availability

The datasets generated during and/or analysed during the current study are not publicly available due to being in Danish.

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Acknowledgements

We thank all participants in the study. We also thank paediatric oncologists Astrid Sehested and Karsten Nysom at the Department of Paediatrics and Adolescent Medicine, Copenhagen University Hospital Rigshospitalet, as well as paediatric oncologist Steen Rosthøj, Department of Paediatrics, Aalborg University Hospital, for the recruitment of patients. Further, we thank Anna Bolette Lund Nielsen, Anne Sofie Skov Faber, Joachim Birch Milan, Andreas Höier Aagesen, Trine Egegaard and Julie Rohold for their help with the transcription of the interviews.

Funding

This study is funded by the Danish Childhood Cancer Foundation (award number 2016-0143) and the Danish Cancer Society (award number R124-A7920-15-S2).

Author information

Authors and Affiliations

  1. Department of Neurosurgery and Paediatric Department, 2092, Copenhagen University Hospital Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark

    Morten Wibroe

  2. Department of Pediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark

    Marianne Vie Ingersgaard

  3. Department of Pediatrics and Adolescent Medicine, Copenhagen University Hospital, Rigshospitalet, Bonkolab 5704, Blegdamsvej 9, Copenhagen, Denmark

    Hanne Bækgaard Larsen

  4. Department of Neurosurgery, 2092, Copenhagen University Hospital Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark

    Marianne Juhler

  5. Department of Oncology, Center for Cancer and Organ diseases, Copenhagen University Hospital Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark

    Karin Piil

  6. Department of Public Health, Aarhus University, Aarhus, Denmark

    Karin Piil

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  1. Morten Wibroe
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  2. Marianne Vie Ingersgaard
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  3. Hanne Bækgaard Larsen
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  4. Marianne Juhler
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  5. Karin Piil
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Contributions

MW and MJ designed the study. MW, MVI and KP designed the interview guide. MW performed all interviews and drafted the first version of the manuscript. MVI, MW and KP performed the analyses. The allocation of categories and the resulting themes were discussed with HBL and MJ. All authors contributed to scientific revision of the manuscript and read and approved the final manuscript.

Corresponding author

Correspondence to Karin Piil.

Ethics declarations

All procedures performed in studies involving human participants were in accordance with the ethical standards of The Danish Health Authority (3-3013-1846/1/) and The Danish Committee on Health Research Ethics (17001523) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This study is registered by The Danish Health Authority (3-3013-1846/1/) and The Danish Committee on Health Research Ethics (17001523).

Consent to participate

Informed consent was obtained from all individual participants included in the study.

Consent for publication

Not applicable.

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Wibroe, M., Ingersgaard, M.V., Larsen, H.B. et al. Living with the cerebellar mutism syndrome: long-term challenges of the diagnosis. Acta Neurochir 163, 1291–1298 (2021). https://doi.org/10.1007/s00701-020-04479-3

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  • DOI: https://doi.org/10.1007/s00701-020-04479-3

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