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The posterior fossa syndrome questionnaire: using science to inform practice

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Abstract

Introduction

Up to 34% of patients with medulloblastoma develop posterior fossa syndrome (PFS) following brain tumor resection and have increased risk of long-term neurocognitive impairments. Lack of agreement in conceptualization and diagnosis of PFS calls for improvements in diagnostic methods. The current study aimed to describe psychometric properties of a new posterior fossa syndrome questionnaire (PFSQ).

Methods

The PFSQ was informed by prior research and developed by a multidisciplinary team with subject matter expertise. Participants (N = 164; 63.4% Male; 78.7% White; Mage at diagnosis = 10.38 years, SD = 5.09, range 3–31 years) included patients with newly diagnosed medulloblastoma enrolled in the SJMB12 clinical trial. Forty-four patients (26.8%) were classified as having PFS based on attending physician’s post-surgical yes/no report. A PFSQ was completed by a neurologist within 2 weeks of coming to St. Jude Children’s Research Hospital for adjuvant treatment, irrespective of suspicion for PFS.

Results

PFSQ items ataxia (100.00%), dysmetria (95.45%), and speech/language changes (79.55%) were most sensitive. However, ataxia (26.50%) and dysmetria (46.61%) demonstrated low specificity. Speech/language changes (81.36%), mutism (95.76%), orofacial apraxia (98.29%) and irritability (96.61%) had high specificity. A principal component analysis found four components: (1) speech/language changes, (2) apraxias (including mutism), (3) motor/oromotor, and (4) emotional lability.

Conclusions

The PFSQ is a dimensional diagnostic approach that can be used to improve diagnostic consistency across clinical and research groups to help accelerate understanding of PFS etiology, identify surgical correlates of risk, predict long-term impairments, and develop targeted interventions. Additional measure validation, including correlation with symptom resolution, is required.

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Data availability

The datasets analyzed during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

The authors thank the patients and families who participated in the SJMB12 clinical trial. We also thank our multidisciplinary team members for their valuable contributions to this work.

Funding

This work was supported, in part, by the National Cancer Institute (St. Jude Cancer Center Support [CORE] Grant [P30-CA21765]) and the American Lebanese Syrian Associated Charities (ALSAC).

Author information

Authors and Affiliations

  1. Department of Psychology, University of Mississippi, University, MS, USA

    Molly E. Wickenhauser

  2. Division of Neurology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Raja B. Khan

  3. Department of Psychology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Darcy Raches, Jason M. Ashford, Kathryn M. W. Russell & Heather M. Conklin

  4. Rehabilitation Services, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Kristin Lyons

  5. Division of Neuro-Oncology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    Giles W. Robinson & Amar Gajjar

  6. Department of Neurosurgery, University of Tennessee, Memphis, TN, USA

    Paul Klimo Jr

Authors
  1. Molly E. Wickenhauser
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  2. Raja B. Khan
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  3. Darcy Raches
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  4. Jason M. Ashford
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  7. Giles W. Robinson
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  8. Amar Gajjar
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  10. Heather M. Conklin
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Contributions

RBK, DR, and HMC originally conceived the idea for the study and acquired study data with the help of GWR, AG, and KL. MEW, JMA, KMWR, and HMC were involved in all aspects of the study including design of the work, data analysis, interpretation, and final manuscript preparation. All authors contributed to writing and editing, and have read and approved the final manuscript.

Corresponding author

Correspondence to Heather M. Conklin.

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Conflict of interest

The authors have no relevant financial or non-financial interests to disclose.

Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of St. Jude Children’s Research Hospital.

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Wickenhauser, M.E., Khan, R.B., Raches, D. et al. The posterior fossa syndrome questionnaire: using science to inform practice. J Neurooncol 157, 465–473 (2022). https://doi.org/10.1007/s11060-022-03990-0

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  • DOI: https://doi.org/10.1007/s11060-022-03990-0

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