Neurocognitive outcomes following postoperative paediatric cerebellar mutism syndrome: A systematic review
Bethany M Horne1,2, Annisha A Attanayake1, Kristian Aquilina3
1Neuropsychology Service, Psychological and Mental Health Services, Great Ormond Street Hospital, London, UK.
Insights
Children with postoperative paediatric cerebellar mutism syndrome (pCMS) experience severe, lasting neurocognitive deficits. Further research is crucial to understand pCMS and improve clinical care for affected children.
Area of Science:
- Neuroscience
- Pediatric Oncology
- Clinical Psychology
Background:
- Postoperative paediatric cerebellar mutism syndrome (pCMS) is a complex condition following posterior fossa tumour surgery.
- Neurocognitive outcomes in children with pCMS require systematic investigation.
Purpose of the Study:
- To systematically review neurocognitive outcomes in children with pCMS.
- Compare neurocognitive function between children with and without pCMS.
- Identify demographic and clinical risk factors moderating outcomes.
Main Methods:
- Systematic literature search of PsycInfo, Medline, and Embase up to December 2024.
- Inclusion of studies on children aged 2-18 years with pCMS and standardized neurocognitive assessment.
- Quality appraisal using established checklists; synthesis without meta-analysis.
Main Results:
- Sixteen studies including 252 children with pCMS and 590 without were analyzed.
- Children with pCMS exhibited significant, long-term impairments in processing speed, psychomotor, and executive functions.
- Literature is limited by small sample sizes, diagnostic ambiguity, and lack of prospective screening.
Conclusions:
- Children with pCMS demonstrate heightened vulnerability to persistent neurocognitive impairments.
- Neurocognitive deficits in pCMS extend beyond the acute postoperative period.
- Further research is essential to understand pCMS and its moderators for improved clinical management.
Aim:
To systematically review neurocognitive outcomes associated with postoperative paediatric cerebellar mutism syndrome (pCMS), comparing children with and without pCMS after posterior fossa tumour surgery, and in relation to moderating demographic and clinical risk factors.
Method:
PsycInfo, Medline, and Embase databases were systematically searched up to December 2024. Studies of children aged 2 to 18 years with pCMS who had undergone standardized neurocognitive assessment were included. Quality was appraised using Institute of Health Economics Quality Appraisal Checklist for Case Series and Quality In Prognosis Studies tools. Synthesis without meta-analysis was conducted.
Results:
Sixteen studies (n = 252 children presenting with pCMS, n = 590 without) met criteria for inclusion. Children who experienced pCMS were found to have pronounced, long-term neurocognitive impairments with severely affected processing speed, psychomotor and executive function, and poorer neurocognitive outcomes generally than children without pCMS. Current literature is limited by small samples, lack of diagnostic clarity or routine prospective screening of pCMS, and limited investigation of factors that may moderate neurocognitive outcomes.
Interpretation:
Children with pCMS have increased vulnerability to neurocognitive impairments which persist beyond the recovery of initial symptoms in the postoperative phase. Dedicated research is needed to further our understanding of the condition and moderators of neurocognitive outcomes to inform clinical care.
