The Neurocognitive Profile of Post-operative Paediatric Cerebellar Mutism Syndrome: A Systematic Review

AIM:To systematically review neurocognitive outcomes associated with Post-operative Paediatric Cerebellar Mutism Syndrome (PPCMS), comparing children with and without PPCMS after posterior fossa tumour surgery, and in relation to moderating demographic and clinical risk factors.METHODSPsycInfo, Medline and Embase databases were systematically searched up to December 2024. Studies of children aged 2-18 years with PPCMS who had undergone standardised 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.RESULTSSixteen studies (PPCMS+n=252, PPCMS-n=590) met criteria for inclusion. Children who experience PPCMS were found to have pronounced, long-term neurocognitive impairments with severely affected processing speed, psychomotor and executive function, and poorer neurocognitive outcomes generally compared to children without PPCMS. Current literature is limited by small samples, lack of diagnostic clarity or routine prospective screening of PPCMS, and limited investigation of factors that may moderate neurocognitive outcomes.INTERPRETATIONChildren with PPCMS have increased vulnerability to neurocognitive impairments which persist beyond the recovery of initial PPCMS symptoms in the post-operative phase. Dedicated research is needed to further our understanding of PPCMS and associated neurocognitive outcomes to inform clinical care.