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The post-operative pediatric cerebellar mutism syndrome (CMS) affects about one-third of children and adolescents following surgical removal of a posterior fossa tumor (PFT). According to the Posterior Fossa Society consensus working definition, CMS is characterized by delayed-onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children, and is frequently accompanied by additional features such as hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to develop a diagnostic scale to grade CMS duration and severity. Thirty consecutively referred subjects, aged 1–17 years (median 8 years, IQR 3–10), were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in case of CMS, for 30 days after the onset ( T  0 ) or until symptom remission. At day 30 ( T  1 ), CMS was classified into mild, moderate, or severe according to the proposed scale. CMS occurred in 13 patients (43%, 95% C.I.: 25.5–62.6%), with mild severity in 4 cases (31%), moderate in 4 (31%), and severe in 5 (38%). At  T  1 , longer symptom persistence was associated with greater severity ( p  = 0.01). Greater severity at  T  0  predicted greater severity at  T  1  ( p  = 0.0001). Children with a midline tumor location and those aged under 5 years at diagnosis were at higher risk of CMS ( p  = 0.025 and  p  = 0.008, respectively). In conclusion, the proposed scale is a simple and applicable tool for estimating the severity of CMS at its onset, monitoring its course over time, and providing an early prognostic stratification to guide treatment decisions.What is Known:  • Post-operative pediatric Cerebellar Mutism Syndrome (CMS) is a complex phenomenon with a wide   spectrum of symptoms that may manifest in children undergoing the resection of a posterior fossa tumor   (PFT) and that can result into long-term impairment.  What is New:  • This study developed and pilot-tested an easily applicable diagnostic and severity scale to grade the   duration and the severity of symptoms of the CMS.  • The proposed scale was found to be a sensitive instrument to identify even mild CMS presentations.  • By scoring not only the duration but also the severity of symptoms the scale allows a more accurate   prognostic stratification for an optimal planning of clinical and rehabilitative interventions.

Development and application of a diagnostic and severity scale to grade post-operative pediatric cerebellar mutism syndrome