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Cerebellar Mutism/Posterior Fossa Syndrome Following Resection of Posterior Fossa Tumor in Pediatric Patients:
Vikrant Setia1, Monirah Zeya2, Arvind Kumar Srivastava2
1Department of Neurosurgery, Geetanjali Medical College and Hospital, Udaipur, Rajasthan.
Insights
Cerebellar mutism syndrome (CMS) occurred in 20% of pediatric posterior fossa tumor patients, often linked to brainstem involvement and resulting in delayed recovery. Dysarthria followed mutism, indicating transient pathway impairment.
Area of Science:
- Pediatric neurosurgery
- Neuro-oncology
- Clinical neurology
Background:
- Cerebellar mutism syndrome (CMS) is a postoperative complication in children with posterior fossa tumors, affecting speech and neurobehavior.
- The incidence of CMS can be as high as 40%.
Purpose of the Study:
- To evaluate the incidence, clinical characteristics, pathophysiology, risk factors, and neuroradiographic features of CMS in pediatric patients with posterior fossa tumors.
- To identify predictors for CMS development and understand its impact on patient outcomes.
Main Methods:
- A cohort of 60 pediatric patients with posterior fossa tumors undergoing surgery via a telovelar approach were studied.
- Detailed pre- and postoperative clinical, radiological, and pathological evaluations were performed to identify risk factors and neuroradiographic features associated with CMS.
- Patients with CMS were compared to those without to determine significant risk factors.
Main Results:
- The incidence of CMS in this cohort was 20%, often accompanied by neurobehavioral abnormalities.
- Brainstem and related structure involvement was a significant risk factor (p=0.03).
- Postoperative brainstem/peduncular edema (p=0.04) and 1-year posterior fossa atrophy (p=0.01) were associated with CMS development. Cerebellar dysarthria followed mutism in all cases (p<0.001), and CMS patients had poorer outcomes.
Conclusions:
- CMS likely results from transient impairment of dentate nuclei pathways, associated with brainstem involvement and leading to poor functional outcomes.
- Neuroradiographic features, including postoperative edema and long-term atrophy, are crucial in understanding CMS pathophysiology and predicting outcomes.
Background:
Cerebellar mutism syndrome (CMS) is a postoperative syndrome of decreased speech seen in children associated with neurobehavioral abnormalities, the incidence of which is up to 40%.
Objectives:
To evaluate pediatric patients with posterior fossa tumors for incidence, clinical characteristics, pathophysiology, risk factors, and neuroradiographic features of this syndrome.
Materials And Methods:
The study included 60 pediatric patients with a posterior fossa tumor who underwent surgery by a telovelar approach. Detailed pre- and postoperative clinical and radiological evaluations were done. Patients with CMS were analyzed and compared with those without mutism to find risk factors for CMS. The presentation and characteristics of cerebellar mutism were studied along with the following risk factors:Clinical-age, sex, cranial nerve deficit, and adjuvant treatment.Radiological-tumor location, hydrocephalus, brainstem invasion, extent of tumor resection, peduncular and brainstem edema, and atrophy of posterior fossa structures.Pathological-histopathology of tumor.The preoperative, immediate postoperative, and 1-year postoperative imaging results were reviewed to assess the neuroradiographic features in the two groups.
Results:
The incidence of this syndrome was 20%. The mutism was accompanied by some neurobehavioral abnormalities ( p -value = 0.05). The most significant finding was the presence of a period of cerebellar dysarthria after the resolution of the muteness ( p -value < 0.001) in all cases. Brainstem and related structures' involvement was the most significant risk factor ( p -value = 0.03). The presence of brainstem and peduncular edema in the immediate postoperative period ( p -value = 0.04) and gross atrophy of posterior fossa structures at 1 year ( p -value = 0.01) showed significance toward the development of CMS. There was delayed neurological recovery in patients with CMS with a poor Glasgow Outcome Score at 1 year of follow-up.
Conclusion:
The clinical presentation of this syndrome in context with neuroradiographic features suggests that it results from transient impairment of the afferent and/or efferent pathways of dentate nuclei that are involved in initiating complex volitional movements and are associated with brainstem involvement of tumor and poor functional outcome.

