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Heralding cerebellar mutism: evidence for pre-surgical language impairment as primary risk factor in posterior fossa
Concezio Di Rocco1, Daniela Chieffo, Paolo Frassanito
1Paediatric Neurosurgery, Catholic University Medical School, Policlinic A. Gemelli, Largo Agostino Gemelli, 8, 00168, Rome, Italy.
Insights
Pre-operative language impairment in children with posterior fossa tumors significantly increases the risk of developing cerebellar mutism syndrome (CMS) after surgery. Normal pre-operative language function protected against CMS development.
Area of Science:
- Pediatric Neuro-oncology
- Neuropsychology
- Neurosurgery
Background:
- Cerebellar mutism syndrome (CMS) is a serious post-operative complication in children following posterior fossa tumor resection.
- Identifying risk factors for CMS is crucial for improving patient outcomes and tailoring pre-operative and post-operative care.
- Pre-existing language impairment (PLI) has been anecdotally suggested as a potential risk factor, but requires further investigation.
Purpose of the Study:
- To identify risk factors for cerebellar mutism syndrome (CMS) in pediatric patients undergoing posterior cranial fossa tumor surgery.
- To investigate the relationship between pre-operative language impairment (PLI) and the development of CMS.
- To evaluate the impact of posterior fossa tumor resection on pre-operative language deficits.
Main Methods:
- Prospective study of 34 children (aged 2-16) with posterior fossa tumors between 2006 and 2008.
- Inclusion criteria excluded very young children, older adolescents, and those with severe pre-operative conditions.
- Standard neurological and radiological evaluations, supplemented by comprehensive pre-operative and post-operative neuropsychological assessments focusing on language function.
Main Results:
- 11 out of 34 children (32.4%) presented with pre-operative language impairment (PLI).
- Seven children (20.6%) developed post-operative cerebellar mutism syndrome (CMS), all of whom were from the PLI group (63% incidence within the PLI group).
- No CMS occurred in children with normal pre-operative language function. PLI regressed in 3/4 of non-CMS patients with pre-operative deficits.
Conclusions:
- Pre-operative language impairment is a significant risk factor for developing cerebellar mutism syndrome after posterior fossa tumor resection.
- PLI may represent a subclinical manifestation of CMS in some pediatric patients with posterior fossa tumors.
- While surgery can improve linguistic function in some with PLI, those with normal pre-operative language are protected from post-operative mutism.
Abstract:
The aim of this study is to identify possible risks factors for the occurrence of cerebellar mutism syndrome (CMS) in children with posterior cranial fossa tumours. Children diagnosed with posterior fossa tumours consecutively admitted to our institution between 2006 and 2008 were the subjects of this prospective study. Besides standard neurological and radiological evaluations, all children underwent thorough neuropsychological assessments at admission and following surgery. Children under two or older than 16 years of age and those with a severe pre-operative clinical condition precluding neuropsychological assessment were excluded. Thirty-four children met the inclusion criteria. They were divided into two groups. Group I consisted of 23 children with normal language on admission and group II had 11 children showing pre-operative language impairment (PLI). PLI was observed in 11 children (32.4%: group II). Post-operatively, seven out of 34 children developed CMS (20.6%), all of them belonging to group II. In group II, indeed, the incidence of CMS was 63%. No case of CMS was observed in group I. PLI regressed after the operation in three out of the four subjects belonging to group II who did not develop CMS. PLI remained unchanged in the last child of this group. Posterior fossa tumour resection can have different effects on children with pre-existing language impairment (PLI). PLI can be considered a subclinical state of CMS in some children with posterior fossa tumour. However, in some children with PLI, the tumour resection may improve the linguistic abilities, as well as the other neurocognitive performances. In the present series, children with normal pre-operative language function did not develop post-operative mutism.
