Cerebellar mutism syndrome: the importance of preoperative language assessment

Abstract

Children undergoing surgical removal of tumors in the posterior cranial fossa can encounter a varied and complex constellation of neurological symptoms, called cerebellar mutism, defined as a disturbance in the planning and programming of motor language with preserved understanding, behavioral disorders such as inattention, visual-spatial disorganization, personality change, as well as ataxia and dysmetria. In the last years, several groups have been trying to establish risk factors or even predictive scores in order to be able at least in part to predict the appearance of speech disorders before surgery. We report on a child with pilocytic astrocytoma of the cerebellar vermis who had already been diagnosed with developmental linguistic delay two years earlier. This disorder initially worsened after surgery and later improved in the following 12 months. The aim of this paper is to emphasize the importance of preoperative neuropsychological evaluation. The present case, along with those reported in the literature, suggests that the risk of long-term cerebellar mutism is higher in children with preoperative speech disorders. In these patients a thorough assessment of cognitive and linguistic functions is therefore necessary to better evaluate the risk of cerebellar mutism after surgery.

Keywords: Cerebellar mutism syndrome; Preoperative neurocognitive assessment.

Fig. 1

T1-weighted, gadolinium-enhanced coronal, sagittal, and axial cerebral MRI showing a large vermian mass with well-defined margins of approximately 57 × 50 × 44 mm. The mixed structure with a solid, gadolinium-enhancing nodule and multi-lobed cystic component was consistent with the diagnosis of pilocytic astrocytoma. Triventricular hydrocephalus and initial descent of the cerebellar tonsils are also evident