Cerebellar mutism syndrome

Abstract

Cerebellar mutism most commonly, but not exclusively, develops in children after surgery for midline cerebellar or intraventricular tumors in the posterior fossa, typically medulloblastoma. Cerebellar mutism syndrome (CMS) comprises a complex set of neurologic and neurocognitive signs and symptoms, the cardinal and central component of which is an initially profound but usually reversible speech disorder. As such, CMS is currently recognized as an extreme form of the so-called cerebellar cognitive affective syndrome (Schmahmann syndrome). The putative cause of CMS is a substantial surgical injury to the proximal components of the bilateral efferent cerebellar pathways, disrupting cerebellar input to the supratentorial brain. The resultant cerebellocerebral diaschisis may lead to supratentorial cortical perfusion depression with frontal predominance. The speech disorder is, therefore, likely an apraxia. As our understanding of the mechanism and the clinical spectrum of CMS evolves, clinically useful preoperative risk stratification schemes, adjustments to surgical strategies and techniques, and possible early therapeutic-rehabilitative measures are being sought and developed to reduce the burden of this severe and particularly handicapping chronic morbidity on affected individuals and their families.

Keywords: cerebellar mutism syndrome; cerebellar tumor; child; diaschisis; efferent cerebellar pathway; posterior fossa surgery; speech apraxia.