Cerebellar mutism

Abstract

Cerebellar mutism - a term introduced by Rekate et al. in 1985 - can be considered as the hallmark feature of the posterior fossa syndrome (PFS). This syndrome was first described in detail by Daly and Love in 1958 and consists of specific linguistic, cognitive, behavioral, and affective symptoms following acute posterior fossa damage in children and adults. Although the syndrome has been exceptionally associated with non-tumoral etiologies, it usually develops after a brief period of relatively normal functioning in the immediate postoperative phase following posterior fossa tumor surgery. While the incidence of the PFS in the pediatric population is estimated to range between 8% and 31% after posterior fossa tumor resection, the condition is extremely rare in adults. As such, the PFS is a clinical condition typically affecting children operated for posterior fossa tumors. Although more than 300 etiologically heterogeneous cases of PFS have been reported in the literature and many hypotheses have been proposed to explain the PFS, the underlying pathophysiological mechanisms remain unclear. This chapter presents a brief overview of the intriguing semiological combination of transient cerebellar mutism and cognitive, behavioral, and affective alterations following acute posterior fossa lesions. Furthermore, the most important pathophysiological hypotheses will be briefly discussed.