Post-operative cerebellar mutism syndrome: rehabilitation issues

Abstract

Introduction Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language. Post-operative cerebellar mutism syndrome (pCMS) is a well-known and frequently occurring complication of cerebellar tumor surgery in children. In the acute stage, children with pCMS may show deterioration of cerebellar motor function as well as pyramidal and cranial neuropathies. Most debilitating is the mutism or the severe reduction of speech and a range of neurobehavioral symptoms that may occur. In the long term, children that recover from pCMS continue to have more motor, behavioral, and cognitive problems than children who did not develop pCMS after cerebellar tumor surgery. The severity of these long-term sequelae seems to be related to the length of the mute phase. Aim of this narrative review The impact of pCMS on patients and families cannot be overstated. This contribution aims to discuss the present knowledge on the natural course, recovery, and rehabilitation of children with pCMS. We suggest future priorities in developing rehabilitation programs in order to improve the long-term quality of life and participation of children after cerebellar tumor surgery and after pCMS in particular.

The Clinical and Prognostic Impact of the Choice of Surgical Approach to Fourth Ventricular Tumors in a Single-Center, Single-Surgeon Cohort of 92 Consecutive Pediatric Patients

“…Immediately after the transient mute phase, almost all children experience dysarthria (30,31), as we have also seen in our patients (90% of 10 CM cases, in our cohort), with long-term persistence of motor speech deficits in 60% of our cases, according to other reports (30).…”

“…The severity of associated neuropsychological deficits after surgery was found to be a negative prognostic factor for long-term motor speech deficits, in terms of clinical/neuropsychological impact ( 30 ). Full recovery is often incomplete in cases of apraxic dysarthria ( 30 , 31 ).…”

Mapping of long-term cognitive and motor deficits in pediatric cerebellar brain tumor survivors into a cerebellar white matter atlas

“…In case of impending high risk for damage of critical cerebro-cerebellar loops, these methods may open a window for neo-adjuvant antitumoral therapy that could contribute to complete tumor resection without compromising integrity of deep cerebellar nuclei and the SCP [47,48]. Future studies will have to address gene polymorphisms determining neurodegeneration as well as neuroregeneration [49] next to neuroprotective and neurorehabilitation measures that contribute to the pattern of functional deficits [50].…”

Posterior Fossa Tumor Rehabilitation: An Up-to-Date Overview

“…It is also much more common for children to have posterior fossa syndrome, as the cerebellum is one of the most frequent locations in pediatric brain tumors under 10 years of age [ 40 ]. Currently, there is no validated efficacy protocol for pCMS rehabilitation [ 41 ].…”

“…For this reason, it is desirable to develop programs aimed at the longitudinal observation of patients in rehabilitation. As previously discussed, Paquier [ 41 ] describes two types of rehabilitative approaches: those face-to-face-oriented to single cognitive domains and those characterized by the use of computers that also enable an home setting. From the face-to-face side, the rehabilitation program that has been proved most effective to date is a phase 3 study with a multidimensional approach.…”