Abstract

IntroductionTumors 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 reviewThe 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.

Highlights

  • Tumors of the cerebellum are the most common brain tumors in children

  • While hydrocephalus was not identified as significantly different across groups, the incidence of persistent hydrocephalus was strongly correlated with language impairment. These findings strongly indicate the need for careful assessment of speech and language both prior to and following surgery

  • Children who present with Post-operative cerebellar mutism syndrome (pCMS) are at substantial risk for greater long-term cognitive and psychosocial morbidity, making them an important group for which interventions and rehabilitation beyond those focusing exclusively on motor and speech functions should be prioritized in the scientific community

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Summary

Introduction

Transient and total cerebellar-induced speechlessness is a complication of cerebellar tumor surgery. In a long-term follow-up study of 24 patients after cerebellar tumor surgery of whom 12 had suffered from pCMS, De Smet et al observed ataxic speech symptoms in less than 25% [16]. Children are often left with persistent dysarthria, language impairment, and dysphagia, among other neurological deficits [15, 18, 21, 36] In their long-term follow-up study, De Smet et al found that all children with pCMS exhibited dysarthria in the immediate postmutism phase, and 91.7% of them had persistent motor speech deficits up to 12 years after surgery [16]. It is recommended that rehabilitation of speech and language in children with pCMS involves multi-phase implementation of evidencebased guidelines and recommendations that identify and highlight risk factors, specific deficits, and knowledge about progression over time [9, 13, 38]. Identify risks of late effects on speech and language, where children tend to grow into their deficits, and the impact on quality of life

Behavioral and cognitive disturbances during pCMS and course of recovery
Cognitive rehabilitation
Pharmacological interventions
Physical exercise
Motor disturbances in the context of pCMS
Findings
Rehabilitation of cerebellar motor problems during and after pCMS

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