The SARA and BARS are reliable and valid measures and appear to be of equal value in determining the severity of ataxia in children with posterior fossa tumours.

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 effectiveness of exercise and physical therapy for children with ataxia is poorly understood. The aim of this systematic review was to critically evaluate the range, scope and methodological quality of studies investigating the effectiveness of exercise and physical therapy interventions for children with ataxia. The following databases were searched: AMED, CENTRAL, CDSR, CINAHL, ClinicalTrials.gov, EMBASE, Ovid MEDLINE, PEDro and Web of Science. No limits were placed on language, type of study or year of publication. Two reviewers independently determined whether the studies met the inclusion criteria, extracted all relevant outcomes, and conducted methodological quality assessments. A total of 1988 studies were identified, and 124 full texts were screened. Twenty studies were included in the review. A total of 40 children (aged 5–18 years) with ataxia as a primary impairment participated in the included studies. Data were able to be extracted from eleven studies with a total of 21 children (aged 5–18 years), with a range of cerebellar pathology. The studies reported promising results but were of low methodological quality (no RCTs), used small sample sizes and were heterogeneous in terms of interventions, participants and outcomes. No firm conclusions can be made about the effectiveness of exercise and physical therapy for children with ataxia. There is a need for further high-quality child-centred research.Electronic supplementary materialThe online version of this article (10.1007/s12311-019-01063-z) contains supplementary material, which is available to authorized users.

Background There is minimal literature specific to motor outcomes in children with posterior fossa tumors (PFTs) despite ataxia being a significant problem in this group. This study aims to report children’s physical outcomes following management of PFT and determine which factors affect severity of ataxia and functional limitations. Methods Forty-two children aged between 5 and 17 and between 1 and 4 years following surgery for PFT were assessed using the Scale for the Assessment and Rating of Ataxia (SARA), the Brief Ataxia Rating Scale (BARS), and the mobility Pediatric Evaluation of Disability Inventory (PEDI) subscale to determine prevalence and severity of ataxia and a measure of physical function. Analysis was undertaken comparing impact of tumor location, tumor histology, adjuvant treatment, age at diagnosis, presence of preoperative ataxia, and presence of cerebellar mutism syndrome (CMS) on ataxia and physical function scores. Results Seventy-one percent of children demonstrated a SARA and BARS score greater than 2. A total of 48% of children had a PEDI-m score greater than 90. There was no correlation between age at diagnosis or preoperative ataxia and assessment scores. There was a significant difference in SARA/BARS and PEDI-mobility scores depending on tumor histology, tumor location, and presence of CMS. Conclusions A high proportion of children (>1 year) following surgery for PFT continue to present with ataxia. Higher ataxia and lower physical function scores were demonstrated in children with medulloblastoma and midline tumors and those diagnosed with CMS. The high prevalence of ataxia demonstrates the need for further research regarding rehabilitation management in this population.

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