Incidence and prognostic factors of ataxia in children with posterior fossa tumors

Abstract

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.

Post-operative cerebellar mutism syndrome: rehabilitation issues

“…Ataxia is often present in children pre-operatively (reported as evident in 60% of children in a meta-analysis by Wilne et al [68]). Ataxia is then thought to increase post-operatively with pCMS highlighted as a risk factor for both more severe [23] and long-term ataxias [29]. Specific tumor location is also noted to be a risk factor for long-term ataxia with involvement of the deep cerebellar nuclei, a predictor for problems over a year after surgery [29].…”

“…Specific tumor location is also noted to be a risk factor for long-term ataxia with involvement of the deep cerebellar nuclei, a predictor for problems over a year after surgery [29]. Overall, there is a lack of understanding regarding the recovery potential for children with motor problems in conjunction with pCMS although recent studies suggest that children who were diagnosed with pCMS are likely to have more often long-term therapy needs [23,27].…”

Endocrine late-effects and bone mineral density after combined treatment of malignant brain tumors in childhood and adolescence

“…Высокая распространенность остеопенических состояний определяет актуальность и необходимость изучения данной проблемы с целью оптимизации ранней диагностики для предотвращения тяжелых осложнений в будущем. Учитывая, что у пациентов с опухолями головного мозга, особенно с локализацией в области задней черепной ямки и мозжечка, нередко наблюдается атаксия, проявляющаяся расстройствами поддержания равновесия и ходьбы, пошатыванием и нарушениями координации движений [ 29 ], в сочетании со сниженной МПК существенно увеличивается риск переломов даже в молодом возрасте.…”

Ataxia and mobility in children following surgical resection of posterior fossa tumour: A longitudinal cohort study

“…Sønderkær et al [ 6 ] reported that ataxia may persist at 10 years after surgery. More recent reporting with standardised outcome measures has also observed approximately 70% [ 7 , 8 ] of children with PFT which demonstrate balance dysfunction more than 1 year following surgery.…”