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后颅窝肿瘤患儿共济失调的发病率及预后因素

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

作者信息

Hartley Helen, Pizer Barry, Lane Steven, Sneade Christine, Williams Rebecca, Mallucci Conor, Bunn Lisa, Kumar Ram

机构信息

Physiotherapy Department, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.

Oncology Department, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.

出版信息

Neurooncol Pract. 2019 May;6(3):185-193. doi: 10.1093/nop/npy033. Epub 2018 Aug 21.

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.

摘要

背景

尽管共济失调在患有后颅窝肿瘤(PFT)的儿童群体中是一个重大问题,但针对该群体运动结局的文献却极少。本研究旨在报告PFT治疗后儿童的身体结局,并确定哪些因素会影响共济失调的严重程度和功能限制。

方法

使用共济失调评估与分级量表(SARA)、简易共济失调评定量表(BARS)以及残疾儿童评定量表(PEDI)中的运动亚量表,对42例年龄在5至17岁之间以及PFT手术后1至4年的儿童进行评估,以确定共济失调的患病率和严重程度以及身体功能指标。分析比较肿瘤位置、肿瘤组织学类型、辅助治疗、诊断时年龄、术前是否存在共济失调以及是否存在小脑缄默综合征(CMS)对共济失调和身体功能评分的影响。

结果

71%的儿童SARA和BARS评分大于2。共有48%的儿童PEDI-m评分大于90。诊断时年龄或术前共济失调与评估评分之间无相关性。根据肿瘤组织学类型、肿瘤位置和CMS的存在情况,SARA/BARS和PEDI-运动评分存在显著差异。

结论

PFT手术后超过1年的儿童中,很大一部分仍存在共济失调。髓母细胞瘤和中线肿瘤患儿以及诊断为CMS的患儿共济失调程度更高,身体功能评分更低。共济失调的高患病率表明需要对该人群的康复管理进行进一步研究。

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