Unità Operativa Riabilitazione Neuroncologica, Scientific Institute, IRCCS E. Medea, Lecco, Italy.
Bioengineering lab, Scientific Institute IRCCS Eugenio Medea, Lecco, Italy.
Pediatr Hematol Oncol. 2020 Nov;37(8):687-695. doi: 10.1080/08880018.2020.1785600. Epub 2020 Jul 24.
Posterior cranial fossa (PCF) tumors in childhood are often associated with ataxia as well as other motor, neurobehavioral and linguistic impairment. The use of a reliable outcome measure is mandatory to evaluate the severity of impairment and monitor rehabilitation effectiveness. The aim of this work is to explore the validity of the Scale for the Assessment and Rating of Ataxia (SARA) in pediatric subjects with ataxia secondary to PCF tumor resection and evaluate the influence of age and comorbidities. Seventy eight patients (3-18 years) were recruited in 5 centers from 2016 to 2018. The age effect on SARA was analyzed by correlating total SARA scores and item scores with age and gradually excluding youngest subjects. The comorbidity effect was evaluated by comparing the ataxia-only group vs a group of subjects with ataxia + dysfunction of cranial nerves or cerebellar mutism (CM) and a group of patients with ataxia + hemiparesis. Several negative correlations between SARA scores and age were found under age 9. Differences between ataxia-only group and the other two groups were closely associated with specific comorbidities (e.g. speech disturbance in cranial nerves or CM group (p value < 0.001) and gait, stance, sitting and finger chase in the hemiparetic group (mean p value 0.022)).
儿童后颅窝 (PCF) 肿瘤常伴有共济失调以及其他运动、神经行为和语言障碍。为了评估损伤的严重程度并监测康复效果，使用可靠的结局测量方法是强制性的。本研究旨在探讨共济失调评估和评分量表 (SARA) 在继发于 PCF 肿瘤切除的小儿患者中的有效性，并评估年龄和合并症的影响。2016 年至 2018 年期间，我们在 5 家中心招募了 78 名患者（3-18 岁）。通过将总 SARA 评分和项目评分与年龄相关联，并逐渐排除最小的患者，分析年龄对 SARA 的影响。通过比较仅存在共济失调的患者与存在共济失调+颅神经功能障碍或小脑缄默（CM）的患者以及存在共济失调+偏瘫的患者，评估合并症的影响。在 9 岁以下，SARA 评分与年龄之间存在多个负相关。仅存在共济失调组与后两组之间的差异与特定的合并症密切相关（例如，颅神经或 CM 组存在言语障碍（p 值 < 0.001），偏瘫组存在步态、站立、坐姿和手指追逐障碍（均值 p 值为 0.022））。
