Masoud Melanie, Gordon Kelly, Hall Amanda, Jasien Joan, Lardinois Kara, Uchitel Julie, Mclean Melissa, Prange Lyndsey, Wuchich Jeffrey, Mikati Mohamad A
Duke University Health System, Duke University School of Medicine, Durham, NC, USA.
Department of Speech Pathology and Audiology, Duke University Health System, Durham, NC, USA.
Dev Med Child Neurol. 2017 Aug;59(8):822-828. doi: 10.1111/dmcn.13443. Epub 2017 May 25.
To characterize motor function profiles in alternating hemiplegia of childhood, and to investigate interrelationships between these domains and with age.
We studied a cohort of 23 patients (9 males, 14 females; mean age 9y 4mo, range 4mo-43y) who underwent standardized tests to assess gross motor, upper extremity motor control, motor speech, and dysphagia functions.
Gross Motor Function Classification System (GMFCS), Gross Motor Function Measure-88 (GMFM-88), Manual Ability Classification System (MACS), and Revised Melbourne Assessment (MA2) scales manifested predominantly mild impairments; motor speech, moderate to severe; Modified Dysphagia Outcome and Severity Scale (M-DOSS), mild-to moderate deficits. GMFCS correlated with GMFM-88 scores (Pearson's correlation, p=0.002), MACS (p=0.038), and MA2 fluency (p=0.005) and accuracy (p=0.038) scores. GMFCS did not correlate with motor speech (p=0.399), MA2 dexterity (p=0.247), range of motion (p=0.063), or M-DOSS (p=0.856). Motor speech was more severely impaired than the GMFCS (p<0.013). There was no correlation between any of the assessment tools and age (p=0.210-0.798).
Our data establish a detailed profile of motor function in alternating hemiplegia of childhood, argue against the presence of worse motor function in older patients, identify tools helpful in evaluating this population, and identify oropharyngeal function as the more severely affected domain, suggesting that brain areas controlling this function are more affected than others.
描述儿童交替性偏瘫的运动功能特征，并研究这些领域之间以及与年龄的相互关系。
我们研究了一组23例患者（9例男性，14例女性；平均年龄9岁4个月，范围4个月至43岁），他们接受了标准化测试以评估粗大运动、上肢运动控制、运动性言语和吞咽功能。
粗大运动功能分类系统（GMFCS）、粗大运动功能测量-88（GMFM-88）、手动能力分类系统（MACS）和修订版墨尔本评估（MA2）量表主要表现为轻度损伤；运动性言语为中度至重度；改良吞咽结果和严重程度量表（M-DOSS）为轻度至中度缺陷。GMFCS与GMFM-88评分（Pearson相关性，p=0.002）、MACS（p=0.038）以及MA2流畅性（p=0.005）和准确性（p=0.038）评分相关。GMFCS与运动性言语（p=0.399）、MA2灵活性（p=0.247）、关节活动范围（p=0.063）或M-DOSS（p=0.856）无关。运动性言语比GMFCS受损更严重（p<0.013）。任何评估工具与年龄之间均无相关性（p=0.210-0.798）。
我们的数据建立了儿童交替性偏瘫运动功能的详细特征，反对老年患者存在更差运动功能的观点，确定了有助于评估该人群的工具，并确定口咽功能是受影响更严重的领域，表明控制该功能的脑区比其他脑区受影响更大。
