Abstract：
BACKGROUND: The most common manifestation of X-linked adrenoleukodystrophy (ALD) is a slowly progressive myeloneuropathy, which leads to imbalance and gait disturbances. The variable progression of the disease complicates evaluation of its progression rate. Wearable sensors allow for easy and frequent balance and gait collection. This study reports baseline data from a longitudinal study on the quantitative assessment of balance and gait with wearable sensors and their clinical relevance.
METHODS: Data were collected from adult patients in two institutions. Postural body sway and gait parameters were measured using accelerometers. Disease severity was measured by the Expanded Disability Severity Scale (EDSS). Falling frequency and quality of life (QOL) were collected in men. The relationship between sway and gait variables and EDSS score, participants\' use of a walking aid, and falling frequency was evaluated.
RESULTS: One hundred twenty individuals with ALD were included. Sway variables significantly differentiate participants\' assistive device use. Sway and gait variables were correlated to the EDSS in both sexes. Both gait speed and sway were correlated with falling frequency in men from one institution. Select QOL subscores were correlated with the EDSS in males from one institution. Accelerometry generated comparable results across sites.
CONCLUSIONS: This study confirms the clinical correlation between spinal cord disease and imbalance and gait in ALD. For the first time, this study shows clinically meaningful relationships for sway and gait with use of an assistive device, falling frequency and QOL. Wearable accelerometers are a valid means to measure sway and gait in ALD. These measures are promising outcomes for clinical trial designs to assess myeloneuropathy in ALD and to monitor disease progression in individuals.
METHODS: Data were collected from adult patients in two institutions. Postural body sway and gait parameters were measured using accelerometers. Disease severity was measured by the Expanded Disability Severity Scale (EDSS). Falling frequency and quality of life (QOL) were collected in men. The relationship between sway and gait variables and EDSS score, participants\' use of a walking aid, and falling frequency was evaluated.
RESULTS: One hundred twenty individuals with ALD were included. Sway variables significantly differentiate participants\' assistive device use. Sway and gait variables were correlated to the EDSS in both sexes. Both gait speed and sway were correlated with falling frequency in men from one institution. Select QOL subscores were correlated with the EDSS in males from one institution. Accelerometry generated comparable results across sites.
CONCLUSIONS: This study confirms the clinical correlation between spinal cord disease and imbalance and gait in ALD. For the first time, this study shows clinically meaningful relationships for sway and gait with use of an assistive device, falling frequency and QOL. Wearable accelerometers are a valid means to measure sway and gait in ALD. These measures are promising outcomes for clinical trial designs to assess myeloneuropathy in ALD and to monitor disease progression in individuals.
摘要：
背景：X连锁肾上腺脑白质营养不良（ALD）最常见的表现是缓慢进行的脊髓神经病变，导致不平衡和步态紊乱。疾病的可变进展使其进展速率的评估复杂化。可穿戴传感器允许容易和频繁的平衡和步态收集。本研究报告了使用可穿戴传感器定量评估平衡和步态及其临床相关性的纵向研究的基线数据。
方法：数据来自两个机构的成年患者。使用加速度计测量姿势身体摇摆和步态参数。通过扩展残疾严重程度量表(EDSS)测量疾病严重程度。收集男性的下降频率和生活质量（QOL）。摇摆和步态变量与EDSS得分之间的关系，参与者使用助行器，并对下降频率进行了评估。
结果：纳入120名ALD患者。摇摆变量显著区分参与者辅助设备的使用。男女的摇摆和步态变量与EDSS相关。步态速度和摇摆都与一个机构的男性跌倒频率相关。选择QOL子得分与一个机构的男性的EDSS相关。加速计在各个站点之间产生了可比的结果。
结论：这项研究证实了脊髓疾病与ALD中的不平衡和步态之间的临床相关性。第一次,这项研究显示了使用辅助装置的摇摆和步态的临床意义的关系，下降频率和QOL。可穿戴加速度计是在ALD中测量摇摆和步态的有效手段。这些措施是临床试验设计的有希望的结果，以评估ALD中的骨髓神经病和监测个体的疾病进展。
方法：数据来自两个机构的成年患者。使用加速度计测量姿势身体摇摆和步态参数。通过扩展残疾严重程度量表(EDSS)测量疾病严重程度。收集男性的下降频率和生活质量（QOL）。摇摆和步态变量与EDSS得分之间的关系，参与者使用助行器，并对下降频率进行了评估。
结果：纳入120名ALD患者。摇摆变量显著区分参与者辅助设备的使用。男女的摇摆和步态变量与EDSS相关。步态速度和摇摆都与一个机构的男性跌倒频率相关。选择QOL子得分与一个机构的男性的EDSS相关。加速计在各个站点之间产生了可比的结果。
结论：这项研究证实了脊髓疾病与ALD中的不平衡和步态之间的临床相关性。第一次,这项研究显示了使用辅助装置的摇摆和步态的临床意义的关系，下降频率和QOL。可穿戴加速度计是在ALD中测量摇摆和步态的有效手段。这些措施是临床试验设计的有希望的结果，以评估ALD中的骨髓神经病和监测个体的疾病进展。
