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首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Muscle strength and motor function throughout life in a cross‐sectional cohort of 180 patients with spinal muscular atrophy types 1c–4
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Muscle strength and motor function throughout life in a cross‐sectional cohort of 180 patients with spinal muscular atrophy types 1c–4

机译：肌肉力量和运动功能在整个横截面积为180名脊柱肌萎缩类型1C-4患者的横断面队列

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Background and purpose Natural history studies in spinal muscular atrophy ( SMA ) have primarily focused on infants and children. Natural history studies encompassing all age groups and SMA types are important for the interpretation of treatment effects of recently introduced survival motor neuron gene‐augmenting therapies. Methods We conducted a cross‐sectional study to investigate muscle strength, Hammersmith Functional Motor Scale (Expanded) score and the patterns of muscle weakness in relation to age and SMA type. Results We included 180 patients with SMA types 1–4 in the age range 1–77.5?years with median disease duration of 18 (range 0–65.8)?years. With the exception of the early phases of disease in which children with SMA types 2 and 3 may achieve new motor skills and show a temporary increase in muscle strength, cross‐sectional data suggested that declining muscle strength and loss of motor skills over time are characteristic of all SMA types. Mean loss of strength was at least 1 point on the Medical Research Council score and 0.5 point on the Hammersmith Functional Motor Scale (Expanded) score per year. Trend lines compatible with deterioration of motor function and muscle strength started in childhood and continued into adulthood. The age at loss of specific motor skills was associated with disease severity. Triceps, deltoid, iliopsoas and quadriceps were the weakest muscles in all patients. Hierarchical cluster analysis did not show a segmental distribution of muscle weakness as suggested previously. Conclusions Progressive muscle weakness and loss of motor function are characteristic of all SMA types and all ages.
机译：脊髓肌肉萎缩（SMA）的背景和目的自然历史研究主要集中在婴儿和儿童上。包含所有年龄组和SMA类型的自然历史研究对于解释最近引入的生存电动机神经元基因增强疗法的治疗效果是重要的。方法我们进行了横截面研究，以研究肌肉力量，锤晶功能电机规模（扩展）得分以及与年龄和SMA型相关的肌肉弱点模式。结果我们包括180名患者1-4岁的患者1-77.5岁，中位数疾病持续时间为18（范围0-65.8）？多年。除了SMA类型2和3种的儿童的疾病早期阶段，可以实现新的运动技能并表现出肌肉力量的暂时增加，横断面数据表明，随着时间的推移，肌肉力量下降和运动技能丧失是特征所有SMA类型。医学研究委员会评分的均值至少为1点，每年哈默史密斯官能线（扩建）分数为0.5点。趋势线与运动功能的恶化兼容，童年中的肌肉力量和肌肉力量持续，并继续进入成年期。特定运动技能损失的年龄与疾病严重程度有关。肱三头肌，三角形，髂卵体和Quadriceps是所有患者中最弱的肌肉。分层聚类分析并未显示先前所提出的肌肉弱势的分段分布。结论渐进式肌肉弱点和运动功能的损失是所有SMA类型和所有年龄的特征。

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