Clinical Muscle Testing Compared with Whole-Body Magnetic Resonance Imaging in Facio-scapulo-humeral Muscular Dystrophy
Clinical Muscle Testing Compared with Whole-Body Magnetic Resonance Imaging in Facio-scapulo-humeral Muscular Dystrophy
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DOI：
10.1007/s00062-015-0386-y
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发表时间：
2016-12-01
影响因子：
2.8
通讯作者：
Weber, M. -A.
中科院分区：
文献类型：
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作者：
Regula, J. U.;Jestaedt, L.;Weber, M. -A.
本研究的目的是评估全身磁共振成像（MRI）在面肩肱肌营养不良症（FSHD）中的临床应用价值。在20例经遗传学证实的FSHD 1患者中，我们前瞻性评估了肌肉受累情况，并将半定量手动肌肉测试结果与其他参数（如疾病持续时间，肌酸激酶（CK）水平和D4Z4位点的重复长度与全身MRI。临床肌肉测试显示，肩胛肌，胸肌和冈下肌是最严重的受影响的肌肉在肩部，和膝屈肌和臀中肌在臀部带。核磁共振成像显示肩部的前锯肌和前锯肌，以及臀部的腘绳肌和内收肌是受影响最严重的肌肉群。总体而言，MRI扫描显示的脂肪变性程度与临床虚弱显著相关。此外，我们可以检测到明显的躯干肌肉的影响。与早期报告相对应，在临床检查和MRI评分中，不对称受累是常见的。此外，MRI显示在相当大比例的肌肉和患者中存在不均匀的肌肉变性。临床和MRI评分均与疾病持续时间显著相关，但与碎片大小或CK水平无关。全身MRI脂肪变性与四肢的临床肌肉测试相关性良好，但提供了更多关于深部或躯干肌肉的信息。它显示了肌肉疾病的结构变化，可能成为评估肌肉受累和随访研究的一个很好的工具。
The objective of this study was to evaluate the clinical usefulness of whole-body magnetic resonance imaging (MRI) in facio-scapulo-humeral muscular dystrophy (FSHD).In 20 patients with genetically proven FSHD1, we prospectively assessed muscular involvement and correlated the results of semi-quantitative manual muscle testing and other parameters such as disease duration, creatine kinase (CK) levels and repeat length of the D4Z4 locus with whole-body MRI.Clinical muscle testing revealed the trapezius, pectoralis and infraspinatus as the most severely affected muscles in the shoulder, and the knee flexors and gluteus medius in the hip girdle. MRI revealed the trapezius and serratus anterior muscles in the shoulder, and the hamstrings and adductor muscles in the hip girdle, as the most severely affected muscle groups. Overall, degrees of fatty degeneration on MRI scans correlated significantly with clinical weakness. Moreover, we could detect clear affection of the trunk muscles. Corresponding to earlier reports, asymmetric involvement was frequent in both clinical examination and MRI scoring. Moreover, MRI revealed inhomogeneous muscle degeneration in a considerable proportion of both, muscles and patients. Both clinical and MRI scores significantly correlated to disease duration, but not to fragment size or CK levels.Fatty degeneration in whole-body MRI correlates well to clinical muscle testing of the extremities but gives more information on deeper or trunk muscles. It shows structural changes in muscular disorders and may become an excellent tool for assessment of muscle involvement and follow-up studies.