Suppr超能文献

杜氏肌营养不良症的肌肉磁共振成像:一种独特模式的证据。

Muscle MRI in Duchenne muscular dystrophy: Evidence of a distinctive pattern.

作者信息

Polavarapu Kiran, Manjunath Mahadevappa, Preethish-Kumar Veeramani, Sekar Deepha, Vengalil Seena, Thomas PriyaTreesa, Sathyaprabha Talakad N, Bharath Rose Dawn, Nalini Atchayaram

机构信息

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore 560029, India; Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences, Bangalore 560029, India.

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore 560029, India.

出版信息

Neuromuscul Disord. 2016 Nov;26(11):768-774. doi: 10.1016/j.nmd.2016.09.002. Epub 2016 Sep 5.

Abstract

The purpose of this study was to describe the pattern of muscle involvement using MRI findings and correlate with functional as well as muscle strength measurements. Fifty genetically confirmed DMD children with a mean age of 7.6 ± 2.8 (4-15 years) underwent muscle MRI and qualitative assessment was done for muscle changes using Mercuri staging for fibro-fatty replacement on T1 sequence and Borsato score for myoedema on STIR sequence. Detailed phenotypic characterisation was done with Manual muscle testing (modified MRC grading) and Muscular Dystrophy Functional Rating Scale (MDFRS). Mercuri scoring showed severe fibro-fatty changes in Gluteus medius, minimus and Adductor magnus followed by moderate to severe changes in Gluteus maximus and Quadriceps muscles. Total sparing of Gracilis, Sartorius and Semimembranosus muscles was observed. Superficial posterior and lateral leg muscles were preferentially involved with sparing of deep posterior and anterior leg muscles. Myoedema showed significant inverse correlation with fatty infiltration in thigh muscles. Similarly, significant inverse correlation was observed between Mercuri scores and MRC grading as well as MDFRS scores. A direct linear correlation was observed between duration of illness and fibro-fatty changes in piriformis, quadriceps and superficial posterior leg muscles. There was no correlation between MRI findings and genotypic characteristics. However, this specific pattern of muscle involvement in MRI could aid in proceeding for genetic testing when clinical suspicion is high, thus reducing the need for muscle biopsy. Fibro fatty infiltration as measured by Mercuri scoring can be a useful marker for assessing the disease severity and progression.

摘要

本研究的目的是利用MRI结果描述肌肉受累模式,并将其与功能及肌肉力量测量结果相关联。50名经基因确诊的杜氏肌营养不良症(DMD)患儿,平均年龄为7.6±2.8岁(4 - 15岁),接受了肌肉MRI检查,并使用T1序列上的Mercuri分期评估纤维脂肪替代的肌肉变化,以及使用短TI反转恢复(STIR)序列上的Borsato评分评估肌水肿。通过徒手肌力测试(改良医学研究委员会(MRC)分级)和肌肉营养不良功能评定量表(MDFRS)进行详细的表型特征描述。Mercuri评分显示,臀中肌、臀小肌和大收肌有严重的纤维脂肪改变,其次是臀大肌和股四头肌有中度至重度改变。观察到股薄肌、缝匠肌和半膜肌完全未受累。小腿后外侧浅表肌肉优先受累,而后侧深部和前侧小腿肌肉未受累。肌水肿与大腿肌肉的脂肪浸润呈显著负相关。同样,Mercuri评分与MRC分级以及MDFRS评分之间也观察到显著负相关。观察到疾病持续时间与梨状肌、股四头肌和小腿后外侧浅表肌肉的纤维脂肪改变之间存在直接线性关系。MRI结果与基因型特征之间没有相关性。然而,MRI中这种特定的肌肉受累模式在临床怀疑度高时有助于进行基因检测,从而减少肌肉活检的必要性。通过Mercuri评分测量的纤维脂肪浸润可以作为评估疾病严重程度和进展的有用标志物。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验