Department of Medical Diagnostic Sciences and Special Therapies, University of Padova, Via Giustiniani 2, 35128, Padova, Italy.
Radiol Med. 2010 Jun;115(4):585-99. doi: 10.1007/s11547-010-0531-2. Epub 2010 Feb 22.
The continuous discovery of new subtypes of neuromuscular disorders demands more accurate imaging analyses. We set out to establish the specific patterns of muscular involution using magnetic resonance imaging (MRI).
A systematic clinical evaluation based on the Medical Research Council scale and MRI was completed in ten patients with calpainopathy [limb-girdle muscular dystrophy (LGMD)-2A], 16 with dysferlinopathy (LGMD-2B), ten with hyaline body myopathy (HBM), six with myotonic dystrophy (MD) types 1 and 5 with MD type 2. Severity of fibroadipose degeneration was specifically staged using T1-weighted sequences. Turbo inversion recovery magnitude (TIRM) sequences were used to assess oedema-like changes.
T1 scans showed recurrent patterns of fibroadipose replacement, whereas TIRM images revealed differences in oedema-like changes between the various diseases. In LGMD, the posterior compartments are more vulnerable to degeneration. In HBM, fatty muscle degeneration and oedema are allocated to muscles of the posterior compartments of the leg. In MD, fatty muscle degeneration and oedematous changes are allocated to muscles of the anterior thigh and posterior lower leg.
Imaging examination suggests a characteristic pattern of muscle involvement. MRI represents an important diagnostic technique useful in differential diagnosis, thanks to the distinctive patterns observed in the distribution of muscular changes between the different muscular diseases.
不断发现新的神经肌肉疾病亚型，要求更准确的影像学分析。我们着手使用磁共振成像（MRI）来建立肌肉萎缩的特定模式。
对 10 例钙蛋白酶病患者（肢带型肌营养不良症 2A 型）、16 例肌营养不良蛋白病患者（肢带型肌营养不良症 2B 型）、10 例玻璃样变体肌病患者、6 例强直性肌营养不良症 1 型和 5 例强直性肌营养不良症 2 型患者进行了基于医学研究委员会量表和 MRI 的系统临床评估。使用 T1 加权序列对纤维脂肪变性的严重程度进行特定分期。使用涡轮反转恢复幅度（TIRM）序列评估水肿样变化。
T1 扫描显示出纤维脂肪替代的反复出现模式，而 TIRM 图像显示出不同疾病之间水肿样变化的差异。在 LGMD 中，后间隔更容易发生退行性变。在 HBM 中，脂肪肌肉变性和水肿被分配到腿部的后间隔肌肉。在 MD 中，脂肪肌肉变性和水肿变化被分配到大腿前侧和小腿后侧的肌肉。
影像学检查提示肌肉受累的特征性模式。MRI 是一种重要的诊断技术，在鉴别诊断中具有重要作用，因为在不同肌肉疾病中观察到肌肉变化分布的独特模式。
