Beltrame Valeria, Ortolan Paolo, Coran Alessandro, Zanato Riccardo, Gazzola Matteo, Frigo Annachiara, Bello Luca, Pegoraro Elena, Stramare Roberto
Department of Medicine, Section of Radiology, University of Padova, Padova, Italy.
Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy.
PLoS One. 2014 Apr 10;9(4):e94427. doi: 10.1371/journal.pone.0094427. eCollection 2014.
To evaluate with Magnetic Resonance (MR) the degree of fatty replacement and edematous involvement in skeletal muscles in patients with Tubular Aggregate Myopathy (TAM). To asses the inter-observer agreement in evaluating muscle involvement and the symmetry index of fatty replacement.
13 patients were evaluated by MR to ascertain the degree of fatty replacement (T1W sequences) according to Mercuri's scale, and edema score (STIR sequences) according to extent and site.
Fatty replacement mainly affects the posterior superficial compartment of the leg; the anterior compartment is generally spared. Edema was generally poor and almost only in the superficial compartment of the leg. The inter-observer agreement is very good with a Krippendorff's coefficient >0.9. Data show a total symmetry in the muscular replacement (McNemar-Bowker test with p = 1).
MR reveals characteristic muscular involvement, and is a reproducible technique for evaluation of TAM. There may also be a characteristic involvement of the long and short heads of the biceps femoris. It is useful for aimed biopsies, diagnostic hypotheses and evaluation of disease progression.
利用磁共振成像（MR）评估管状聚集性肌病（TAM）患者骨骼肌的脂肪替代程度和水肿累及情况。评估观察者间在评估肌肉受累情况和脂肪替代对称性指数方面的一致性。
对13例患者进行MR检查，根据Mercuri量表确定脂肪替代程度（T1加权序列），并根据范围和部位确定水肿评分（短反转恢复序列）。
脂肪替代主要影响小腿后浅部肌群；前部肌群一般未受累。水肿通常较轻，几乎仅见于小腿浅部肌群。观察者间一致性非常好，Krippendorff系数>0.9。数据显示肌肉替代完全对称（McNemar-Bowker检验，p = 1）。
MR显示出特征性的肌肉受累情况，是评估TAM的可重复技术。股二头肌长头和短头可能也有特征性受累。它有助于进行有针对性的活检、诊断假设及疾病进展评估。
