MRI在进行性肌营养不良中的应用价值

Diagnostic Value of MRI in Progressive Muscular Dystrophy

目的探讨进行性肌营养不良(progressive muscular dystrophy,PMD)的骨骼肌MRI表现与临床的相关性及其应用价值。资料与方法对22例经临床表现、血清肌酸激酶(CPK)、肌电图检查及开放式骨骼肌活检、组织及免疫病理学证实的PMD患者的临床及影像资料进行回顾性分析。结果各型肌病MRI受累肌肉分布特征为:杜兴型和贝克型为大腿前部肌群;肢带型2B型为大腿后部肌群;远端型中Welander型为大腿后部肌群及小腿前群、外侧群肌肉;Nonaka型为小腿前群、外侧群肌肉;Miyoshi型为小腿后部肌群;先天性为大、小腿后部肌群;强直性为大、小腿前、后肌群。MRI上的病变程度与病程无一致性关系。除假肥大型无肌肉水肿表现外,其余类型均有不同程度的肌肉水肿。结论 MRI表现提示不同的肌病类型有特定的分布,有助于临床鉴别某些类型的肌病。利用对脂肪沉积和水肿敏感的MRI序列,有助于理解肌病的病理过程,协助临床检查监测对治疗的反应。

Objective To investigate MRI features of progressive muscular dystrophy and clinical manifestation as well as the diagnostic value of MRI. Materials and Methods The clinical and imaging materials of 22 cases progressive muscular dystrophy were analyzed retrospectively, which were confirmed by clinical manifestation, creatine kinase （CK）, Electromyography（EEG） and muscle biopsy including immunohistochemical studies. Results MR imaging showed involved muscles to be predominantly in anterior muscles of thigh in Duchenne and Becher muscular dystrophy, in posterior muscles of thigh in girdle muscular dystrophy, in posterior muscles of thigh and anterior and lateral muscles of calf in distal muscular dystrophy type Welander, in anterior and lateral muscles of calf type Nonaka, in posterior muscles of calf in type Miyoshi, in posterior muscles of thigh and calf in congenital muscular dystrophy, in anterior and posterior muscles of thigh and calf in myotonic dystrophy. There was no consistency between the degree on MRI and the duration of disease. Various degree edemas were showed in all type of cases except Duchenne and Becher muscular dystrophy. Conclusion MR findings suggest that a specific pattern of muscular involvement is established for each muscular disease, which may be helpful for the differentiation of some kinds of muscular disorders. MRI sequence which is sensitive to fatty degeneration and edema may be helpful for understanding the pathological course and monitor therapeutic evaluation.