Clinical，pathological and skeletal muscle magnetic resonance characteristics of tonic muscular dystrophy type1
- VernacularTitle:强直性肌营养不良I型临床、病理、骨骼肌磁共振特点
- Author:
Cheng JIANG
1
;
Yongjuan LIN
1
;
Feng LI
1
Author Information
- Publication Type:Journal Article
- Keywords: Myotonic dystrophy 1 ； Clinical manifestation ； Pathology ； Muscle magnetic resonance imaging
- From: Journal of Apoplexy and Nervous Diseases 2022;39(1):33-37
- CountryChina
- Language:Chinese
- Abstract: To summarize characteristics of the clinical manifestation，pathology and lower limb muscle magnetic resonance imaging in 11 patients diagnosed as myotonic dystrophy 1.Methods Eleven patients with myotonic myopathy 1，who were admitted to Nanjing Drum Tower Hopsital from January 2012 to October 2020，were chosen in our study. All patients accepted clinical examination，skeletal muscle biopsy，and 5 of them received lower limb muscle magnetic resonance imaging （MRI）. Clinical data were collected for retrospective analysis.Results All 11 patients were observed muscle myotonia，weakness or amyotrophy to some extent and the latter two symptoms were more serious in distal limb than in proximal. Under the light microscopy，type I filber atrophy in ten cases，nucleus moving inward，nucleus gathering and sarcoplasmic masses in some cases were found.That fatty infiltration appears asymmetrical distribution and more serious in distal limb compared with the proximal limb in five cases was observed by MRI. Among lower limbs，vastus medialis，vastus lateralis，vastus intermedius，tibialis anterior，gastrocnemius，soleus muscles were the most severely affected.Conclusion Muscle magnetic resonance imaging examination is a trustworthy method for definite diagnosis of myotonic myopathies.
- Full text:2024072722152135554强直性肌营养不良I型临床、_病理、_骨骼肌磁共振特点.pdf
