Learning objectives
Document the imaging appearance of hereditary and acquired myopathies.
Evaluate the role of magnetic resonance in identification and classification of hereditary and acquired myopathies.
Background
ABBREVIATIONS
INTRODUCTION
Muscle - gross anatomy
Muscle tissue consists of muscle fibers that are capable of contraction along their longitudinal axis.
Skeletal muscle in particular is a highly specialized organ system evolved for locomotion and energy metabolism in multicellular organisms.
The main elements that constitute a muscle tissue are displayed in Fig.
below.
Other fundamental structures relevant to muscle physiology include tendons,
nerves and blood vessels.
Muscle - micro-anatomy
Within each skeletal muscle cell or fiber there are cylindrical structures called myofibrils.
They extend and...
Findings and procedure details
THE ROLE OF MRI
MRI quite adequately complements findings from other diagnostic tools such as muscle biopsy and electromyography,
which may not be decisive for establishing a final diagnosis in patients with overlapping findings or non-specific clinical phenotypes and together may hint at a smaller number of possible conditions.
In general,
muscle MRI has a higher diagnostic yield in slowly progressive diseases in which selective patterns of muscle involvement can be discerned through a prolonged period of time.
Mild,
episodic or extensive and severe involvement...
Conclusion
Myopathies are a challenging group of disorders that require awareness of the clinical setting,
extent of tissue involvement and presence of different organ abnormalities.
Because of the often patchy and multifocal distribution of primary muscle disorders,
neuromuscular imaging has become an important tool for diagnosis,
monitoring and management as a co-adjuvant to other techniques such as electromyography,
genetic analysis and tissue biopsy.
References
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Dam L,
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Muscle imaging in inherited and acquired muscle diseases.
European journal of -neurology (2016) 23: 688 – 703
Janin A,
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Nuclear envelopathies: a complex LINC between nuclear envelope and pathology.
Journal of rare diseases (2017); 12: 147
Cellular and molecular mechanisms underlying muscular dystrophy.
The journal of cell biology (2013) 201 (4): 499 - 510
Moeller TB,
Reif E.
Pocket atlas of sectional anatomy Volumes I-III.
Thieme (2007)...