Concurrent Paraspinous Myopathy and Myasthenia Gravis

Area of Science:

• Neurology
• Neuromuscular Disorders

Background:

• Paraspinous myopathy is a rare neuromuscular disorder affecting spinal muscles, causing symptoms like dropped head.
• Myasthenia gravis (MG) can cause camptocormia due to paraspinal muscle junction dysfunction, usually responsive to treatment.
• Concomitant MG and paraspinous myopathy is exceptionally rare, with only two prior reports.

Purpose of the Study:

• To report a case series of patients with coexisting myasthenia gravis and paraspinous myopathy.
• To highlight the under-recognition of this combined condition.
• To emphasize the distinct clinical presentation and treatment challenges.

Main Methods:

• Case series analysis of 5 patients.
• Review of clinical features, electrophysiologic findings, and histologic evidence.
• Comparison with existing literature on paraspinous myopathy and MG.

Main Results:

• Five patients with confirmed coexisting paraspinous myopathy and MG were identified.
• Neck and trunk extensor weakness in these patients was not solely due to neuromuscular junction dysfunction.
• The condition demonstrated resistance to standard MG treatments, suggesting a distinct myopathic process.

Conclusions:

• Coexisting paraspinous myopathy and myasthenia gravis is an under-recognized clinical entity.
• Patients presenting with neck or trunk extensor weakness, especially if resistant to treatment, should be evaluated for this combined disorder.
• Early recognition and consideration of paraspinous myopathy are crucial for appropriate patient management.