J Rheum Dis.  2016 Aug;23(4):261-265. 10.4078/jrd.2016.23.4.261.

A Case of Polymyositis Presenting as Bent Spine Syndrome

• Choi J
• Kim D
• Jun JB
• Lee S
• Paik SS
• Sung YK

• KMID: 2352470
• DOI: http://doi.org/10.4078/jrd.2016.23.4.261

Abstract

Polymyositis (PM) is a subset of idiopathic inflammatory myopathies. The muscles involved with PM are typically proximal and distal limb muscles, but paraspinal muscles are rarely affected. The primary PM clinical symptom is gradual proximal muscle weakness but unusually abnormal trunk posture. Bent spine syndrome (BSS), also referred to camptocormia, is defined as an abnormal flexion of the trunk, appearing in standing position. An idiopathic axial myopathy is the most common cause of primary BSS. A few cases of inflammatory myopathy, a secondary BSS, have been reported. We describe a 59-year-old polymyositis patient with normal finding on an magnetic resonance imaging femur scan who presented with BSS only, myopathic findings on electromyography and elevation of muscle enzymes.

Keyword

Polymyositis; Bent spine syndrome; Camptocormia

MeSH Terms

Electromyography
Extremities
Femur
Humans
Magnetic Resonance Imaging
Muscle Weakness
Muscles
Muscular Diseases
Myositis
Paraspinal Muscles
Polymyositis*
Posture
Spine*

Figure

• 

  Figure 1. Magnetic resonance imaging scan for thighs at rehospitalization. Axial T2-weighted scan (A) and axial fatsaturated T2-weighted scan (B) revealed bilaterally fatty atrophy of semimembranosus muscle (arrow) without active inflammation.

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  Figure 2. Axial lumbar spine magnetic resonance imaging. Fat saturated T1-weighted scan (A) and T2-weighted scan (B) showed paraspinous muscle edema with enhancement, especially worse on the right iliocostalis muscle (arrow) and longissimus (arrow head) at the level of L2 and L3.

• 

  Figure 3. Pathologic finding of paraspinal muscle. (A) The right longissimus muscle demonstrates endomysial lymphocytic infiltration (H&E, ×100). (B) CD8 cytotoxic T-cell in the same tissue (immunohistochemical stain, ×200).

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