J Korean Rheum Assoc.  2008 Sep;15(3):244-249. 10.4078/jkra.2008.15.3.244.

Primary Sjogren's Syndrome Associated with Recurrent Transverse Myelitis

• Lee HH
• Park SH

• KMID: 1270203
• DOI: http://doi.org/10.4078/jkra.2008.15.3.244

Abstract

Sjogren's syndrome is a chronic autoimmune disorder due to lymphocytes infiltrations of the exocrine glands and is clinically characterized by dry eyes and dry mouth. Though Sjogren's syndrome can affect the nervous system, acute transverse myelitis is an extremely rare CNS complication that progresses rapidly, and requires early diagnosis and therapy. Here, we report a 49-year-old female who had primary Sjogren's syndrome that manifested itself as acute transverse myelitis. She presented with motor weakness and hypesthesia of her right lower extremity. During her first attack, MRI of the spine revealed high signal intensities on T2-weighted images in her spinal cord at the levels of C6 through T11. Symptoms improved after 5 days of intravenous methylprednisolone (1 g/day) therapy, but recurred 9 months later. She had another MRI which showed acute transverse myelitis involving the spinal cord at the levels of C2 to T8. Her symptoms remained well controlled with treatment consisting of 5 days of methylpredisolone infusion (1 g/day) and concomitant intravenous cyclophosphamide 750 mg every 4 weeks.

Keyword

Sjogren's syndrome; Transverse myelitis

MeSH Terms

Female
Humans

Figure

• 

  Fig. 1. Diffuse high signal intensities are seen in the spinal cord from C6 to T11 on T2 weighted image without enhancement after infusion of the contrast media, mainly central gray matter involvement when she was first diagnosed with transverse myelitis.

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  Fig. 2. Diffuse high signal intensities are seen in the spinal cord with intramedullary high signal intensity change in the cervical and upper thoracic levels (C2 to T9 levels).

Reference

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