Han Hee Lee; Seung Min Jeong; Joo Ha Lee; Jae Wuk Kwak; Kwi Young Kang; Ji Hyeon Ju; Ho Youn Kim; Sung Hwan Park

doi:10.4078/jkra.2008.15.3.244

Journal List > J Korean Rheum Assoc > v.15(3) > 1003629

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Lee, Jeong, Lee, Kwak, Kang, Ju, Kim, and Park: Primary Sjögren's Syndrome Associated with Recurrent Transverse Myelitis

Original Article

J Korean Rheum Assoc 2008;15(3):244-249.

Published online: 20 September 2008

DOI: https://doi.org/10.4078/jkra.2008.15.3.244

Primary Sjögren's Syndrome Associated with Recurrent Transverse Myelitis

Han Hee Lee, Seung Min Jeong, Joo Ha Lee, Jae Wuk Kwak, Kwi Young Kang, Ji Hyeon Ju, Ho Youn Kim, Sung Hwan Park

Department of Internal Medicine, Kangnam St. Mary's Hospital, The Catholic University of Korea, Seoul, Korea

Received 30 April 2008 Accepted 16 June 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Sjögren'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 Sjögren'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 Sjögren'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.

Keywords: Sjö gren's syndrome, Transverse myelitis

References

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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.

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).

Table 1.

Comparison of features of 3 cases: primary Sjögren's syndrome presenting as acute transverse myelitis in

Korea
	Choi et al (4)	Choi et al (5)	This case
Age of onset/Sex	37/F	43/F	49/F
Chief complaint	Both leg numbness & Both leg weakness, Rt. arm Both leg weakness,
	voiding difficulty	& both leg paresthesia,	hypoesthesia
		voiding difficulty	(neck∼umbilicus)
Rheumatoid factor	＋	＋	−
Anti-nuclear antibody	1:320	1:320	1:800
Anti-SSA/SSB antibodies	＋/＋	＋/−	＋/＋
C3/C4 (mg/dL)	90/15	81.9/16.9	85.7/13.5
CSF protein (mg/dL)	180	62.4	138.97
IgG	↑	↑	↑
Oligoclonal bands (CSF)	−	−	−
MRI finding：high signal	C6-T7	C3-T3	C2-T8
in T2 weighted image

Methylprednisolone 1 g/day Treatment for 10 days → oral prednisolone, cyclophosphamide pulse Tx. Methylprednisolone 1 g/day for 5 days → oral prednisolone, cyclophosphamide pulse Tx. Methylprednisolone 1 g/day for 5 days → oral prednisolone, cyclophosphamide pulse Tx.

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