JRD Journal of Rheumatic Diseases J Rheum Dis 2093-940X 2233-4718 The Korean College of Rheumatology 10.4078/jrd.2013.20.5.319 jrd-20-319 Case Report A Case of Sjögren's Syndrome Associated with Neuromyelitis Optica KimKyung Han ParkSu-Min KimBo-sung HanSeung Hee LeeSang Yeob ChungWon Tae LeeSung Won Division of Rheumatology, Department of Internal Medicine, College of Medicine, Dong-A University, Busan, Korea Corresponding to : Sung Won Lee, Division of Rheumatology, Department of Internal Medicine, College of Medicine, Dong-A University, 1, Dongdaesin-dong 3-ga, Seo-gu, Busan 602-715, Korea. E-mail : leesw@dau.ac.kr 10 2013 31 10 2013 20 5 319 322 13082012 11102012 15102012 Copyright © 2013 The Korean College of Rheumatology 2013 This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract

Neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating disease, characterized by optic neuritis and myelitis. NMO is a very uncommon and serious neurologic manifestation of Sjögren's syndrome. We report on a 32-year-old female with NMO as central nerve system involvement of Sjögren's syndrome. She had a transverse myelitis ten years ago and did not have symptoms for a long period of time. She visited the emergency center because of worsening weakness of both limbs. She had an appendectomy three days ago before hospitalization. Cervical spinal magnetic resonance imaging showed increased signal intensity in T2-weighted images from the cervical (C2) to the upper thoracic (T4) spinal cord. As serum NMO-IgG was positive, we diagnosed neuromyelitis optica and treated with high dose steroid, but failed. Therefore, we treated with plasmapheresis and the patient was discharged without any neurological deficits.

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In T2 weighted magnetic resonance imaging, sagittal view shows extensive longitudinal high signal intensity lesion from cervical (C2) to upper thoracic (T4) spinal cord (A). Also transverse view show high signal intensity with cord swelling in C5 level (B) (whtie arrow).

In T2 weighted magnetic resonance imaging, sagittal (A) and transverse (B) view shows decreased high signal intensity lesion, compared to prior test (C3-T3), after 1 month later (whtie arrow).