J Rheum Dis.  2013 Oct;20(5):319-322. 10.4078/jrd.2013.20.5.319.

A Case of Sjogren's Syndrome Associated with Neuromyelitis Optica

• Kim KH
• Park SM
• Kim BS
• Han SH
• Lee SY
• Chung WT
• Lee SW

• KMID: 2223009
• DOI: http://doi.org/10.4078/jrd.2013.20.5.319

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 Sjogren's syndrome. We report on a 32-year-old female with NMO as central nerve system involvement of Sjogren'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.

Keyword

Sjogren's syndrome; Neuromyelitis optica

MeSH Terms

Adult
Appendectomy
Demyelinating Diseases
Emergencies
Extremities
Female
Hospitalization
Humans
Magnetic Resonance Imaging
Myelitis
Myelitis, Transverse
Neurologic Manifestations
Neuromyelitis Optica*
Optic Neuritis
Plasmapheresis
Sjogren's Syndrome*
Spinal Cord

Figure

• 

  Figure 1. 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).

• 

  Figure 2. 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).

Reference

References

1. Alexander EL, Provost TT, Stevens MB, Alexander GE. Neurologic complications of primary Sjögren's syndrome. Medicine (Baltimore). 1982; 61:247–57.

2. Mandler RN. Neuromyelitis optica - Devic's syndrome, update. Autoimmun Rev. 2006; 5:537–43.

3. Minagar A, Alexander JS, Fowler MR, Long AC, Kelley RE. Devic disease: clinical course, pathophysiology, and management. Pathophysiology. 2002; 9:33.

4. Lucchinetti CF, Mandler RN, McGavern D, Bruck W, Gleich G, Ransohoff RM, et al. A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica. Brain. 2002; 125:1450–61.

5. Williams CS, Butler E, Román GC. Treatment of myel-opathy in Sjögren syndrome with a combination of prednisone and cyclophosphamide. Arch Neurol. 2001; 58:815–9.

6. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007; 6:805–15.

7. Protti A, Erminio C, Piccolo I, Spreafico C, Colombo F, Ghezzi A. An unusual case with relapsing neuromyelitis optica associated with undifferentiated connective tissue disease. Neurol Sci. 2004; 25(Suppl 4):S383–5.

8. Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004; 364:2106–12.

9. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006; 66:1485–9.

10. Koo YS, Yoo JK, Kwon DY, Park MH, Koh SB, Kim BJ, et al. Neuromyelitis optica with positive anti-Ro and anti-La antibodies. J Korean Neurol Assoc. 2009; 27:446–8.

11. Keegan M, Pineda AA, McClelland RL, Darby CH, Rodriguez M, Weinshenker BG. Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology. 2002; 58:143–6.

12. Mandler RN, Ahmed W, Dencoff JE. Devic's neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology. 1998; 51:1219–20.

13. Jacob A, Weinshenker BG, Violich I, McLinskey N, Krupp L, Fox RJ, et al. Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol. 2008; 65:1443–8.

14. Takahashi T, Fujihara K, Nakashima I, Misu T, Miyazawa I, Nakamura M, et al. Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre. Brain. 2007; 130:1235–43.

15. Bonnan M, Cabre P. Plasma exchange in severe attacks of neuromyelitis optica. Mult Scler Int. 2012; 2012; 787630.