The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
권호 표지

A Case of Sensorimotor Polyneuropathy in a Patient with Sjogren's Syndrome

다발신경병증과 근위축을 동반한 쇼그렌 증후군 1예

  • Kim, Eun-Jeong (Department of Internal Medicine, College of Medicine, Chosun University) ;
  • Lee, Hee-Jeong (Department of Internal Medicine, College of Medicine, Chosun University) ;
  • Jeong, Yu-Jin (Department of Internal Medicine, College of Medicine, Chosun University) ;
  • Hong, Su-Jin (Department of Internal Medicine, College of Medicine, Chosun University) ;
  • Kim, Yun-Sung (Department of Internal Medicine, College of Medicine, Chosun University) ;
  • Kim, Hyun-Sook (Department of Internal Medicine, College of Medicine, Chosun University)
  • 김은정 (조선대학교 의과대학 내과학교실) ;
  • 이희정 (조선대학교 의과대학 내과학교실) ;
  • 정유진 (조선대학교 의과대학 내과학교실) ;
  • 홍수진 (조선대학교 의과대학 내과학교실) ;
  • 김윤성 (조선대학교 의과대학 내과학교실) ;
  • 김현숙 (조선대학교 의과대학 내과학교실)
  • Published : 2011.08.01
⟨ Previous Next ⟩

Abstract

Sjogren's syndrome is a chronic inflammatory disorder characterized by lymphocytic infiltration of the exocrine glands, especially the lacrimal and salivary glands. Although it is characterized primarily by a particular form of dry eyes (keratoconjunctivitis sicca) and a dry mouth (xerostomia), this condition may affect a wide variety of organ systems, including the skin, lungs, heart, and kidneys, and it may rarely have neurological manifestations. The peripheral neuropathy results from nerve damage due to vasculitis or nerve sheath demyelination, which adversely affects the ability of nerves to send messages. We report the case of a 71-year-old woman who presented with polyneuropathy of the lower limbs that was diagnosed as Sjogren's syndrome but progressed to muscle atrophy during her treatment.

근력약화와 따끔거림을 주소로 내원한 환자에서 일차성 쇼그렌 증후군에서 병발한 다발신경병증을 진단하고 고용량 스테로이드 치료 및 면역억제제 치료하며 경과관찰 중 근위축까지 진행된 1예를 경험하였기에 문헌고찰과 함께 증례 보고하는 바이다.

Keywords

References

  1. Fox RI, Saito I. Criteria for diagnosis of Sjogren's syndrome. Rheum Dis Clin North Am 1994;20:391-407.
  2. Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ. Sjogren's syndrome: a clinical, pathological and serological study of sixty-two cases, 1965. Medicine (Baltimore) 1992;71:386-401;discussion 401-403.
  3. Lee CW, Lee WK, Lee JS, et al. Clinical analysis of Sjogren's syndrome. J Korean Rheum Assoc 1997;4:139-146.
  4. Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evoluation, and morbidity and mortality of primary Sjogren's syndrome. Semin Arthritis Rheum 2000;29:296-304. https://doi.org/10.1016/S0049-0172(00)80016-5
  5. Talal N. Sjogren's syndrome: historical overview and clinical spectrum of disease. Rheum Dis Clin North Am 1992;18:507-515.
  6. Choi SW, Jung HC, Kim JC, Kang BS, Kim YM. A case of primary Sjogren's syndrome associated with acute transverse myelitis. J Korean Rheum Assoc 2006;13:86-90.
  7. Grant IA, Hunder GG, Homburger HA, Dyck PJ. Peripheral neuropathy associated with sicca comple. Neurology 1997;48:855-862. https://doi.org/10.1212/WNL.48.4.855
  8. Dyck PJ, Oviatt KF, Lambert EH. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis. Ann Neurol 1981;10:222-226. https://doi.org/10.1002/ana.410100304
  9. Griffin JW, Cornblath DR, Alexander E, et al. Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjogren's syndrome. Ann Neurol 1990;27:304-315. https://doi.org/10.1002/ana.410270313
  10. Alexander EL, Provost TT, Stevens MB, Alexander GE. Neurologic complications of primary Sjogren's syndrome. Medicine (Baltimore) 1982;61:247-257.
  11. Williams CS, Butler E, Roman GC. Treatment of myelopathy in Sjogren's syndrome with a combination of prednisone and cyclophosphamide. Arch Neurol 2001;58:815-819. https://doi.org/10.1001/archneur.58.5.815
  12. De Seze J, Delalande S, Fauchais AL, et al. Myelopathies secondary to Sjogren's syndrome: treatment with monthly intravenous cyclophosphamide associated with corticosteroids. J Rheumatol 2006;33:709-711.
  13. Ramos-Casals M, Solans R, Rosas J, et al. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore) 2008;87:210-219. https://doi.org/10.1097/MD.0b013e318181e6af
  14. Mellgren SI, Conn DL, Stevens JC, Dyck PJ. Peripheral neuropathy in primary Sjögren's syndrome. Neurology 1989;39:390-394. https://doi.org/10.1212/WNL.39.3.390
  15. Kaltreider HB, Talal N. The neuropathy of Sjogren's syndrome: trigeminal nerve involvement. Ann Intern Med 1969;70:751-762. https://doi.org/10.7326/0003-4819-70-4-751
  16. Kaplan JG, Rosenberg R, Reinitz E, Buchbinder S, Schaumburg HH. Invited review: peripheral neuropathy in Sjögren's syndrome. Muscle Nerve 1990;13:570-579. https://doi.org/10.1002/mus.880130703
  17. Gemignani F, Marbini A, Pavesi G, et al. Peripheral neuropathy associated with primary Sjogren's syndrome. J Neurol Neurosurg Psychiatry 1994;57:983-986. https://doi.org/10.1136/jnnp.57.8.983
  18. Rafai MA, Boulaajaj FZ, Moutawakil F, et al. Neurological manifestations revealing primitive Gougerot-Sjogren syndrome: 9 cases. Joint Bone Spine 2009;76:139-145. https://doi.org/10.1016/j.jbspin.2008.03.010
  19. Caroyer JM, Manto MU, Steinfeld SD. Severe sensory neuronopathy responsive to infliximab in primary Sjogren's syndrome. Neurology 2002;59:1113-1114. https://doi.org/10.1212/WNL.59.7.1113