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Comparison of the Clinical Manifestations, Brain MRI and Prognosis between NeuroBeh$\c{c}$et's Disease and Neuropsychiatric Lupus

  • Cho, Byung-Sik (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine) ;
  • Kim, Hyun-Sook (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine) ;
  • Oh, Su-Jin (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine) ;
  • Ko, Hyeok-Jae (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine) ;
  • Yoon, Chong-Hyun (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine) ;
  • Jung, So-Lyung (Department of Radiology, The Catholic University of Korea College of Medicine) ;
  • Min, Do-June (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine) ;
  • Kim, Wan-Uk (Division of Rheumatology, Department of Internal Medicine, The Catholic University of Korea College of Medicine)
  • Published : 20070600

Abstract

Background : Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBeçhet disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients. Methods : Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE. Results : The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients. Conclusion : NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.

Keywords

Acknowledgement

Supported by a grant from MOST (KOSEF) through The Systems Bio-Dynamics Research Center.

References

  1. Nadeau SE. Neurologic manifestations of connective tissue disease. Neurol Clin 20:151-178, 2002 https://doi.org/10.1016/S0733-8619(03)00057-4
  2. Akman-Demir G, Serdaroglu P, Tasci B. Clinical patterns of neurological involvement in Behçet's disease syndrome: evaluation of 200 patients. Brain 122:2171-2182, 1999 https://doi.org/10.1093/brain/122.11.2171
  3. Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, Kocer N, Yazici H. Behçet's disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248:95-103, 2001 https://doi.org/10.1007/s004150170242
  4. Siva A, Altintas A, Saip S. Behçet's syndrome and the nervous system. Curr Opin Neurol 17:347-357, 2004 https://doi.org/10.1097/00019052-200406000-00017
  5. Salmon JE, Kimberly RP, Agarti VD. Systemic lupus erythematosus: immunopathology. In: Hochberg MC, Silman A, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. vol. 2. 3rd ed. p. 1297-1315, London, Mosby, 2003
  6. Sanna G, Bertolaccini ML, Mathieu A. Central nervous system lupus: a clinical approach to therapy. Lupus 12:935-942, 2003 https://doi.org/10.1191/0961203303lu505oa
  7. Jennekens FG, Kater L. The central nervous system in systemic lupus erythematosus: part 1. clinical syndromes: a literature investigation. Rheumatology 41:605-618, 2002 https://doi.org/10.1093/rheumatology/41.6.605
  8. The American College of Rheumatology. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 42:599-608, 1999 https://doi.org/10.1002/1529-0131(199904)42:4<599::AID-ANR2>3.0.CO;2-F
  9. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271-1277, 1982
  10. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet 335:1078-1080, 1990
  11. Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC, Brey R, Derksen R, Harris EN, Hughes GR, Triplett DA, Khamashta MA. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42:1309-1311, 1999 https://doi.org/10.1002/1529-0131(199907)42:7<1309::AID-ANR1>3.0.CO;2-F
  12. Fieschi C, Rasura M, Anzini A, Beccia M. Central nervous system vasculitis. J Neurol Sci 153:159-171, 1998 https://doi.org/10.1016/S0022-510X(97)00288-8
  13. Siva A. Vasculitis of the nervous system. J Neurol 248:451-468, 2001 https://doi.org/10.1007/s004150170154
  14. Scolding NJ, Joseph FG. The neuropathology and pathogenesis of systemic lupus erythematosus. Neuropathol Appl Neurobiol 28:173- 189, 2002 https://doi.org/10.1046/j.1365-2990.2002.00406.x
  15. Jennekens FG, Kater L. The central nervous system in systemic lupus erythematosus: part 2. pathogenetic mechanisms of clinical syndromes: a literature investigation. Rheumatology 41:619-630, 2002 https://doi.org/10.1093/rheumatology/41.6.619
  16. Meroni PL, Tincani A, Sepp N, Raschi E, Testoni C, Corsini E, Cavazzana I, Pellegrini S, Salmaggi A. Endothelium and the brain in CNS lupus. Lupus 12:919-928, 2003 https://doi.org/10.1191/0961203303lu503oa
  17. Graham JW, Jan W. MRI and the brain in systemic lupus erythematosus. Lupus 12:891-896, 2003 https://doi.org/10.1191/0961203303lu498oa
  18. Akman-Demir G, Bahar S, Coban O, Tasci B, Serdaroglu P. Cranial MRI in Behçet's disease: 134 examinations of 98 patients. Neuroradiology 45:851-859, 2003 https://doi.org/10.1007/s00234-003-1102-1
  19. Rovaris M, Viti B, Ciboddo G, Gerevini S, Capra R, Jaunucci G, Comi G, Filippi M. Brain involvement in systemic immune mediated disease: magnetic resonance and magnetization transfer imaging study. J Neurol Psychiary 68:170-177, 2000
  20. Kocer N, Islak C, Siva A, Saip S, Akman C, Kantarci O, Hamuryudan V. CNS involvement in neuro-Behçet's syndrome: an MR study. AJNR Am J Neuroradiol 20:1015-1024, 1999
  21. Bosma GP, Steens SC, Petropoulos H, Admiraal-Behloul F, van den Haak A, Doornbos J, Huizinga TW, Brooks WM, Harville A, Sibbitt WL Jr, van Buchem MA. Multisequence magnetic resonance imaging study of neuropsychiatric systemic lupus erythematosus. Arthritis Rheum 50:3195-3202, 2004 https://doi.org/10.1002/art.20512
  22. West SG. Lupus and the central nervous system. Curr Opin Rheumatol 8:408-414, 1996 https://doi.org/10.1097/00002281-199609000-00004