Clinical Rheumatology

, Volume 29, Issue 6, pp 683–686 | Cite as

Proposing an algorithm for treatment of different manifestations of neuro-Behcet’s disease

Brief Report


“Neuro-Behcet’s disease” (NBD) is the constellation of neurologic manifestations as a direct consequence of Behcet’s disease usually confirmed by imaging studies and/or cerebrospinal fluid analysis. The authors propose a therapeutic algorithm for neuro-Behcet’s disease based upon pathological, clinical, prognostic, and medico-economical issues based on available evidences. The authors divide anti-NBD armamentarium to first-line, second-line, and experimental drugs. These drugs should be administered hierarchically in treatment of parenchymal manifestations of neuro-Behcet’s disease. First-line drug include corticosteroids, azathioprine, methotrexate, and cyclophosphamide. Second-line drugs are tumor necrosis factor (TNF) alpha blocking drugs, interferon-α, chlorambucil, and mycophenolate mofenil. Experimental drugs include other “targeted therapies” than anti-TNF antibodies, tolerization therapy and stem cell transplantation. Cerebral venous sinus thrombosis associated with Behcet’s disease should be treated by short-term corticosteroids and anticoagulation with or without immunosuppressive drugs.


Behcet’s syndrome Nervous system Neuro-Behcet’s disease Treatment 



The authors have no relevant affiliations with any organization or entity with financial interest in or conflict of interest with subject matters discussed in manuscript.


  1. 1.
    Borhani Haghighi A, Pourmand R, Nikseresht AR (2005) Neuro-Behcet’s disease: a review. The Neurologist 11:80–89CrossRefPubMedGoogle Scholar
  2. 2.
    Borhani Haghighi A, Samangooei S, Ashjazadeh N et al (2006) Neurological manifestation of Behcet’s disease. Saudi Med J 27(10):1542–1546PubMedGoogle Scholar
  3. 3.
    Siva A, Kantarcy O, Saip S, Hamuryudan V, Altyntas A, Yazici H (1998) Neuro-Behcet syndrome: prognostic factors and survival. J Neurol 245:362Google Scholar
  4. 4.
    Hatemi G, Silman A, Bang D et al (2008) EULAR recommendations for the management of Behcet’s disease: report of a task force of the European Standing Committee for International Clinical Studies Including Therapeutics (ESCISIT). Ann Rheum Dis 67:1656–1662CrossRefPubMedGoogle Scholar
  5. 5.
    Borhani Haghighi A (2009) Treatment of Neuro-Behcet’s disease: an update. Exp Rev Neurother 9(4):565–574CrossRefGoogle Scholar
  6. 6.
    Yazici H, Pazarli H, Barnes CG et al (1990) A controlled trial of azathioprine in Behcet’s syndrome. N Engl J Med 322(5):326–328CrossRefGoogle Scholar
  7. 7.
    Hamuryudan V, Ozyazgan Y, Hizli N et al (1997) Azathioprine in Behcet’s syndrome: effects on long-term prognosis. Arthritis Rheum 40(4):769–774CrossRefPubMedGoogle Scholar
  8. 8.
    Hirohata S, Suda H, Hashimoto T (1998) Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behcet’s disease. J Neurol Sci 159(2):181–185CrossRefPubMedGoogle Scholar
  9. 9.
    Kikuchi H, Aramaki K, Hirohata S (2003) Low dose MTX for progressive neuro-Behcet’s disease. A follow-up study for 4 years. Adv Exp Med Biol 528:575–578CrossRefPubMedGoogle Scholar
  10. 10.
    Hamza M, Meddeb S, Mili I, Ouertani A (1992) Bolus of cyclophosphamide and methylprednisolone in uveitis in Behcet’s disease. Preliminary results with the use of new criteria of evaluation. Ann Med Interne (Paris) 143(7):438–441Google Scholar
  11. 11.
    Du LT, Fain O, Wechsler B, Cochereau I et al (1990) Value of “bolus” cyclophosphamide injections in Behcet’s disease. Experience of 17 cases. Presse Med 19(9):1355–1358PubMedGoogle Scholar
  12. 12.
    Sfikakis PP, Markomichelakis N, Alpsoy E et al (2007) Anti-TNF therapy in the management of Behcet’s disease—review and basis for recommendations. Rheumatology (Oxford) 46(5):736–741CrossRefGoogle Scholar
  13. 13.
    O’Duffy JD, Robertson DM, Goldstein NP (1984) Chlorambucil in the treatment of uveitis and meningoencephaliltis of Behcet’s disease. Am J Med 76:75–84CrossRefPubMedGoogle Scholar
  14. 14.
    Kotter I, Gunaydin I, Zierhut M, Stubiger N (2004) The use of interferon alpha in Behcet disease: review of the literature. Semin Arthritis Rheum 33:320–335CrossRefPubMedGoogle Scholar
  15. 15.
    Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS (2008) Treatment of venous thrombosis associated with Behcet’s disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol 27(2):201–205CrossRefPubMedGoogle Scholar
  16. 16.
    Yazici H, Fresko I, Yurdakul S (2007) Behcet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 3(3):148–155CrossRefPubMedGoogle Scholar
  17. 17.
    Siva A (2001) Vasculitis of the nervous system. J Neurol 248:451–468CrossRefPubMedGoogle Scholar
  18. 18.
    Bank I, Weart C (1984) Dural sinus thrombosis in Behcet’s disease. Arthritis Rheum 27:816–818CrossRefPubMedGoogle Scholar
  19. 19.
    Barlas S (1999) Behcet’s disease. An insight from a vascular surgeon’s point of view. Acta Chir Belg 99(6):274–281PubMedGoogle Scholar

Copyright information

© Clinical Rheumatology 2010

Authors and Affiliations

  1. 1.Transgenic Technology Research CenterShiraz University of Medical SciencesShirazIran
  2. 2.Department of NeurologyShiraz University of Medical SciencesShirazIran
  3. 3.Research Center for Traditional Medicine and history of medicineShiraz University of Medical SciencesShirazIran

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