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Behçet’s Disease and Nervous System Involvement

  • Neurologic Manifestations of Systemic Disease (N Scolding and C Rice, Section Editors)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Management of neuro-Behçet’s disease can be divided into two stages: treatment of acute attacks and prevention of relapses. Treatment of acute attacks is accomplished by high-dose intravenous corticosteroids followed by maintenance treatment with oral steroids for 6–12 months depending on the type and severity of the neurological involvement. Relapses can be prevented by using immunosuppressants. Oral immunosuppressants such as azathioprine and mycophenolate are the most widely utilized agents for this purpose. Patients who are refractory or who cannot tolerate these medications can be managed by cyclophosphamide, interferon alpha, or anti-TNF-α monoclonal antibodies such as infliximab, etanercept, and adalimumab. Recent reports showed that newer agents such as tocilizumab, canakinumab, and anakinra, which exert their biological activity through IL-1 and IL-6 pathways, are also promising treatment alternatives for progressive or relapsing patients.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Behçet H. Über residivierende, aphtöse durch ein Virus verursachtes Geschwüre am Mund, am Auge und an der Genitalien. Derm Wschr. 1937;105(36):1152–63.

    Google Scholar 

  2. Gül A. Pathogenesis of Behçet’s disease: autoinflammatory features and beyond. Semin Immunopathol. 2015;37(4):413–8. This is an excellent review that gives a comprehensive account of pathogenic factors involved in Behçet’s disease.

    Article  PubMed  Google Scholar 

  3. Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, et al. Behçet’s disease—a contemporary review. J Autoimmun. 2009;32(3–4):178–88.

    Article  CAS  PubMed  Google Scholar 

  4. Yurdakul S, Yazici H. Behçet’s syndrome. Best Pract Res Clin Rheumatol. 2008;22(5):793–809.

    Article  PubMed  Google Scholar 

  5. Hatemi G, Yazici H. Behçet’s syndrome and micro-organisms. Best Pract Res Clin Rheumatol. 2011;25(3):389–406.

    Article  PubMed  Google Scholar 

  6. Kaya Tİ. Genetics of Behçet’s disease. Pathol Res Int. 2012;2012:912589.

    Article  Google Scholar 

  7. Mat C, Yurdakul S, Sevim A, Özyazgan Y, Tüzün Y. Behçet’s syndrome: facts and controversies. Clin Dermatol. 2013;31(4):352–61.

    Article  PubMed  Google Scholar 

  8. Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014;121:1703–23.

    Article  PubMed  Google Scholar 

  9. Cicek D, Dağlı AF, Aydin S, Baskaya Dogan F, Dertlioğlu SB, et al. Does hepcidin play a role in the pathogenesis of aphthae in Behçet’s disease and recurrent aphthous stomatitis? J Eur Acad Dermatol Venereol. 2014;28(11):1500–6.

    Article  CAS  PubMed  Google Scholar 

  10. Vural B, Uğurel E, Tüzün E, Kürtüncü M, Zuliani L, Cavuş F, et al. Anti-neuronal and stress-induced-phosphoprotein 1 antibodies in neuro-Behçet’s disease. J Neuroimmunol. 2011;239(1–2):91–7.

    Article  CAS  PubMed  Google Scholar 

  11. Sakane T, Suzuki N, Nagafuchi H. Etiopathology of Behçet’s disease: immunological aspects. Yonsei Med J. 1997;38(6):350–8.

    Article  CAS  PubMed  Google Scholar 

  12. Marta M, Santos E, Coutinho E, Silva AM, Correia J, Vasconcelos C, et al. The role of infections in Behçet disease and neuro-Behçet syndrome. Autoimmun Rev. 2015;14(7):609–15.

    Article  CAS  PubMed  Google Scholar 

  13. Na SY, Park MJ, Park S, Lee ES. Up-regulation of Th17 and related cytokines in Behçet’s disease corresponding to disease activity. Clin Exp Rheumatol. 2013;31(3 Suppl 77):32–40.

    PubMed  Google Scholar 

  14. Wang C, Tian Y, Ye Z, Kijlstra A, Zhou Y, Yang P. Decreased interleukin 27 expression is associated with active uveitis in Behçet’s disease. Arthritis Res Ther. 2014;16(3):R117.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Ye Z, Wang C, Kijlstra A, Zhou X, Yang P. A possible role for interleukin 37 in the pathogenesis of Behcet’s disease. Curr Mol Med. 2014;14(4):535–42.

    Article  CAS  PubMed  Google Scholar 

  16. Remmers EF, Cosan F, Kirino Y, Ombrello MJ, Abaci N, Satorius C, et al. Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet’s disease. Nat Genet. 2010;42(8):698–702. This is a genome-wide association study with over 300.000 SNP in 1215 patients with Behçet’s disease. This study underlines the importance of MHC class I region along with cytokine and cytokine receptor polymorphisms.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Zierhut M, Mizuki N, Ohno S, Inoko H, Gül A, Onoé K, et al. Immunology and functional genomics of Behçet’s disease. Cell Mol Life Sci. 2003;60(9):1903–22.

    Article  CAS  PubMed  Google Scholar 

  18. Sakly K, Lahmar R, Nefzi F, Hammami S, Harzallah O, Sakly N, et al. Phenotypic abnormalities of peripheral blood mononuclear cells in patients with Behçet’s disease and association with HLA-B51 expression. Immunol Invest. 2014;43(5):463–78. This recent study provides an extensive list of peripheral blood immunophenotypes in association with disease activity in Behçet’s disease and emphasizes the significance of immune dysregulation in pathogenesis of Behçet’s disease.

    Article  CAS  PubMed  Google Scholar 

  19. Saruhan-Direskeneli G, Yentür SP, Akman-Demir G, Işik N, Serdaroğlu P. Cytokines and chemokines in neuro-Behçet’s disease compared to multiple sclerosis and other neurological diseases. J Neuroimmunol. 2003;145(1–2):127–34.

    Article  CAS  PubMed  Google Scholar 

  20. Akman-Demir G, Tüzün E, Içöz S, Yeşilot N, Yentür SP, Kürtüncü M, et al. Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine. 2008;44(3):373–6.

    Article  CAS  PubMed  Google Scholar 

  21. Koné-Paut I, Geisler I, Wechsler B, Ozen S, Ozdogan H, Rozenbaum M, et al. Familial aggregation in Behçet’s disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999;135(1):89–93.

    Article  PubMed  Google Scholar 

  22. de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behçet’s disease: a systematic review and meta-analysis of case–control genetic association studies. Arthritis Rheum. 2009;61(10):1287–96.

    Article  PubMed  Google Scholar 

  23. Piga M, Mathieu A. Genetic susceptibility to Behcet’s disease: role of genes belonging to the MHC region. Rheumatology (Oxford). 2011;50(2):299–310.

    Article  CAS  Google Scholar 

  24. Zhang M, Xu WD, Wen PF, Liang Y, Liu J, Pan HF, et al. Polymorphisms in the tumor necrosis factor gene and susceptibility to Behcet’s disease: an updated meta-analysis. Mol Vis. 2013;19:1913–24.

    CAS  PubMed  PubMed Central  Google Scholar 

  25. Vaccarino L, Triolo G, Accardo-Palombo A, Scola L, Palmeri M, Bova M, et al. Pathological implications of Th1/Th2 cytokine genetic variants in Behçet’s disease: data from a pilot study in a Sicilian population. Biochem Genet. 2013;51(11–12):967–75. This article gives a comprehensive account of the impact of cytokine gene polymorphisms on the risk of Behçet’s disease development.

    Article  CAS  PubMed  Google Scholar 

  26. Qi J, Hou S, Zhang Q, Liao D, Wei L, Fang J, et al. A functional variant of pre-miRNA-196a2 confers risk for Behcet’s disease but not for Vogt-Koyanagi-Harada syndrome or AAU in ankylosing spondylitis. Hum Genet. 2013;132(12):1395–404.

    Article  CAS  PubMed  Google Scholar 

  27. Yu H, Liu Y, Bai L, Kijlstra A, Yang P. Predisposition to Behçet’s disease and VKH syndrome by genetic variants of miR-182. J Mol Med (Berl). 2014;92(9):961–7.

    Article  CAS  Google Scholar 

  28. Oner T, Yenmis G, Tombulturk K, Cam C, Kucuk OS, Yakicier MC, et al. Association of pre-miRNA-499 rs3746444 and pre-miRNA-146a rs2910164 polymorphisms and susceptibility to Behcet’s disease. Genet Test Mol Biomarkers. 2015;19(8):424–30.

    Article  CAS  PubMed  Google Scholar 

  29. Mizuki N, Meguro A, Ota M, Ohno S, Shiota T, Kawagoe T, et al. Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet’s disease susceptibility loci. Nat Genet. 2010;42(8):703–6.

    Article  CAS  PubMed  Google Scholar 

  30. Xavier JM, Shahram F, Davatchi F, Rosa A, Crespo J, Abdollahi BS, et al. Association study of IL10 and IL23R-IL12RB2 in Iranian patients with Behçet’s disease. Arthritis Rheum. 2012;64(8):2761–72.

    Article  CAS  PubMed  Google Scholar 

  31. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain. 1999;122(11):2171–82.

    Article  PubMed  Google Scholar 

  32. Borhani Haghighi A, Pourmand R, Nikseresht AR. Neuro-Behçet disease. A review. Neurologist. 2005;11(2):80–9.

    Article  PubMed  Google Scholar 

  33. Aykutlu E, Baykan B, Akman-Demir G, Topcular B, Ertas M. Headache in Behçet’s disease. Cephalalgia. 2006;26(2):180–6.

    Article  CAS  PubMed  Google Scholar 

  34. Seyahi E, Caglar E, Ugurlu S, Kantarci F, Hamuryudan V, Sonsuz A, et al. An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet’s syndrome followed up at a single, dedicated center. Semin Arthritis Rheum. 2015;44(5):602–9.

    Article  PubMed  Google Scholar 

  35. Lakhanpal S, Tani K, Lie JT, Katoh K, Ishigatsubo Y, Ohokubo T. Pathologic features of Behçet’s syndrome: a review of Japanese autopsy registry data. Hum Pathol. 1985;16(8):790–5.

    Article  CAS  PubMed  Google Scholar 

  36. Akman-Demir G, Baykan-Kurt B, Serdaroglu P, Gürvit H, Yurdakul S, Yazici H, et al. Seven-year follow-up of neurologic involvement in Behçet syndrome. Arch Neurol. 1996;53(7):691–4.

    Article  CAS  PubMed  Google Scholar 

  37. Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS, et al. Diagnosis and management of Neuro-Behçet’s disease: international consensus recommendations. J Neurol. 2014;261(9):1662–76.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Bahar S, Coban O, Gürvit IH, Akman-Demir G, Gökyiğit A. Spontaneous dissection of the extracranial vertebral artery with spinal subarachnoid haemorrhage in a patient with Behçet’s disease. Neuroradiology. 1993;35(5):352–4.

    Article  CAS  PubMed  Google Scholar 

  39. Krespi Y, Akman-Demir G, Poyraz M, Tugcu B, Coban O, Tuncay R, et al. Cerebral vasculitis and ischaemic stroke in Behçet’s disease: report of one case and review of the literature. Eur J Neurol. 2001;8(6):719–22.

    Article  CAS  PubMed  Google Scholar 

  40. Oktem-Tanör O, Baykan-Kurt B, Gürvit IH, Akman-Demir G, Serdaroğlu P. Neuropsychological follow-up of 12 patients with neuro-Behçet disease. J Neurol. 1999;246(2):113–9.

    Article  PubMed  Google Scholar 

  41. Yesilot N, Mutlu M, Gungor O, Baykal B, Serdaroglu P, Akman-Demir G. Clinical characteristics and course of spinal cord involvement in Behçet’s disease. Eur J Neurol. 2007;14(7):729–37.

    Article  CAS  PubMed  Google Scholar 

  42. Shugaiv E, Kiyat-Atamer A, Tüzün E, Deymeer F, Oflazer P, Parman Y, et al. Coexistence of Guillain-Barré syndrome and Behçet’s disease. Clin Exp Rheumatol. 2013;31(3 Suppl 77):88–9.

    PubMed  Google Scholar 

  43. Deniz O, Cayköylü A, Vural G, Albayrak Y, Temel S, Aydin I, et al. A case study of neuro-psycho-Behçet’s syndrome presenting with psychotic attack. Clin Neurol Neurosurg. 2009;111(10):877–9.

    Article  PubMed  Google Scholar 

  44. Mimura M, Kato M, Kashima H. Neuro-Behcet’s disease presenting with amnesia and frontal dysfunction. Clin Neurol Neurosurg. 2009;111(10):889–92.

    Article  PubMed  Google Scholar 

  45. Calabrò RS, Grugno R, Muscarà N, Bramanti P. Pitfalls in the diagnosis of Neuro-Behcet presenting with psychiatric symptoms at onset: a case report! Neurol Sci. 2013;34(10):1827–8.

    Article  PubMed  Google Scholar 

  46. Noel N, Hutié M, Wechsler B, Vignes S, Le Thi H-BD, Amoura Z, et al. Pseudotumoural presentation of neuro-Behcet’s disease: case series and review of literature. Rheumatology (Oxford). 2012;51(7):1216–25.

    Article  Google Scholar 

  47. Kuriwaka R, Kunishige M, Nakahira H, Inoue H, Higashi T, Tokumoto Y, et al. Neuro-Behçet’s disease with chorea after remission of intestinal Behçet’s disease. Clin Rheumatol. 2004;23(4):364–7.

    Article  PubMed  Google Scholar 

  48. Gardner RC, Schmahmann JD. Ataxia and cerebellar atrophy—a novel manifestation of neuro-Behçet disease? Mov Disord. 2008;23(2):307–8.

    Article  PubMed  Google Scholar 

  49. Akman-Demir G, Bahar S, Coban O, Tasci B, Serdaroglu P. Cranial MRI in Behcet’s disease: 134 MRI examinations of 98 patients. Neuroradiology. 2003;45(12):851–9.

    Article  CAS  PubMed  Google Scholar 

  50. Saruhan-Direskeneli G, Yentür SP, Mutlu M, Shugaiv E, Yesilot N, Kürtüncü M, et al. Intrathecal oligoclonal IgG bands are infrequently found in neuro-Behcet’s disease. Clin Exp Rheumatol. 2013;31(3 Suppl 77):25–7. This study underlines the significance of cerebrospinal fluid oligoclonal band detection in the differential diagnosis of neuro-Behçet’s disease and multiple sclerosis.

    PubMed  Google Scholar 

  51. Çoban O, Bahar S, Akman-Demir G, Serdaroğlu P, Baykan-Kurt B, Tolun R, et al. Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet’s disease from other CNS diseases? Neuroradiology. 1999;41(4):255–60.

    Article  PubMed  Google Scholar 

  52. Yesilot N, Bahar S, Yılmazer S, Mutlu M, Kurtuncu M, Tuncay R, et al. Cerebral venous thrombosis in Behcet’s disease compared to those associated with other etiologies. J Neurol. 2009;256(7):1134–42.

    Article  CAS  PubMed  Google Scholar 

  53. Uluduz D, Kürtüncü M, Yapıcı Z, Seyahi E, Kasapçopur Ö, Özdoğan H, et al. Clinical characteristics of pediatric onset neuro-Behcet disease. Neurology. 2011;77(21):1900–5.

    Article  CAS  PubMed  Google Scholar 

  54. Kotake S, Higashi K, Yoshikawa K, Sasamoto Y, Okamoto T, Matsuda H. Central nervous system symptoms in patients with Behçet disease receiving cyclosporine therapy. Ophthalmology. 1999;106(3):586–9.

    Article  CAS  PubMed  Google Scholar 

  55. Akman-Demir G, Ayranci O, Kurtuncu M, Vanli EN, Mutlu M, Tugal-Tutkun I. Cyclosporine for Behçet’s uveitis: is it associated with an increased risk of neurological involvement? Clin Exp Rheumatol. 2008;26(4 Suppl 50):S84–90.

    CAS  PubMed  Google Scholar 

  56. Noel N, Bernard R, Wechsler B, Resche-Rigon M, Depaz R, Le Thi Huong Boutin D, et al. Long-term outcome of neuro-Behçet’s disease. Arthritis Rheumatol. 2014;66(5):1306–14. This study underlines the effect of HLA-B51 status on the long-term outcome of NBD patients suggesting a negative influence on the disease course.

    Article  PubMed  Google Scholar 

  57. Akman-Demir G, Saip S, Siva A. Behçet’s disease. Curr Treat Options Neurol. 2011;13(3):290–310.

    Article  PubMed  Google Scholar 

  58. Shugaiv E, Tüzün E, Mutlu M, Kiyat-Atamer A, Kurtuncu M, Akman-Demir G. Mycophenolate mofetil as a novel immunosuppressant in the treatment of neuro-Behçet’s disease with parenchymal involvement: presentation of four cases. Clin Exp Rheumatol. 2011;29(4 Suppl 67):S64–7.

    PubMed  Google Scholar 

  59. Kikuchi H, Aramaki K, Hirohata S. Low dose MTX for progressive neuro-Behçet’s disease. A follow-up study for 4 years. Adv Exp Med Biol. 2003;528:575–8.

    Article  CAS  PubMed  Google Scholar 

  60. Hirohata S, Kikuchi H, Sawada T, Nagafuchi H, Kuwana M, Takeno M, et al. Retrospective analysis of long-term outcome of chronic progressive neurological manifestations in Behcet’s disease. J Neurol Sci. 2015;349(1–2):143–8.

    Article  PubMed  Google Scholar 

  61. Kikuchi H, Aramaki K, Hirohata S. Effect of infliximab in progressive neuro-Behçet’s syndrome. J Neurol Sci. 2008;272(1–2):99–105. This is an interesting study implicating that the beneficial effect of infliximab may be through inhibition of IL-6 production.

    Article  CAS  PubMed  Google Scholar 

  62. Kuroda R, Suzuki J, Muramatsu M, Tasaki A, Yano M, Imai N, et al. Efficacy of infliximab in neuro-Behçet’s disease presenting with isolated longitudinally extensive transverse myelitis. J Neurol. 2013;260(12):3167–70.

    Article  PubMed  PubMed Central  Google Scholar 

  63. Di Filippo M, Di Gregorio M, Nannini C, Gaetani L, Gallina C, Floridi P, et al. Infliximab monotherapy for neuro-Behçet’s disease: a case report. J Neurol Sci. 2014;347(1–2):389–90.

    Article  PubMed  Google Scholar 

  64. Kanemaru H, Makino T, Jinnin M, Yonemitsu A, Makino K, Ihn H. Case of neuro-Behçet’s disease successfully maintained a remission using infliximab for 2 years. J Dermatol. 2013;40(8):632–4.

    Article  PubMed  Google Scholar 

  65. Belzunegui J, López L, Paniagua I, Intxausti JJ, Maíz O. Efficacy of infliximab and adalimumab in the treatment of a patient with severe neuro-Behçet’s disease. Clin Exp Rheumatol. 2008;26(4 Suppl 50):S133–4.

    CAS  PubMed  Google Scholar 

  66. Alty JE, Monaghan TM, Bamford JM. A patient with neuro-Behçet’s disease is successfully treated with etanercept: further evidence for the value of TNFalpha blockade. Clin Neurol Neurosurg. 2007;109(3):279–81.

    Article  PubMed  Google Scholar 

  67. Robinson AB, Gallentine WB, Rabinovich CE. Pediatric neuro-Behçet’s disease responsive to adalimumab. Pediatr Neurol. 2010;43(4):291–3.

    Article  PubMed  Google Scholar 

  68. Leccese P, D’Angelo S, Angela P, Coniglio G, Olivieri I. Switching to adalimumab is effective in a case of neuro-Behcet’s disease refractory to infliximab. Clin Exp Rheumatol. 2010;28(4 Suppl 60):S102.

    PubMed  Google Scholar 

  69. Addimanda O, Pipitone N, Pazzola G, Salvarani C. Tocilizumab for severe refractory neuro-Behçet: three cases IL-6 blockade in neuro-Behçet. Semin Arthritis Rheum. 2015;44(4):472–5.

    Article  CAS  PubMed  Google Scholar 

  70. Pagnini I, Bondi T, Simonini G, Giani T, Marino A, Cimaz R. Successful treatment with canakinumab of a paediatric patient with resistant Behçet’s disease. Rheumatology (Oxford). 2015;54(7):1327–8.

    Article  Google Scholar 

  71. Cantarini L, Vitale A, Scalini P, Dinarello CA, Rigante D, Franceschini R, et al. Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. 2015;34(7):1293–301.

    Article  PubMed  Google Scholar 

  72. Nichols JC, Ince A, Akduman L, Mann ES. Interferon-alpha 2a treatment of neuro-Behcet disease. J Neuroophthalmol. 2001;21(2):109–11.

    Article  CAS  PubMed  Google Scholar 

  73. De Cata A, Intiso D, Bernal M, Molinaro F, Mazzoccoli G, D’Alessandro V, et al. Prolonged remission of neuro-Behcet disease following autologous transplantation. Int J Immunopathol Pharmacol. 2007;20(1):91–6.

    PubMed  Google Scholar 

  74. Erdem H, Dinç A, Pay S, Simşek I, Uysal Y. A neuro-Behçet’s case complicated with intracranial hypertension successfully treated by a lumboperitoneal shunt. Joint Bone Spine. 2006;73(2):200–1.

    Article  PubMed  Google Scholar 

  75. Wroblova K, Kolorz M, Batovsky M, Zboril V, Suchankova J, Bartos M, et al. Gene polymorphisms involved in manifestation of leucopenia, digestive intolerance, and pancreatitis in azathioprine-treated patients. Dig Dis Sci. 2012;57(9):2394–401.

    Article  CAS  PubMed  Google Scholar 

  76. Park JY, Chung YR, Lee K, Song JH, Lee ES. Clinical experience of Interferon alfa-2a treatment for refractory uveitis in Behçet’s disease. Yonsei Med J. 2015;56(4):1158–62.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  77. Chroni E, Tsambaos D. Interferon-alpha for the treatment of severe entero- and neuro-Behçet’s disease. J Dermatol. 2015;42(2):229–30.

    Article  CAS  PubMed  Google Scholar 

  78. Riera-Mestre A, Martínez-Yélamos S, Martínez-Yélamos A, Vidaller A, Pujol R. Neuro-Behçet and neurotoxicity due to cyclosporine. Rev Clin Esp. 2008;208(4):205–6.

    Article  CAS  PubMed  Google Scholar 

  79. Olivieri I, Latanza L, Siringo S, Peruz G, Di Iorio V. Successful treatment of severe Behçet’s disease with infliximab in an Italian Olympic athlete. J Rheumatol. 2008;35(5):930–2.

    CAS  PubMed  Google Scholar 

  80. Kurata I, Tsuboi H, Takahashi H, Abe S, Ebe H, Hagiwara S, et al. Successful treatment with infliximab for refractory uveitis in a hemodialysis patient with Behçet’s disease and a review of the literature for infliximab use in patients on hemodialysis. Intern Med. 2015;54(12):1553–7.

    Article  PubMed  Google Scholar 

  81. Ma D, Zhang CJ, Wang RP, Wang L, Yang H. Etanercept in the treatment of intestinal Behcet’s disease. Cell Biochem Biophys. 2014;69(3):735–9.

    Article  CAS  PubMed  Google Scholar 

  82. Tanida S, Inoue N, Kobayashi K, Naganuma M, Hirai F, Iizuka B, et al. Adalimumab for the treatment of Japanese patients with intestinal Behçet’s disease. Clin Gastroenterol Hepatol. 2015;13(5):940–8.e3.

    Article  CAS  PubMed  Google Scholar 

  83. Dogra S, Khullar G. Tumor necrosis factor-α antagonists: side effects and their management. Indian J Dermatol Venereol Leprol. 2013;79 Suppl 7:S35–46.

    Article  PubMed  Google Scholar 

  84. Vitale A, Rigante D, Caso F, Brizi MG, Galeazzi M, Costa L, et al. Inhibition of interleukin-1 by canakinumab as a successful mono-drug strategy for the treatment of refractory Behçet’s disease: a case series. Dermatology. 2014;228(3):211–4.

    Article  PubMed  Google Scholar 

  85. Urbaniak P, Hasler P, Kretzschmar S. Refractory neuro-Behçet treated by tocilizumab: a case report. Clin Exp Rheumatol. 2012;30(3 Suppl 72):S73–5.

    CAS  PubMed  Google Scholar 

  86. Shapiro LS, Farrell J, Borhani Haghighi A. Tocilizumab treatment for neuro-Behcet’s disease, the first report. Clin Neurol Neurosurg. 2012;114(3):297–8.

    Article  PubMed  Google Scholar 

  87. Li S. Analysis of 27 cases of large vascular lesions in 161 cases of Behcet’s disease: clinical manifestations and treatment outcome. Clin Rheumatol. 2014;33(5):671–5.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Istanbul Faculty of Medicine, Department of Neurology, Istanbul University, Istanbul, Turkey

    Murat Kürtüncü MD

  2. Institute of Experimental Medicine and Research, Department of Neuroscience, Istanbul University, Istanbul, Turkey

    Erdem Tüzün MD

  3. School of Medicine, Department of Neurology, Istanbul Bilim (Science) University, Istanbul, Turkey

    Gulsen Akman-Demir MD

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  1. Murat Kürtüncü MD
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  3. Gulsen Akman-Demir MD
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Correspondence to Gulsen Akman-Demir MD.

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This article is part of the Topical Collection on Neurologic Manifestations of Systemic Disease

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Kürtüncü, M., Tüzün, E. & Akman-Demir, G. Behçet’s Disease and Nervous System Involvement. Curr Treat Options Neurol 18, 19 (2016). https://doi.org/10.1007/s11940-016-0405-6

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