Skip to main content

Advertisement

SpringerLink
Log in

Neurological Complications of Behçet’s Syndrome

  • Infection (B Jubelt, Section Editor)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Behçet’s syndrome is an autoinflammatory disease of uncertain cause characterized by mucous membrane ulceration, arthritis and inflammation within most tissues. Its prevalence differs throughout different populations, but neurological complications arise in 10 % of all those affected. The majority develop inflammation within the central nervous system which may remit but may also progressively worsen without treatment. This article reviews the epidemiology, clinical characteristics, natural history and management of the disorders, and emphasizes recent considerable advances in our understanding of the various treatments which may be beneficial across the spectrum of disease types.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

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

  1. • Yazici H, Fresko I, Yurdukal S. Behçet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007; 3:148–55. This is the best review on systemic manifestations of the disease published to date.

    Google Scholar 

  2. Yamamoto SI, Toykawa H, Matsubara J, et al. A nationwide survey of Behçet’s disease in Japan. I. Epidemiological survey. Jpn J Ophthalmol. 1974;18:282–90.

    Google Scholar 

  3. Yurdukal S, Gunaydin I, Tuzun Y, Tankurt N, Parzarli H, Ozyazgan Y, et al. The prevalence of Behçet’s syndrome in a rural area in Northern Turkey. J Rheumatol. 1988;15:820–2.

    Google Scholar 

  4. Kidd D. An epidemiological survey of Behçet’s syndrome and its neurological complications in Hertfordshire, UK. Adv Exp Med Biol. 2003;528:95–7.

    Article  PubMed  Google Scholar 

  5. Serdaroglu P, Yazici H, Ozdemir C, Yurdakul S, Bahar S, Atkin E. Neurologic involvement in Behçet’s syndrome: a prospective study. Arch Neurol. 1989;46:265–9.

    Article  PubMed  CAS  Google Scholar 

  6. • Al Araji A, Kidd D. Neuro-Behcet’s disease: epidemiology, clinical characteristics and management. Lancet Neurol. 2009;8:192–204. The most comprehensive study of neurological manifestations published to date.

  7. 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;16:790–5.

    Article  PubMed  CAS  Google Scholar 

  8. Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications in Behçet’s syndrome. Brain. 1999;122:2181–94.

    Google Scholar 

  9. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain. 1999;122:2171–80.

    Article  PubMed  Google Scholar 

  10. Al-Fahad SA, Al-Araji AH. Neuro-Behçet’s disease in Iraq: a study of 40 patients. J Neurol Sci. 1999;170:105–11.

    Article  PubMed  CAS  Google Scholar 

  11. Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, et al. Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol. 2001;248:95–103.

    Article  PubMed  CAS  Google Scholar 

  12. Ideguchi H, Suda A, Takeno M, Ihata A, Ueda A, Ohno S, et al. Neurological manifestations of Behçet’s disease in Japan: a study of 54 patients. J Neurol. 2010;257:1012–20.

    Article  PubMed  Google Scholar 

  13. Shimojo S, Yamamoto N, Matsuda T, Yuinari T. Asymptomatic neuro-Behçet’s disease. J Neuroimaging. 1998;8:59–60.

    PubMed  CAS  Google Scholar 

  14. Yesilot N, Shehu M, Oktem-Tonor O, Serdaroglu P, Akman-Demir G. Silent neurological involvement in Behçet’s disease. Clin Exp Rheumatol. 2006;24 Suppl 42:S65–70.

    PubMed  CAS  Google Scholar 

  15. Bousser MG, Bletry O, Launay M, Portier E, Giullard A, Castaigne P. Thromboses veineuses cerebrales au cours de la maladie de Behcet. Rev Neurol. 1980;136:753–62.

    PubMed  CAS  Google Scholar 

  16. Wechsler B, Vidaihet M, Piette PC, Bousser MG, Dell Isola B, Bletry O, et al. Cerebral venous sinus thrombosis in Behçet’s disease; clinical study and long term follow up of 25 cases. Neurology. 1992;42:614–18.

    Article  PubMed  CAS  Google Scholar 

  17. • Aguiar de Sousa D, Mestre T, Ferro JM. Cerebral venous thrombosis in Behçet’s disease: a systematic review. J Neurol. 2011;258:719–27. This is an important study which helps to define the treatment of this manifestation of the disease; physicians inexperienced in the management of the condition find it difficult to decide on optimum therapy.

  18. Lee SH, Yoon PH, Park SJ, Kim DI. MRI findings in neuro-Behçet’s disease. Clin Radiol. 2001;56:485–94.

    Article  PubMed  CAS  Google Scholar 

  19. Alkan A, Goktan A, Karincaoglu Y, Kamisli S, Dogan M, Oztanir N, et al. Brain perfusion MRI findings in patients with Behçet’s disease. ScientificWorldJournal. 2012;2012:261502.

    Article  PubMed  Google Scholar 

  20. Kang DW, Chu K, Cho JY, Koo JS, Yoon BW, Roh JK, et al. Diffusion-weighted MRI in neuro-Behçet’s disease. J Neurol Neurosurg Psychiatry. 2001;70:412–3.

    Article  PubMed  CAS  Google Scholar 

  21. Coban O, Bahar S, Akman-Demir G, Tasci G, Yudakul S, Yazici H, et al. Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet’s disease from other central nervous system diseases? Neuroradiology. 1999;41:255–60.

    Article  PubMed  CAS  Google Scholar 

  22. Sarhan-Direskeneli G, Yentur SP, Akman-Demir G, Isik N, Serdaroglu P. Cytokines and chemokines in neuro-Behçet’s disease compared to multiple sclerosis and other neurological diseases. J Neuroimmunol. 2003;145:127–34.

    Article  Google Scholar 

  23. Hirohata S, Isshi K, Oguchi H, Ohse T, Haraoka H, Takeuchi A, et al. Cerebrospinal fluid interleukin-6 in progressive neuro-Behçet’s syndrome. Clin Immunol Immunopathol. 1997;82:12–7.

    Article  PubMed  CAS  Google Scholar 

  24. 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 risk association studies. Arthritis Rheum. 2009;61:1287–96.

    Article  PubMed  Google Scholar 

  25. Maldini C, Lavalley MP, Cheminant M, de Menthon M, Mahr A. Relationships of HLA-B51 or B5 genotype with Behçet’s disease clinical characteristics: systematic review and mate-analysis of observational studies. Rheumatology (Oxford). 2012;51:887–900.

    Article  Google Scholar 

  26. Hadfield MG, Aydin F, Lippman HR, Kubal WS, Sanders KM. Neuro-Behçet’s disease. Clin Neuropathol. 1996;15:249–55.

    PubMed  CAS  Google Scholar 

  27. Arai Y, Kohno S, Takahashi Y, Miyajima Y, Tsutsui Y. Autopsy case of neuro-Behçet’s disease with multifocal neutrophilic perivascular inflammation. Neuropathology. 2006;26:579–85.

    Article  PubMed  Google Scholar 

  28. Hirohata S. Histopathology of central nervous system lesions in Behçet’s disease. J Neurol Si. 2008;267:41–7.

    Article  CAS  Google Scholar 

  29. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, et al. Management of Behçet’s disease: a systematic literature review for the EULAR evidence based recommendations for the management of Behçet’s disease. Ann Rheum Dis. 2008;67:1656–62.

    Article  PubMed  CAS  Google Scholar 

  30. Ait Ben Haddou EH, Imounan F, Regragui W, Mouti O, Benchakroune N, Abouqal R, et al. Neurological manifestations of Behçet’s disease: evaluation of 40 patients treated by cyclophosphamide. Rev Neurol (Paris). 2012;168:344–9.

    Article  CAS  Google Scholar 

  31. Piptone N, Olivieri I, Padula A, et al. Infliximab for the treatment of neuro-Behçet’s disease: a case series and literature review. Arthritis Rheum. 2008;59:285–90.

    Article  Google Scholar 

  32. • Giardina A, Ferrante A, Ciccia F, Vadala M, Giardina E, Triolo G. One year study of the efficacy and safety of infliximab in the treatment of patients with ocular and neurological Behçet’s disease refractory to standard immunosuppressive drugs. Rheumatol Int. 2011;31:33–7. This study is an important assessment of the most modern forms of treatment of severe manifestations of the disease.

  33. Olivieri I, Leccese P, D’Angelo S, Padula A, Nigro A, Palazzi C, et al. Efficacy of adalimumab in patients with Behçet’s disease unsuccessfully treated with infliximab. Clin Exp Rheumatol. 2011;29 Suppl 67:S54–57.

    PubMed  Google Scholar 

Download references

Disclosure

No potential conflicts of interest relevant to this article were reported.

Author information

Authors and Affiliations

  1. Department of Clinical Neurosciences, Royal Free Hospital, London, NW3 2QG, UK

    Desmond Kidd

Authors
  1. Desmond Kidd
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Desmond Kidd.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kidd, D. Neurological Complications of Behçet’s Syndrome. Curr Neurol Neurosci Rep 12, 675–679 (2012). https://doi.org/10.1007/s11910-012-0316-1

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11910-012-0316-1

Keywords

Search

Navigation