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Neuro-Behçet Syndrome: Clinical Features

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Neuro-Behçet’s Disease

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Abstract

The neurologic involvement of Behçet’s syndrome (BS) is termed as neuro-Behçet syndrome (NBS). Direct neurological involvement of BS may be classified into two forms: (1) parenchymal (p-NBS) and (2) vascular involvement. p-NBS with a rate of 75% among neurological involvement of BS usually presents with an acute-subacute brain stem syndrome. Headache is the most common clinical feature of both parenchymal and cerebral venous sinus thrombosis. Given that dysarthria, ataxia, and hemiparesis are the major clinical features of p-NBS accompanying the headache in almost all cases, magnetic resonance imaging (MRI) and cerebrospinal fluid might help clinicians to differentiate the headache occurring in p-NBS from the nonstructural headache of BS.

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References

  1. Uygunoğlu U, Siva A. Behçet’s syndrome and nervous system involvement. Curr Neurol Neurosci Rep. 2018;18(7):35.

    PubMed  Google Scholar 

  2. Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82:60–76.

    Google Scholar 

  3. Al-Araji A, Kidd DP. Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009;8:192–204.

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  5. Siva A, Kantarci OH, Saip S, et al. Behçet’s disease: diagnostic & prognostic aspects of neurological involvement. J Neurol. 2001;248:95–103. 24.

    CAS  PubMed  Google Scholar 

  6. Siva A, Esatoglu SN, Uygunoglu U, et al. Could neurological involvement in Behçet’s disease occur atypically? Neurology. 2018;90(15 Supplement):P5. 410.

    Google Scholar 

  7. Uygunoglu U, Zeydan B, Ozguler Y, et al. Myelopathy in Behçet’s disease: the bagel sign. Ann Neurol. 2017;82(2):288–98.

    PubMed  Google Scholar 

  8. Kocer N, Islak C, Siva A, et al. CNS involvement in neuro-Behcet’s syndrome: an MR study. Am J Neuroradiol. 1999;20:1015–24.

    CAS  PubMed  Google Scholar 

  9. McMenemey WH, Lawrence BJ. Encephalomyelopathy in Behçet disease: report of necropsy findings in two cases. Lancet. 1957;2:353–8.

    Google Scholar 

  10. Siva A, Saip S. The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J Neurol. 2009;256(4):513–29.

    PubMed  Google Scholar 

  11. Uygunoglu U, Saip S, Siva A. Chapter 28: Behcet’s syndrome and nervous system involvement. In: Lisak RP, Truong DD, Carroll WM, et al., editors. International neurology. 2nd ed. Chichester/Hoboken: John Wiley & Sons, Ltd; 2016. p. 88–93.

    Google Scholar 

  12. Taskapilioglu O, Seferoglu M, Akkaya C, et al. Delayed diagnosis of a neuroBehcet patient with only brainstem and cerebellar atrophy: literature review. J Neurol Sci. 2009;277:160–3.

    PubMed  Google Scholar 

  13. Serdaroğlu P, Yazici H, Ozdemir C, Yurdakul S, Bahar S, Aktin E. Neurologic involvement in Behçet’s syndrome. A prospective study. Arch Neurol. 1989;46(3):265–9.

    PubMed  Google Scholar 

  14. 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(Pt 11):2171–82.

    PubMed  Google Scholar 

  15. Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications in Behçet’s syndrome. Brain. 1999;122(Pt 11):2183–94.

    PubMed  Google Scholar 

  16. Siva A. Common clinical and imaging conditions misdiagnosed as multiple sclerosis: a current approach to the differential diagnosis of multiple sclerosis. Neurol Clin. 2018;36(1):69–117.

    PubMed  Google Scholar 

  17. Ishido M, Horita N, Takeuchi M, Shibuya E, Yamane T, Kawagoe T, et al. Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis. Sci Rep. 2017;7(1):10196.

    PubMed  PubMed Central  Google Scholar 

  18. Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983;33:1444–52.

    CAS  PubMed  Google Scholar 

  19. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.

    CAS  Google Scholar 

  20. 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.

    CAS  PubMed  Google Scholar 

  21. Aguiar de Sousa D, Mestre T, Ferro JM. Cerebral venous thrombosis in Behçet’s disease: a systematic review. J Neurol. 2011;258(5):719–27.

    CAS  PubMed  Google Scholar 

  22. Uluduz D, Midi I, Duman T, et al. Behçet’s disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study. Rheumatology (Oxford). 2018;58(4):600–8. https://doi.org/10.1093/rheumatology/key153.

    Article  CAS  Google Scholar 

  23. Yesilot N, Bahar S, Yilmazer S, et al. Cerebral venous thrombosis in Behçet’s disease compared to those associated with other etiologies. J Neurol. 2009;256(7):1134–42.

    CAS  PubMed  Google Scholar 

  24. Uluduz D, Kürtüncü M, Yapıcı Z, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011;77(21):1900–5.

    CAS  PubMed  Google Scholar 

  25. Shi J, Huang X, Li G, Wang L, Liu J, Xu Y, Zeng X, Zheng W. Cerebral venous sinus thrombosis in Behçet’s disease: a retrospective case-control study. Clin Rheumatol. 2018;37(1):51–7.

    PubMed  Google Scholar 

  26. Matsumoto T, Uekusa T, Fukuda Y. Vasculo-Behçet’s disease: a pathologic study of eight cases. Hum Pathol. 1991;22(1):45–51.

    CAS  PubMed  Google Scholar 

  27. Kizilkilic O, Albayram S, Adaletli I, et al. Endovascular treatment of Behçet’s disease-associated intracranial aneurysms: report of two cases and review of the literature. Neuroradiology. 2003;45(5):328–34.

    PubMed  Google Scholar 

  28. Siva A, Özdogan H, Yazici H, et al. Headache, neuro-psychiatric and computerized tomography findings in Behçet’s syndrome. In: Lehner T, Barnes CG, editors. Recent advances in Behçet’s disease. London: Royal Society of Medicine Service; 1986. p. 247–54.

    Google Scholar 

  29. Oktem-Tanor O, Baykan-Kurt B, Gurvit IH, Akman-Demir G, Serdaroglu P. Noel neuropsychological follow-up of 12 patients with neuro-Behcet disease. J Neurol. 1999;246(2):113–9.

    CAS  PubMed  Google Scholar 

  30. Cavaco S, da Silva AM, Pinto P, et al. Cognitive functioning in Behçet’s disease. Ann N Y Acad Sci. 2009;1173:217–26.

    CAS  PubMed  Google Scholar 

  31. Saip S, Siva A, Altintas A, et al. Headache in Behcet’s syndrome. Headache. 2005;45:911–9.

    PubMed  Google Scholar 

  32. Borhani Haghighi A, Aflaki E, Ketabchi L. The prevalence and characteristics of different types of headache in patients with Behçet’s disease, a case-control study. Headache. 2008;48(3):424–9.

    PubMed  Google Scholar 

  33. Kale N, Agaoglu J, Icen M, Yazici I, Tanik O. The presentation of headache in neuro-Behçet’s disease: a case-series. Headache. 2009;49(3):467–70.

    PubMed  Google Scholar 

  34. Avci O, Kutluay E, Argon M, Erdem S, Tahsin Gunes A. Subclinical cerebral involvement in Behcet’s disease: a SPECT study. Eur J Neurol. 1998;5(1):49–53.

    CAS  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, Istanbul University Cerrahpaşa School of Medicine, Istanbul, Turkey

    Ugur Uygunoglu & Aksel Siva

Authors
  1. Ugur Uygunoglu
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  2. Aksel Siva
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Editors and Affiliations

  1. Istanbul University, Istanbul, Turkey

    Erdem Tüzün

  2. Istanbul University, Istanbul, Turkey

    Murat Kürtüncü

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Uygunoglu, U., Siva, A. (2021). Neuro-Behçet Syndrome: Clinical Features. In: Tüzün, E., Kürtüncü, M. (eds) Neuro-Behçet’s Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-55273-2_8

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